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1c81c6b1-87ca-4b7d-b0e3-39ab2c4d3a42

A 45-year-old man presents to the clinic for evaluation of weakness in his arms and legs. The symptoms started gradually, and are now more noticeable and interfering with his ability to work as an electrician. On physical examination, the cranial nerves are normal, but there is weakness of his left handgrip and right leg quadriceps with loss of muscle bulk. There are obvious fasciculations over the left forearm and right thigh. Tone is increased in the arms and legs and the reflexes are brisk. Which of the following is the most likely diagnosis?

amyotrophic lateral sclerosis (ALS)

myotonic muscular dystrophy

amyotonia congenita

tabes dorsalis

0a

multi

The most common initial symptom of ALS is weakness and wasting of the extremities. The fasciculations can be a very prominent part of the disease. This is rare in other neurologic disorders.

Medicine

C.N.S.

A 45-year-old man presents to the clinic for evaluation of weakness in his arms and legs. The symptoms started gradually, and are now more noticeable and interfering with his ability to work as an electrician. On physical examination, the cranial nerves are normal, but there is weakness of his left handgrip and right leg quadriceps with loss of muscle bulk. There are obvious fasciculations over the left forearm and right thigh. Tone is increased in the arms and legs and the reflexes are brisk.

Which of the following is the most likely diagnosis?

A. amyotrophic lateral sclerosis (ALS)

0e9b9822-5a51-480b-9c9a-3ef7fc89b686

A 46-year-old woman presents with fever, hemoptysis, weight loss, and night sweats. She has never smoked. She recently returned returned from a month-long trip to Asia. A chest radiograph reveals apical lesions with cavitation in the left lung. A purified protein derivative (PPD) test is placed, and 48 hours later an 18-mm wheal develops. Sputum cultures reveal numerous acid-fast organisms. This patient is put on ontact precautions, and a regimen for tuberculosis is staed. Which of the following disorders does this patient most likely have?

Acquired immunodeficiency syndrome

Congenital immunodeficiency

Miliary tuberculosis, with seeding of distal organs with innumerable small millet seed-like lesions

Secondary tuberculosis, resulting from activation of a prior Ghon complex, with spread to a new pulmonary site

3d

multi

Tuberculosis, at one time a frequent hazard in the United States, is now relatively uncommon except in immunocompromised individuals Primary tuberculosis is the initial infection by M. tuberculosis, and is restricted to the primary, or Ghon complex. Cavitation and selective localization to the pulmonary apices are characteristics of secondary tuberculosis. Secondary tuberculosis may spread through the lymphatics and blood to other organs, resulting in miliary tuberculosis

Pathology

Systemic Pathology

A 46-year-old woman presents with fever, hemoptysis, weight loss, and night sweats. She has never smoked. She recently returned returned from a month-long trip to Asia. A chest radiograph reveals apical lesions with cavitation in the left lung. A purified protein derivative (PPD) test is placed, and 48 hours later an 18-mm wheal develops. Sputum cultures reveal numerous acid-fast organisms. This patient is put on ontact precautions, and a regimen for tuberculosis is staed.

Which of the following disorders does this patient most likely have?

D. Secondary tuberculosis, resulting from activation of a prior Ghon complex, with spread to a new pulmonary site

521d1361-c99d-4bfc-aa9a-993b8e4a6385

A 28-year-old male with AIDS presents with moderate proteinuria and hypeension. Histologic sections of the kidney reveal the combination of normal-appearing glomeruli and occasional glomeruli that have deposits of hyaline material. No increased cellularity or necrosis is noted in the abnormal glomeruli. Additionally, there is cystic dilation of the renal tubules, some of which are filled with proteinaceous material. Electron microscopy reveals focal fusion of podocytes, and immunofluorescence examination finds granular IgM/C3 deposits. What is the best diagnosis for this renal abnormality?

Diffuse proliferative glomerulonephritis (DPGN)

Focal segmental glomerulonephritis (FSGN)

Focal segmental glomerulosclerosis (FSGS)

Membranous glomerulopathy (MGN)

2c

multi

Focal segmental glomerulosclerosis (FSGS) is a glomerular disorder that accounts for about 10% of the cases of nephrotic syndrome. FSGS, which affects children and adults, begins as a focal process, affecting only some glomeruli. In the earliest stage, only some of the juxtamedullary glomeruli show changes. Eventually, some glomeruli in other pas of the coex are affected. In the late stages of the disease, the process may become diffuse, affecting most or all glomeruli. Initially, the process is also segmental, involving some but not all of the lobules within an individual glomerular tuft. The involved area shows sclerosis and may show hyalinosis lesions. Eventually some glomeruli show sclerosis of the entire tuft (global sclerosis). Electron microscopy shows increased mesangial matrix and dense granular mesangial deposits. Immunofluorescence typically shows granular mesangial fluorescence for IgM and C3. Because of the focal nature of FSGS, early cases can be difficult to distinguish from minimal change disease (MCD). Clinically, the nephrotic syndrome of FSGS is more severe than that of minimal change disease and is nonselective. The process is much less responsive to steroids and is much more prone to progress to chronic renal failure. It tends to recur in transplanted kidneys. FSGS can be seen in the setting of AIDS nephropathy and heroin nephropathy. Reference: Robbins & Cotran Pathologic Basis of Disease, 9edition

Pathology

miscellaneous

A 28-year-old male with AIDS presents with moderate proteinuria and hypeension. Histologic sections of the kidney reveal the combination of normal-appearing glomeruli and occasional glomeruli that have deposits of hyaline material. No increased cellularity or necrosis is noted in the abnormal glomeruli. Additionally, there is cystic dilation of the renal tubules, some of which are filled with proteinaceous material. Electron microscopy reveals focal fusion of podocytes, and immunofluorescence examination finds granular IgM/C3 deposits.

What is the best diagnosis for this renal abnormality?

C. Focal segmental glomerulosclerosis (FSGS)

5bf1c089-0478-42c8-87f7-7a3f82da64b7

A 55-year-old woman who has a history of severe depression and had a radical mastectomy for carcinoma of breast one year back develops polyuria nocturia and excessive thirst - Laboratory values are as follows: Serum sodium 149 m Eq/L, Serum potassium 3.6 m Eq/L, Serum calcium 9.5 mg/dl, Glucose 110 mg.dl, Bun 30 mg/dl, Urine osmolarity 150 in Osm kg. The most likely clinical diagnosis would be -

Psychogenic polydipsia

Renal glycosuria

Hypercalcicuria

Diabetes insipidus

3d

single

Medicine

A 55-year-old woman who has a history of severe depression and had a radical mastectomy for carcinoma of breast one year back develops polyuria nocturia and excessive thirst - Laboratory values are as follows: Serum sodium 149 m Eq/L, Serum potassium 3.6 m Eq/L, Serum calcium 9.5 mg/dl, Glucose 110 mg.dl, Bun 30 mg/dl, Urine osmolarity 150 in Osm kg.

The most likely clinical diagnosis would be -

D. Diabetes insipidus

e09491a1-829b-404c-8031-aa08f5734000

28-year-old pregnant lady comes to the ER with headache, blurring of vision and vomiting. On examination, her BP is 195/150 mm Hg, ankle edema present, urinary proteinuria present. On inquiry, she is primi and it is 23 weeks of gestation. All on a sudden, she develops convulsion. Intravenous magnesium sulphate is introduced immediately and she recovers smoothly. During discharge, which of the following medication will be justified?

Nifedipine

Metoproplof

Methyldopa

Olmesartan

1b

multi

Answer: b) MetoprololMethyldopa is still the drug of choice for many obstetricians in pregnancy, because extensive experience has shown that it does not harm the fetus.Nifedipine is also used in pregnancy, but it is kept in second class. Nifedipine can reduce BP immediately and it is unpredictable even sometimes large enough to cause cerebral ischemia.Beta blockers can cause growth retardation in first and second trimester. It can be used in last trimester in combination with hydralazine to combat the unpleasant tachycardia.ACE-inhibitors and ARBs (i.e. - Losartan, Olmesartan) are absolutely contraindicated during pregnancy, where they cause fetal death, typically mid-trimester.

Gynaecology & Obstetrics

Miscellaneous (Gynae)

28-year-old pregnant lady comes to the ER with headache, blurring of vision and vomiting. On examination, her BP is 195/150 mm Hg, ankle edema present, urinary proteinuria present. On inquiry, she is primi and it is 23 weeks of gestation. All on a sudden, she develops convulsion. Intravenous magnesium sulphate is introduced immediately and she recovers smoothly.

During discharge, which of the following medication will be justified?

B. Metoproplof

960a2d7e-786c-4fe2-8c4a-424807d584f2

A 40-year-old man has been bothered by oral candidiasis, fever, and diarrhea for the past year. On physical examination, he has muscle wasting. His weight is 70% of normal for his height and age. He has generalized nontender lymphadenopathy, but no hepatosplenomegaly. He developed three irregular, 1- to 2-cm, reddish-purple, nodular skin lesions on his forearm in the past 3 months. Laboratory findings show hemoglobin, 12.2 g/dL; hematocrit, 36.5%; MCV, 85 mm3; platelet count, 188,000/ mm3; and WBC count, 2460/ mm3 with 82% segmented neutrophils, 4% bands, 6% lymphocytes, 6% monocytes, and 2% eosinophils. Infection with which of the following organisms is most likely to produce these findings?

Hepatitis C virus

Herpes simplex virus

HIV

Mycobacterium leprae

2c

multi

The reddish-purple lesions are typical of Kaposi sarcoma in a patient with wasting syndrome, oral thrush, and lymphopenia characteristic of HIV infection with AIDS. Hepatitis C virus is unlikely to produce skin lesions or lymphopenia of this degree. Herpes simplex virus infections may be seen more frequently in HIV infection, but the lesions are typically vesicular and are located in the perioral or perianal regions. Hansen disease, caused by Mycobacterium leprae infection, may produce a faint reddish rash that fades, followed by hypopigmentation or anesthesia of affected skin and sometimes nodular deforming lesions developing over years. Staphylococcal skin infections tend to produce localized abscesses, such as furuncles and boils. Streptococcal skin infections may manifest as abscesses or as cellulitis.

Pathology

Infectious Disease

A 40-year-old man has been bothered by oral candidiasis, fever, and diarrhea for the past year. On physical examination, he has muscle wasting. His weight is 70% of normal for his height and age. He has generalized nontender lymphadenopathy, but no hepatosplenomegaly. He developed three irregular, 1- to 2-cm, reddish-purple, nodular skin lesions on his forearm in the past 3 months. Laboratory findings show hemoglobin, 12.2 g/dL; hematocrit, 36.5%; MCV, 85 mm3; platelet count, 188,000/ mm3; and WBC count, 2460/ mm3 with 82% segmented neutrophils, 4% bands, 6% lymphocytes, 6% monocytes, and 2% eosinophils.

Infection with which of the following organisms is most likely to produce these findings?

C. HIV

92c4e03e-f5c3-4384-9f63-d2e62b7234e3

A 25 years female was found in the room with 100% burns on her body. The tongue was protruding out; the body was in pugilistic attitude with heat ruptures, peeling of skin, and heat haematoma and heat fractures of the skull. Carboxyhemoglobin was 25% and soot particles were present in the trachea. Which of the combinations of two findings will establish that the burns were antemortem in nature-

Heat haematoma & heat ruptures

Heat fracture of skull and peeling of skin

Heat haematoma and pugilistic attitude

Carboxy hemoglobin (25%) and soot particles in trachea

3d

single

Presence of carbon particles (soot) in respiratory passages and elevated level of carboxy-hemoglobin confirms that victim was alive when the burn occurred. i.e. Ante-mortem burn.

Forensic Medicine

A 25 years female was found in the room with 100% burns on her body. The tongue was protruding out; the body was in pugilistic attitude with heat ruptures, peeling of skin, and heat haematoma and heat fractures of the skull. Carboxyhemoglobin was 25% and soot particles were present in the trachea.

Which of the combinations of two findings will establish that the burns were antemortem in nature-

D. Carboxy hemoglobin (25%) and soot particles in trachea

09bde39a-49d5-449d-97c6-e20d4529ae8c

Guddu, a 5–year–old female was brought to the emergency with fever, headache and confusion. A provisional diagnosis of bacterial meningitis was made. The baby developed a severe allergic reaction to penicillin aroud six months back. She was admitted and intravenous antibiotics were started. Few days later her investigations revealed as: Hemoglobin – 6.0 g/L Erythrocyte count – 1.2 x 106/mm3 Platelets – 60000/mm3 Leukocyte count – 1500/mm3 Which of the following is the most likely drug responsible for the above findings ?

Gentamicin

Chloramphenicol

Doxycycline

Vancomycin

1b

multi

Pharmacology

Guddu, a 5–year–old female was brought to the emergency with fever, headache and confusion. A provisional diagnosis of bacterial meningitis was made. The baby developed a severe allergic reaction to penicillin aroud six months back. She was admitted and intravenous antibiotics were started.

Few days later her investigations revealed as: Hemoglobin – 6.0 g/L Erythrocyte count – 1.2 x 106/mm3 Platelets – 60000/mm3 Leukocyte count – 1500/mm3 Which of the following is the most likely drug responsible for the above findings ?

B. Chloramphenicol

f85738bf-cb6a-4679-8097-d59da9aab463

A 36 year old male presents with a complaint of passing dark-reddish urine. He states that yesterday he played basketball for 4 hours, which was the first time he had exercised in 4 months. He awoke this morning with sore muscles and discolored urine. Physical examination is unremarkable. The urine is reddish-brown in color; dipstick test for blood is positive, the pH is 5.1, the specific gravity 1.03. Microscopic examination of the urine reveals no red blood cells. What is the most likely etiology for this presentation?

Hemolyzed blood in the urine

Ingestion of foods that contained red dye C. Myoglobinuria

Myoglobinuria

Nephrolithiasis

2c

multi

The appearance of a dark-reddish urine is suggestive of a number of conditions, including hematuria; however, there are no red blood cells present on microscopic examination. Fuhermore, the dipstick test for blood is positive, which is suggestive of myoglobinuria. Myoglobin is a pigment that, if present in the urine, will produce a positive dipstick test, even in the absence of red blood cells. Myoglobinuria can be caused when an unconditioned individual paakes in intense physical exeion. This extreme physical activity leads to muscle cell breakdown and the release of myoglobin into the blood, which is then filtered by the kidneys and excreted into the urine. If there was hemolyzed blood in the urine , a few red blood cells should have been detected on urinalysis. Ingestion of foods that contained red dye can discolor the urine; however, it would not produce a positive dipstick test. Nephrolithiasis generally results in the appearance of blood cells in the urine. Ref: Kennelly P.J., Rodwell V.W. (2011). Chapter 6. Proteins: Myoglobin & Hemoglobin. In D.A. Bender, K.M. Botham, P.A. Weil, P.J. Kennelly, R.K. Murray, V.W. Rodwell (Eds), Harper's Illustrated Biochemistry, 29e.

Biochemistry

A 36 year old male presents with a complaint of passing dark-reddish urine. He states that yesterday he played basketball for 4 hours, which was the first time he had exercised in 4 months. He awoke this morning with sore muscles and discolored urine. Physical examination is unremarkable. The urine is reddish-brown in color; dipstick test for blood is positive, the pH is 5.1, the specific gravity 1.03. Microscopic examination of the urine reveals no red blood cells.

What is the most likely etiology for this presentation?

C. Myoglobinuria

071f1b94-3e92-4da4-bd9c-a12bf8a4138f

A 40-year-old man complains of nausea, vomiting, diarrhea, and cramping abdominal pain. His temperature is 38degC , blood pressure 90/60 mm Hg, and pulse rate 90/minute. On physical examination, the patient appears dehydrated, with sunken eyeballs, dry tongue, and poor skin turgor. Hyperpigmentation is noted in the palmar creases and the gingival margins. Laboratory results include fasting serum glucose of 62 mg/dL, BUN of 27 mg/dL, Na of 122 mEq/L, and K of 6.5 mEq/L. Which of the following is the most likely cause of this patient's symptoms?

Amyloidosis

Autoimmunity

Metastatic cancer

Sarcoidosis

1b

multi

- Given clinical features suggests the diagnosis of Primary chronic adrenal insufficiency (Addison disease). - MCC of addison disease in developed countries - Autoimmunity - MCC of addison disease in developing countries like India - Tuberculosis - It is fatal wasting disorder caused by failure of adrenal glands to produce glucocoicoids, mineralocoicoids & androgens. - If untreated, Addison disease is characterized by weakness, weight loss, gastrointestinal symptoms, hypotension, electrolyte disturbances & hyperpigmentation.

Pathology

Adrenal Gland

A 40-year-old man complains of nausea, vomiting, diarrhea, and cramping abdominal pain. His temperature is 38degC , blood pressure 90/60 mm Hg, and pulse rate 90/minute. On physical examination, the patient appears dehydrated, with sunken eyeballs, dry tongue, and poor skin turgor. Hyperpigmentation is noted in the palmar creases and the gingival margins. Laboratory results include fasting serum glucose of 62 mg/dL, BUN of 27 mg/dL, Na of 122 mEq/L, and K of 6.5 mEq/L.

Which of the following is the most likely cause of this patient's symptoms?

B. Autoimmunity

a26c3a77-a853-41dc-a22e-3c5042caa70f

An 8-year-old child has had abdominal pain and dark urine for 10 days. Physical examination shows blotchy purple skin lesions on the trunk and extremities. Urinalysis shows hematuria and proteinuria. Serologic test results are negative for MPO-ANCA (P-ANCA) and PR3-ANCA (C-ANCA). A skin biopsy specimen shows necrotizing vasculitis of small dermal vessels. A renal biopsy specimen shows immune complex deposition in glomeruli, with some IgA-rich immune complexes. Which of the following is the most likely diagnosis?

Giant cell arteritis

Henoch-Schonlein purpura

Polyarteritis nodosa

Takayasu arteritis

1b

multi

In children, Henoch-Schonlein purpura is the multisystemic counterpart of the IgA nephropathy seen in adults. The immune complexes formed with IgA produce the vasculitis that affects mainly arterioles, capillaries, and venules in the skin, gastrointestinal tract, and kidney. In older adults, giant cell arteritis is seen in external carotid branches, principally the temporal artery unilaterally. Polyarteritis nodosa is seen most often in small muscular arteries and sometimes veins, with necrosis and microaneurysm formation followed by scarring and vascular occlusion. This occurs mainly in the kidney, gastrointestinal tract, and skin of young to middle-aged adults. Takayasu arteritis is seen mainly in children and involves the aorta (particularly the arch) and branches such as coronary and renal arteries, with granulomatous inflammation, aneurysm formation, and dissection. Telangiectasias are small vascular arborizations seen on the skin or mucosal surfaces.

Pathology

Blood Vessels

An 8-year-old child has had abdominal pain and dark urine for 10 days. Physical examination shows blotchy purple skin lesions on the trunk and extremities. Urinalysis shows hematuria and proteinuria. Serologic test results are negative for MPO-ANCA (P-ANCA) and PR3-ANCA (C-ANCA). A skin biopsy specimen shows necrotizing vasculitis of small dermal vessels. A renal biopsy specimen shows immune complex deposition in glomeruli, with some IgA-rich immune complexes.

Which of the following is the most likely diagnosis?

B. Henoch-Schonlein purpura

6865f332-6232-457b-9d77-f9ecacb27436

A 55-year-old woman presents to the clinic for evaluation of several weeks of fever, abdominal pain, weight loss, and lack of energy. Three days prior to the assessment, she developed a left foot drop. Her blood pressure is 160/90 mm Hg, pulse 80/min, and the physical examination confirms left peroneal nerve damage and a bilateral sensory neuropathy in both legs. There are no skin rashes. Laboratory evaluation reveals an ESR of 105 mm/h, WBC of 14,000/mL, and negative serologic tests for antineutrophil cytoplasmic antibody (ANCA) and ANA. The eosinophil count is normal, and urinalysis is negative for casts, protein, and red cells. A clinical diagnosis of polyarteritis nodosa is made. Which of the following is the most likely mechanism for renal injury in this condition?

nephrotic syndrome

diffuse glomerulonephritis

granuloma formation

necrotizing vasculitis of vessels

3d

multi

This patient has polyarteritis nodosa (PAN) and in classic PAN, unlike microscopic polyangiitis, both small and medium vessels are involved. The renal lesions are ischemic secondary to fibrinoid necrosis of the vessels. In microscopic polyangiitis, a diffuse glomerulonephritis is frequently present. The most common organ systems involved are the kidneys, musculoskeletal system, and peripheral nervous system.

Medicine

Immunology and Rheumatology

A 55-year-old woman presents to the clinic for evaluation of several weeks of fever, abdominal pain, weight loss, and lack of energy. Three days prior to the assessment, she developed a left foot drop. Her blood pressure is 160/90 mm Hg, pulse 80/min, and the physical examination confirms left peroneal nerve damage and a bilateral sensory neuropathy in both legs. There are no skin rashes. Laboratory evaluation reveals an ESR of 105 mm/h, WBC of 14,000/mL, and negative serologic tests for antineutrophil cytoplasmic antibody (ANCA) and ANA. The eosinophil count is normal, and urinalysis is negative for casts, protein, and red cells. A clinical diagnosis of polyarteritis nodosa is made.

Which of the following is the most likely mechanism for renal injury in this condition?

D. necrotizing vasculitis of vessels

67e3c03b-bb69-4b6f-ad91-d13e742aa65b

A 50-year-old lady presented with a 3-month history of pain in the lower third of the right thigh. There was no local swelling; tenderness was present on deep pressure. Plain X-rays showed an ill-defined intra medullary lesion with blotchy calcification at the lower end of the right femoral diaphysis, possibly enchondroma or chondrosarcoma. Sections showed a cailaginous tumor. Which of the following histological features (if seen) would be most helpful to differentiate the two tumours?

Focal necrosis and lobulation

Tumor permeation between bone trabecuae at periphery

Extensive myxoid change

High cellularity

1b

single

Ans. is 'b' i.e., Tumour permeation between bony trabeculae at periphery Chondroma (enchondroma) Vs Chondrosarcoma o The term chondroma is used for benign neoplasm composed of cailage. It is of two types : - i) Enchondroma : - Cailage tumors occuring within the bone. ii) Periosteal chondroma : - Cailage tumors occuring on the surface of the bone. o Chondrosarcoma is the malignant tumor of cailage. Growth potential of chondroma is limited and mostly remain stable, but they have a potential to undergo malignant (sarcomatous) change to become chondrosarcoma. lnfact, enchondromas are considered to be a low grade chondrosarcoma. o A painful chondroid neoplasm in a large bone in an adult patient can be either enchondroma or chondrosarcoma o The differentiation between a low grade chondrosarcoma and an enchondroma can be difficult. o To make distinction between these two neoplasms, one has to make use of Cytological changes Myxoid quality of stroma Permeative characteristics Among these permeation, maifested as marrow spaces filled with the neoplasm and entrapment of preexisting bony trabeuculae is the most impoant sign of malignancy.

Pathology

A 50-year-old lady presented with a 3-month history of pain in the lower third of the right thigh. There was no local swelling; tenderness was present on deep pressure. Plain X-rays showed an ill-defined intra medullary lesion with blotchy calcification at the lower end of the right femoral diaphysis, possibly enchondroma or chondrosarcoma. Sections showed a cailaginous tumor.

Which of the following histological features (if seen) would be most helpful to differentiate the two tumours?

B. Tumor permeation between bone trabecuae at periphery

552b8010-b294-44cf-b882-efc1d9d9011d

A 45-year-old woman has experienced worsening arthritis of her hands and feet for the past 15 years. On physical examination, there are marked deformities of the hands and feet, with ulnar deviation of the hands and swan-neck deformities of the fingers. Laboratory studies show an elevated level of rheumatoid factor. CBC shows hemoglobin, 11.6 g/dL; hematocrit, 34.8%; MCV, 87 mm3; platelet count, 268,000/ mm3; and WBC count, 6800/ mm3. There is a normal serum haptoglobin level, serum iron concentration of 20 mg/ dL, total iron-binding capacity of 195 mg/dL, percent saturation of 10.2, and serum ferritin concentration of 317 ng/mL. No fibrin split products are detected. The reticulocyte concentration is 1.1%. What is the most likely mechanism underlying this patient's hematologic abnormalities?

Autoantibodies against RBC membranes

Impaired synthesis of b-globin chains

Inadequate usage of stored iron

Sequestration of RBCs in splenic sinusoids

2c

single

The iron concentration and iron-binding capacity are low; however, in contrast to the finding in anemia of iron deficiency, the serum ferritin level is increased. This increase is typical of anemia of chronic disease. In this case, the chronic disease is rheumatoid arthritis. Underlying chronic inflammatory or neoplastic diseases increases the secretion of cytokines such as interleukin-1, tumor necrosis factor, and interferon-g. These cytokines promote the sequestration of iron in storage compartments and depress erythropoietin production. Autoantibody hemolytic anemias occur in several autoimmune diseases, such as systemic lupus erythematosus, but not usually in patients with rheumatoid arthritis, as in this case. Normal serum haptoglobin rules out intravascular hemolysis; iron is recycled at a rapid rate. Impaired synthesis of b-globin chains gives rise to b-thalassemia, also characterized by hemolysis. Complement lysis is enhanced in paroxysmal nocturnal hemoglobinuria, which results from mutations in the PIGA gene. Patients with this disorder have a history of infections. Sequestration of RBCs in the spleen occur when RBC membranes are abnormal, as in hereditary spherocytosis or sickle cell anemia, or RBCs are coated by antibodies, as in autoimmune hemolytic anemias. Metastases are space-occupying lesions (myelophthisic process) that can lead to leukoerythroblastosis, with nucleated RBCs and immature WBCs appearing on the peripheral blood smear.

Pathology

Blood

A 45-year-old woman has experienced worsening arthritis of her hands and feet for the past 15 years. On physical examination, there are marked deformities of the hands and feet, with ulnar deviation of the hands and swan-neck deformities of the fingers. Laboratory studies show an elevated level of rheumatoid factor. CBC shows hemoglobin, 11.6 g/dL; hematocrit, 34.8%; MCV, 87 mm3; platelet count, 268,000/ mm3; and WBC count, 6800/ mm3. There is a normal serum haptoglobin level, serum iron concentration of 20 mg/ dL, total iron-binding capacity of 195 mg/dL, percent saturation of 10.2, and serum ferritin concentration of 317 ng/mL. No fibrin split products are detected. The reticulocyte concentration is 1.1%.

What is the most likely mechanism underlying this patient's hematologic abnormalities?

C. Inadequate usage of stored iron

d41b6154-c6db-4a8e-95c5-5bc24b4de39e

A 30-year-old theatre actress developed few wrinkles on the face. The treating physician advised her to have local injections of a drug. This drug is also indicated in cervical dystonia and other spastic disorders like cerebral palsy. Very recently, it has also been approved for prophylaxis of migraine. The physician warned of the drug to cause dry mouth and blurring of vision. The actress searched the compound on internet and found the site of action of the drug as shown in the Figure below.Which of the following drug is being talked about?

Hemicholinium

Vesamicol

Botulinum toxin

Physostigmine

2c

single

Ans. (C) Botulinum toxin(Ref: KK Sharma 2/e p217)As shown in the diagram, the drug is inhibiting the exocytosis of ACh. Botulinum toxin act by this mechanism. Other features pointing towards botulinum toxin are:Anticholinergic adverse effects (dry mouth, blurring of vision)Use in wrinkles, spastic disorders, prophylaxis of migraine.

Pharmacology

A.N.S.

A 30-year-old theatre actress developed few wrinkles on the face. The treating physician advised her to have local injections of a drug. This drug is also indicated in cervical dystonia and other spastic disorders like cerebral palsy. Very recently, it has also been approved for prophylaxis of migraine. The physician warned of the drug to cause dry mouth and blurring of vision.

The actress searched the compound on internet and found the site of action of the drug as shown in the Figure below.Which of the following drug is being talked about?

C. Botulinum toxin

f6b8f3c0-c8da-4a7c-a43c-8d6d5624892c

A 41 y/o male presents with complaints of inability to achieve proper erections during sexual intercourse. He repos that there were no abnormalities till last month, when on one occasion he tried having sex with wife while he was drunk. He was not able to achieve a proper erection at that time and since than, on four other occasions , he was not able to have an erection during attempted sex. He repos that his morning erections are fine and erection during masturbation was also normal. He is a diabetic, and the blood repos show, FBS-103 mg/dl, HbA1C- 6.6. His BP was 138/88 mm Hg What is the likely cause of erection disturbances?

Anxiety

Diabetes

Alcohol

Hypeension

0a

single

History of sudden onset, normal morning erections and normal erection during masturbation is suggestive of Psychogenic Erectile dysfunction. The cause is usually performance anxiety. To differentiate between psychogenic and organic erectile dysfunction best test is ask for nocturnal / early morning erections as patient is relaxed in these conditions. So in the above case it rules out all organic causes.

Psychiatry

Sexual Disorders

A 41 y/o male presents with complaints of inability to achieve proper erections during sexual intercourse. He repos that there were no abnormalities till last month, when on one occasion he tried having sex with wife while he was drunk. He was not able to achieve a proper erection at that time and since than, on four other occasions , he was not able to have an erection during attempted sex. He repos that his morning erections are fine and erection during masturbation was also normal. He is a diabetic, and the blood repos show, FBS-103 mg/dl, HbA1C- 6.6.

His BP was 138/88 mm Hg What is the likely cause of erection disturbances?

A. Anxiety

d154cc81-3c5e-459e-a6bf-39c0bc7fe923

A 37 year old woman has cyclical premenstrual pain. Her breasts have a "lumpy-bumpy" texture on palpation. A biopsy is performed. The histopathologic features include small cysts lined by epithelial cells with apocrine metaplasia, calcium deposits, areas of fibrosis, increased number of acini (adenosis), and foci of florid hyperplasia of ductal epithelium. Which of these changes increase the risk of breast cancer?

Adenosis

Apocrine metaplasia

Calcium deposits

Epithelial hyperplasia

3d

multi

Fibrocystic changes usually come to clinical attention by causing pain(often cyclical, in premenstrual phase), palpable lumps, or mammographic densities and calcifications. A "lumpy-bumpy" texture is caused by cysts and fibrosis. Fibrocystic changes, per se, do not increase the risk of developing breast cancer, unless there are associated proliferative changes. Epithelial hyperplasia is defined as an increase in the number of epithelial cell layers in the ductal epithelium. Florid epithelial hyperplasia leads to an increased risk of developing carcinoma, especially if there is associated cellular atypia (atypical ductal hyperplasia). Adenosis refers to an increase in the number of acini and can be observed in fibrocystic changes as well as in other breast conditions, such as sclerosing adenosis. Physiologic adenosis is pa of the changes that occur during pregnancy. Adenosis is not associated with increased risk of breast cancer, except as a component of sclerosing adenosis. Apocrine metaplasia describes a benign change of breast epithelial cells that come to resemble the apocrine epithelium of sweat glands. Apocrine cells have abundant granular eosinophilic cytoplasm. Apocrine metaplasia is seen frequently in fibrocystic changes, as well as in normal breast. Calcium deposition is a nonspecific finding that may occur in a number of both benign and malignant breast changes, including fibrocystic changes, ductal carcinoma in situ, and invasive carcinoma. Calcification is not clinically significant except for its diagnostic value. Mammographic detection of calcium may serve to guide biopsy procedures. Ref: Hunt K.K., Newman L.A., Copeland E.M., Bland K.I. (2010). Chapter 17. The Breast. In F.C. Brunicardi, D.K. Andersen, T.R. Billiar, D.L. Dunn, J.G. Hunter, J.B. Matthews, R.E. Pollock (Eds), Schwaz's Principles of Surgery, 9e.

Surgery

A 37 year old woman has cyclical premenstrual pain. Her breasts have a "lumpy-bumpy" texture on palpation. A biopsy is performed. The histopathologic features include small cysts lined by epithelial cells with apocrine metaplasia, calcium deposits, areas of fibrosis, increased number of acini (adenosis), and foci of florid hyperplasia of ductal epithelium.

Which of these changes increase the risk of breast cancer?

D. Epithelial hyperplasia

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A 67-year-old man with an 18-year history of type 2 diabetes mellitus presents for a routine physical examination. His temperature is 36.9 C (98.5 F), his blood pressure is 158/98 mm Hg and his pulse is 82/minute and regular. On examination, the physician notes a non tender, pulsatile, mass in the mid-abdomen. A plain abdominal x-ray film with the patient in the lateral position reveals spotty calcification of a markedly dilated abdominal aoic walI. Which of the following physiologic observations helps to account for the fact that 75% of the aneurysms of this patient's type are found in the abdomen and only 25% principally involve the thorax?

Diastolic pressure is greater in the abdominal aoa in the supine position

Negative intrathoracic pressure reduces aoic wall tension in the thorax

The average blood flow in the abdominal aoa is greater than that in the thoracic aoa

The average blood pressure in the abdominal aoa is higher than that in the thoracic aoa

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Increased blood pressure is a strong risk factor for atherosclerosis, and humans pay a price for their erect sitting and standing postures. In these postures, the abdominal aoa experiences the weight of a column of blood added to the pressure produced by the hea. In the supine posture, the pressures in the thoracic and abdominal aoa are similar. So, if an average daily pressure is taken, the abdominal aoa tends to have a significantly higher pressure than does the thoracic aoa. Diastolic pressure is actually greater in the thoracic aoa compared to the abdominal aoa in the supine position. However, the systolic blood pressure is greater in the abdominal aoa. A negative intrathoracic pressure would tend to increase transmural pressure across the wall of the thoracic aoa, and thereby increase wall tension and promote the development of aneurysms. Blood flow in the abdominal aoa is less than that in the thoracic aoa, because some blood leaves the aoa through its thoracic branches. Ref: Creager M.A., Loscalzo J. (2012). Chapter 248. Diseases of the Aoa. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds),Harrison's Principles of Internal Medicine, 18e.

Physiology

A 67-year-old man with an 18-year history of type 2 diabetes mellitus presents for a routine physical examination. His temperature is 36.9 C (98.5 F), his blood pressure is 158/98 mm Hg and his pulse is 82/minute and regular. On examination, the physician notes a non tender, pulsatile, mass in the mid-abdomen. A plain abdominal x-ray film with the patient in the lateral position reveals spotty calcification of a markedly dilated abdominal aoic walI.

Which of the following physiologic observations helps to account for the fact that 75% of the aneurysms of this patient's type are found in the abdomen and only 25% principally involve the thorax?

D. The average blood pressure in the abdominal aoa is higher than that in the thoracic aoa

9830e794-73a8-4129-8e06-38034a902855

An 8-year old child was brought to you for a high grade fever of 3 days duration with vomiting headache and decreased responsiveness. Examination revealed a distressed child , in shock with maculopapular and ecchymotic rashes on the skin with bleeding from venepuncture sites and hepatomegly. Investigation showed PCV =65% WBC 4000 platelet 25000 & mildly prolonged PT. CXR revealing left sided pleural effusion. Diagnosis is ?

Meningococcemia with DIC

Dengue heamorrhagic fever

Leptosporisis

Complicated malaria

1b

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Generalized body aches, high grade fever followed by clinical deterioration and bleeding diathesis with raised hematocrit (65%) and low platelet and leucopenia strongly suggest dengue haemorrhagic fever, although meningococcemia is always a close diagnosis and many times requires serological confirmation. Complicated malaria usually has anemia and splenomegaly Leptospirosis may occur in icteric as well as nonicteric form. In nonicteric form shock and skin rashes are uncommon. Prominent features are severe myalgias and exquisite tenderness.

Surgery

An 8-year old child was brought to you for a high grade fever of 3 days duration with vomiting headache and decreased responsiveness. Examination revealed a distressed child , in shock with maculopapular and ecchymotic rashes on the skin with bleeding from venepuncture sites and hepatomegly. Investigation showed PCV =65% WBC 4000 platelet 25000 & mildly prolonged PT. CXR revealing left sided pleural effusion.

Diagnosis is ?

B. Dengue heamorrhagic fever

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Mother of a 10 year old male child is concerned that he appears smaller than other boys of his age. Intraoral examination reveals that the patient is in his mixed dentition stage. Permanent incisors and 1st molars are found to be present. Mandibular permanent canines are erupting and maxillary deciduous molars are present. Physical growth status of a child may vary from the chronologic age in many children, but it correlates well with skeletal age. Which is the best radiographic analysis to ascertain the skeletal age of this patient?

Witt’s analysis

Demirjian’s system

Steiner’s analysis

Fishman’s index

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FISH MAN'S SKELETAL MATURITY INDICATORS Keonord S Fishman proposed a system for evaluation for skeletal maturation in 1982, Fishman made use of four anatomical sites located on the thumb, third finger, fifth finger and radius.  Note: Witt’s and Steiner’s analysis are cephalometric analysis and not a method to analyze skeletal age.  Demirjian’s system is a method of analysing dental age and not skeletal age.

Dental

Mother of a 10 year old male child is concerned that he appears smaller than other boys of his age. Intraoral examination reveals that the patient is in his mixed dentition stage. Permanent incisors and 1st molars are found to be present. Mandibular permanent canines are erupting and maxillary deciduous molars are present. Physical growth status of a child may vary from the chronologic age in many children, but it correlates well with skeletal age.

Which is the best radiographic analysis to ascertain the skeletal age of this patient?

D. Fishman’s index

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A 4-month-old infant has undergone surgical treatment for meningo myelo encephalocele. A CT tomogram of head was made immediately after birth (see Figure below). At birth, an operation was carried out in the posterior cranial fossa to partially replace brain cerebellar contents to an intracranial position. In investigations for progressive hydrocephalus, it is noted that there is herniation of the cerebellar tonsils through the foramen magnum, and a diagnosis of Arnold-Chiari syndrome is established. This syndrome may also include which of the following?Tomogram from CT head taken 4 months previously (immediately after birth). Opening in the posterior cranial fossa showing brain and meninges protruding into sac (axial view).

Fusion of the frontal lobes

Fusion of the temporal, parietal, and occipital lobes

Abnormal elongation of the medulla and lower cranial nerves

Partial or complete absence of the pituitary gland

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Abnormal elongation of the medulla and lower cranial nerves may be evident in Arnold- Chiari syndrome. Additional features include fusion of the corpora quadrigemina, leading to a "beaked" tectum; partial or complete absence of the corpus callosum; and microgyria. The corpora quadragemina are relay stations for hearing (inferior corpora quadragemina) and the light reflex (superior copora quadragemina), and they form the posterior surface of the midbrain.

Surgery

Nervous System

A 4-month-old infant has undergone surgical treatment for meningo myelo encephalocele. A CT tomogram of head was made immediately after birth (see Figure below). At birth, an operation was carried out in the posterior cranial fossa to partially replace brain cerebellar contents to an intracranial position. In investigations for progressive hydrocephalus, it is noted that there is herniation of the cerebellar tonsils through the foramen magnum, and a diagnosis of Arnold-Chiari syndrome is established. This syndrome may also include which of the following?Tomogram from CT head taken 4 months previously (immediately after birth).

Opening in the posterior cranial fossa showing brain and meninges protruding into sac (axial view).

C. Abnormal elongation of the medulla and lower cranial nerves

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An acutely ill 18-year-old female is brought to the emergency depament. The patient is febrile and markedly hypotensive, and her mental status is obtunded. Numerous petechial and purpuric hemorrhages are scattered over the trunk, and aspiration of a lesion reveals neutrophils engulfing gram-negative diplococci. Serum sodium is markedly decreased, and serum potassium is increased. Coagulation testing reveals increased prothrombin time, activated paial thromboplastin time, and fibrin-fibrinogen split products. Which of the following is most likely?

Conn syndrome

Hyperprolactinoma

Neuroblastoma

Waterhouse-Friderichsen syndrome

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- Given pt is presenting with signs & symptoms of Waterhouse Friderichsen syndrome, a devastating consequence of disseminated meningococcal infection. - This disease is characterized by hemorrhagic destruction of adrenals complicated by disseminated intravascular coagulation.

Pathology

Adrenal Gland

An acutely ill 18-year-old female is brought to the emergency depament. The patient is febrile and markedly hypotensive, and her mental status is obtunded. Numerous petechial and purpuric hemorrhages are scattered over the trunk, and aspiration of a lesion reveals neutrophils engulfing gram-negative diplococci. Serum sodium is markedly decreased, and serum potassium is increased. Coagulation testing reveals increased prothrombin time, activated paial thromboplastin time, and fibrin-fibrinogen split products.

Which of the following is most likely?

D. Waterhouse-Friderichsen syndrome

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A 45 year old man presents to a physician with back pain and facial pain. Physical examination demonstrates coarse facial features and kyphosis. Laboratory examination is remarkable for elevated alkaline phosphatase. .X-ray studies demonstrate skull thickening with narrowing of foramina, and bowing of the femur and tibia. Bone biopsy reveals a mosaic pattern of bone spicules with prominent osteoid seams. Which of the following neoplasms occurs at an increased frequency in patients with this disorder?

Astrocytoma

Hodgkin's lymphoma

Meningioma

Osteosarcoma

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The phrase "mosaic pattern" of newly formed woven bone is a specific tip-off for Paget's disease of bone, and is not seen in other bone conditions. The clinical and radiologic presentation are typical; an increased hat size may also be a clue. In its early stages, Paget's disease is characterised by osteolysis, producing patchwork areas of bone resorption with bizarre, large osteoclasts. In the middle stage of the disease, secondary osteoblastic activity compensates with new bone formation, producing the mosaic pattern. In late Paget's, the bones are dense and osteosclerotic. Paget's disease is suspected to be related to prior viral infection, but the cause remains mysterious. Complications include myelophthisic anemia, high output cardiac failure, pain secondary to nerve compression, deformities secondary to skeletal changes, and in about 1% of patients, osteosarcoma or other sarcoma, typically involving the jaw, pelvis, or femur. An increased incidence of astrocytomas is associated with tuberous sclerosis.Hodgkin's lymphoma is usually a disease of young adults, although older patients may have the lymphocyte-depleted form.Meningiomas are mostly benign tumors that affect adults, especially females. There may be an association with breast cancer, possibly related to high estrogen states. Ref: Wyatt C., Butterwoh IV J.F., Moos P.J., Mackey D.C., Brown T.G. (2008). Chapter 19. Pathology of the Bones and Joints. In C. Wyatt, J.F. Butterwoh IV, P.J. Moos, D.C. Mackey, T.G. Brown (Eds), Pathology: The Big Picture.

Pathology

A 45 year old man presents to a physician with back pain and facial pain. Physical examination demonstrates coarse facial features and kyphosis. Laboratory examination is remarkable for elevated alkaline phosphatase. .X-ray studies demonstrate skull thickening with narrowing of foramina, and bowing of the femur and tibia. Bone biopsy reveals a mosaic pattern of bone spicules with prominent osteoid seams.

Which of the following neoplasms occurs at an increased frequency in patients with this disorder?

D. Osteosarcoma

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A 5-year-old boy is brought in by his mother because of petechial bleeding and bruises on his torso and limbs. The child doesn't have any other signs or symptoms and doesn't appear to be ill. Mother reports gastrointestinal infection several weeks prior to petechiae and bruising. CBC shows thrombocytopenia (<20xl09/L). Other parameters of CBC are within expected range for age. Also, PT, PTT and metabolic panels are all within referent range. What is the expected outcome of this blood disorder?

Complete resolution is expected

Survival rate is up to 70% depending on risk stratification

Lifelong disease dependent on factor VIII substitution

Lifelong disease dependent on factor IX substitution

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Answer: a) Complete resolution is expectedDiagnosis appears to be UP. It usually follows viral infections in children aged 2 to 5 years and is of autoimmune origin. It is self-limiting in most cases and resolves completely in 6 weeks to 6 months in 80% of children.IMMUNE THROMBOCYTOPENIC PURPURA (ITP)CHRONIC ITP* More common form* Most common in adult women < 40 yrs (female-to-male ratio -3:1)* Can be associated with connective tissue diseases, lymphoproliferative disease, medications and infections (hepatitis C virus and HIV)* Auto-antibodies (mostly IgG) against platelet membrane glycoproteins (llb-llla or Ib-IX)* Spleen is the major site of destruction of platelets* Petechiae, ecchymosis, easy bruising, bleeding from nose and gums, menorrhagia* Splenomegaly & lymphadenopathy - rare* Subarachnoid and intracerebral hemorrhage are rare but fatal complications* Wet purpura (blood blisters in the mouth)* Peripheral smear shows abnormally large platelets (megathrombocytes) & low platelet count* Bone marrow - hypercellular & increased megakaryocytes* Bleeding time prolonged* PT and PTT are normalTreatment* Initial treatment for patients without significant bleeding: Glucocorticoids (Prednisone or Dexa) +- IV immunoglobulin or anti-D or anti-CD20 antibody (rituximab)* Two-thirds of patients respond to initial treatment, but most people relapse following reduction of the corticosteroid dose* Patients with severe ITP and/or symptoms of bleeding: hospital admission and combined therapy (high-dose glucocorticoids + IVIgG or anti-RhO (D) therapy)* Relapse: Splenectomy* Refractory cases: Thrombopoietin receptor agonists (Romiplostim and eltrombopag) or splenectomyACUTE ITP* Disease of childhood* Affects both sexes equally* Onset is abrupt* Many cases are preceded by viral illness* The usual interval between the infection and onset of purpura is 2 weeks* Self-limited, spontaneous resolution in 6 months* 20% children (those without viral prodrome) have persistent low platelet counts after 6 months

Pathology

Blood

A 5-year-old boy is brought in by his mother because of petechial bleeding and bruises on his torso and limbs. The child doesn't have any other signs or symptoms and doesn't appear to be ill. Mother reports gastrointestinal infection several weeks prior to petechiae and bruising. CBC shows thrombocytopenia (<20xl09/L). Other parameters of CBC are within expected range for age. Also, PT, PTT and metabolic panels are all within referent range.

What is the expected outcome of this blood disorder?

A. Complete resolution is expected

3b6e62a4-8100-433a-9f62-d95802c0729b

A 25-year-old woman complains of persistent bleeding for 5 days after a dental extraction. She has noticed easy bruisability since childhood and was given a blood transfusion at age 17 because of prolonged bleeding after an apparently minor cut. She denies ecchymoses or bleeding into joints. Her father has noticed similar symptoms but has not sought medical care. Physical examination is normal except for mild oozing from the dental site. She does not have splenomegaly or enlarged lymph nodes. Her CBC is normal, with a platelet count of 230,000. Her prothrombin time is normal, but the partial thromboplastin time is mildly prolonged. The bleeding time is 12 minutes (normal 3-9 minutes). What is most appropriate way to control her bleeding?

Factor VIII concentrate

Fresh frozen plasma

Desmopressin (DDAVP)

Whole blood transfusion

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This woman's lifelong history of excessive bleeding suggests an inherited bleeding problem, as does the positive family history. The prolonged PTT indicates a deficiency of factors VIII, IX, XI, or XII, but the commonest of these deficiencies (classic hemophilia A and Christmas disease, or hemophilia B) are vanishingly rare in women. Furthermore, the continued oozing from dental sites and the absence of ecchymoses or hemarthroses suggest a platelet function disorder, as does the prolonged bleeding time. Von Willebrand disease is an autosomal dominant condition that leads to both platelet and factor VIII dysfunction and is the likeliest diagnosis in this patient. Although factor VIII concentrates can be used for life-threatening bleeding, most will respond to desmopressin, which raises the von Willebrand factor level in the most common form (the so- called type 1 form) of this disease. Mild von Willebrand disease is fairly common (1 in 250 individuals). Fresh frozen plasma and whole blood are much less effective ways to deliver factor YIII. Platelet transfusion would not be as effective as correction of the von Willebrand factor level.

Medicine

Oncology

A 25-year-old woman complains of persistent bleeding for 5 days after a dental extraction. She has noticed easy bruisability since childhood and was given a blood transfusion at age 17 because of prolonged bleeding after an apparently minor cut. She denies ecchymoses or bleeding into joints. Her father has noticed similar symptoms but has not sought medical care. Physical examination is normal except for mild oozing from the dental site. She does not have splenomegaly or enlarged lymph nodes. Her CBC is normal, with a platelet count of 230,000. Her prothrombin time is normal, but the partial thromboplastin time is mildly prolonged. The bleeding time is 12 minutes (normal 3-9 minutes).

What is most appropriate way to control her bleeding?

C. Desmopressin (DDAVP)

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A 53 year old male presents with an enlarged supraclavicular lymph node, and examination reveals enlargement of the Waldeyer ring of oropharyngeal lymphoid tissue. There is no hepatosplenornegaly. Lymph node biopsy reveals replacements by a monomorphous population of large lymphoid cells with enlarged nuclei and prominent nucleoli. The CBC is normal except for finding of mild anemia. Immunohistochemical staining and flow cytometry of the node reveals that most lymphoid cells are CDl9 CD10=,CD3-, CD15-, and negative (TdT). These findings are most consistent with a diagnosis of

Chronic lymphadenitis

Diffuse large B-cell lymphoma

Hodgkin disease

Lymphoblastic lymphoma

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Diffuse large B-cell lymphoma occurs in older individuals and frequently presents as localized disease with extranodal involvement, paicularly of the Waldeyer ring. The staining pattern indicates a B-cell proliferation (CD19, CD1O). T-cell (CD3) and monocytic (CD15) markers are absent, TdT can be expressed in B- lineage cells at an earlier stage of maturation. Small lymphocytic lymphoma is also a B-cell neoplasm, but it manifests with widespread lymphadenopathy, liver and spleen enlargement, and lymphocytosis, Lymphoblastic lymphoma is a T-cell neoplasm that occurs typically in the mediastinum of children. Reed-Stem berg cells characterize Hodgkin disease. In chronic lymphaderiitis, the lymph node has many cell types-macrophages, lymphocytes, and plasma cells. A monomorphous infiltrate is typical of non-Hodgkin lymphomas.

Medicine

A 53 year old male presents with an enlarged supraclavicular lymph node, and examination reveals enlargement of the Waldeyer ring of oropharyngeal lymphoid tissue. There is no hepatosplenornegaly. Lymph node biopsy reveals replacements by a monomorphous population of large lymphoid cells with enlarged nuclei and prominent nucleoli. The CBC is normal except for finding of mild anemia. Immunohistochemical staining and flow cytometry of the node reveals that most lymphoid cells are CDl9 CD10=,CD3-, CD15-, and negative (TdT).

These findings are most consistent with a diagnosis of

B. Diffuse large B-cell lymphoma

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A 22-year-old man has a sudden loss of vision in the right eye. On physical examination, there is a subluxation of the right crystalline lens. On auscultation of the chest, a midsystolic click is audible. An echocardiogram shows a floppy mitral valve and dilated aortic arch. The patient's brother and his cousin are similarly affected. He is prescribed a beta-blocker. A genetic defect involving which of the following substances is most likely to be present in this patient?

Collagen

Dystrophin

Fibrillin-1

NF1 protein

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Marfan syndrome is an autosomal dominant condition that is most often caused by qualitative defects in fibrillin from missense mutations in the fibrillin (FBN1) gene. An abnormal collagen gene can cause osteogenesis imperfecta and Ehlers-Danlos syndrome. Genetic mutations in the dystrophin gene are involved in Duchenne and Becker muscular dystrophies. The NF1 protein is abnormal in neurofibromatosis type 1. Disordered spectrin causes hereditary spherocytosis.

Pathology

Genetics

A 22-year-old man has a sudden loss of vision in the right eye. On physical examination, there is a subluxation of the right crystalline lens. On auscultation of the chest, a midsystolic click is audible. An echocardiogram shows a floppy mitral valve and dilated aortic arch. The patient's brother and his cousin are similarly affected. He is prescribed a beta-blocker.

A genetic defect involving which of the following substances is most likely to be present in this patient?

C. Fibrillin-1