intravascular large b - cell lymphoma ( ivlbcl ) is a rare subtype of extranodal diffuse large b - cell lymphoma . ivlbcl is characterized by the presence of lymphoma cells only in the lumina of small vessels , particularly capillaries.1 clinical presentation is extremely heterogeneous , ranging from monosymptomatic forms such as fever , pain or local symptoms , to rapidly progressing multiorgan failure . the atypical characteristics of ivlbcl , including the absence of marked lymphadenopathy and aggressive clinical behavior , tend to result in the delay of timely and accurate diagnosis . thus , the prognosis of ivlbcl is extremely poor.2 median survival ranges from a few months without treatment to several years with rituximab - containing polychemotherapy.3 microscopic findings of primary and secondary hepatic lymphomas ( non - hodgkin or hodgkin lymphoma ) include infiltration of lymphoid tissue that is composed of small lymphocytes with oval and irregular nuclei and clear cytoplasm in the liver parenchyma.4 in cases of hodgkin or non - hodgkin lymphomas , the ct appearance of hepatic involvement can be divided into three categories : solitary hepatic mass lesions ranging from less than 1 cm to approximately 10 cm , multifocal hepatic lesions similar in appearance to metastatic disease or multifocal hepatoma , and diffuse hepatic involvement with lesions that often have the same attenuation as hepatic parenchyma.5 on mri , lymphomatous lesions usually have low signal intensity on t1-weighted images and variable signal intensity on t2-weighted images . the lesions usually show mild enhancement after gadolinium chelate administration.6 the literature contains little about state - of - the art imaging findings for ivlbcl . we present a case of hepatic involvement of ivlbcl with ct and mri findings that might be helpful for ivlbcl diagnosis . we describe an ivlbcl patient who presented with dyspnea , fever and elevated liver enzyme levels . a 58-year - old man who was previously healthy presented with six - week history of dyspnea and a one - day history of fever . body temperature at presentation was 38.8. laboratory studies revealed a white blood cell count of 10.2  10/l with hemoglobin 8.5 g / dl . total bilirubin level was 2.6 mg / dl ( normal range , 0.2 - 1.5 mg / dl ) . liver enzyme levels were elevated with an alanine aminotransferase 72 u / l ( normal range , 0 - 40 u / l ) , aspartate aminotransferase 151 u / l ( normal range , 0 - 40 u / l ) , and alkaline phosphatase 155 u / l ( normal range , 53 - 128 u / l ) . lactate dehydrogenase ( ldh ) was 6151 iu / l ( normal range , 240 - 480 erythrocyte sedimentation rate ( esr ) was 12 mm / hr ( normal range , 0 - 22 mm / hr ) . to evaluate the cause of dyspnea , chest ct was performed and there was no demonstrable abnormality to explain the symptom . on laboratory findings patient showed anemia which was suspected as the cause of dyspnea . the patient was known to have a hemangioma in liver segment vii that had been incidentally found in 2004 and had grown from 1.4 cm to 3.0 cm on follow - up ultrasonography . therefore , imaging studies were performed to further evaluate the hepatic hemangioma and investigate the cause of the elevated liver enzymes . contrast - enhanced dynamic ct of the liver showed multiple ill - defined non - enhancing areas in the hepatic lobes . lesions appeared as low - attenuating areas of geographic pattern compared with normal - appearing parenchyma during all three phases . 2 ) revealed multiple mass - like lesions with ill - defined margins and homogeneous hypointensity on pre - contrast t1-weighted images and homogeneous hyperintensity on pre - contrast t2-weighted images . the lesions showed little contrast enhancement during dynamic scans and diffusion restriction on diffusion - weighted images . hepatic vascular structures within the lesions were normal without mass effects , similar to the ct images . ultrasonography - guided percutaneous biopsy was performed under the impression of a suspected unusual malignant tumor . microscopic analysis showed abundant tumor cells filling the hepatic sinusoids and tumor cells positive for cd20 ( fig . 3 ) . the patient was found to have no abnormality in central nerve system , skin , and other parts of the abdomen on physical examination and imaging studies . immediately after diagnosis , chemotherapy with cyclophosphamide , doxorubicin , vincristine , prednisone and monoclonal antibody rituximab ( r - chop ) was initiated , and after a few days , fever began to subside . despite its recognition as a distinct disease , no large studies of ivlbcl have been reported.7 skin and the central nervous system are the most commonly involved sites and ivlbcl of the liver is very rare . to our knowledge intravascular large b - cell lymphoma , a subtype of diffuse large b - cell lymphoma in the who classification , is a very rare non - hodgkin 's lymphoma that is estimated to be < 1% of all lymphomas . ivlbcl is characterized by nearly exclusive intravascular neoplastic proliferation of b lymphocytes , usually without involvement of lymphoid tissues and peripheral blood.8 ivlbcl has been described in patients from 34 - 90 years of age , with a median age of 70 years . it occurs equally in women and men and usually shows rapid progression and short survival , with only transient remission.1 ivlbcl can involve any organ and is usually widely disseminated at the time of diagnosis . because of its distribution , ivlbcl can present with a wide range of signs and symptoms that are generalized and nonspecific ( e.g. , b symptoms , cytopenias ) , or localized and related to the involved organ ( e.g. , cutaneous lesions , neurologic deficits , hepatic or renal insufficiency ) . these findings result in a variety of possible differential diagnoses including autoimmune , infectious , and neoplastic . due to the lack of lymph node involvement or formation of mass lesions , the clinical picture is further complicated and the ability to make a prompt diagnosis is undermined.9 laboratory findings are not specific but indicative for suspected ivlbcl . increased serum ldh and 2-microglobulin levels are observed in more than 80% to 90% of ivlbcl patients . altered hepatic , renal , or thyroid function tests are observed in 15% to 20% . these functional tests should be always included in staging work - up for ivlbcl.10 the predominant microscopic finding of ivlbcl is atypical blastoid cells with multiple mitoses that exclusively proliferate within vascular and sinusoidal structures of lymph nodes , liver , lung or bone marrow . immunhistochemical staining is positive for the bcell markers cd20 and cd79a in the absence of staining for t - cell markers . occasionally , cd5 can be expressed as well.8 radiologic findings in the case presented here revealed low attenuating infiltrative mass lesions with no enhancement on dynamic ct images . the lesion showed homogeneous hypointensity on t1-weighted images , homogeneous hyperintensity on t2-weighted images and diffusion restriction . both primary and secondary hepatic lymphomas ( non - hodgkin lymphoma or hodgkin disease ) tend to show mild enhancement after contrast administration including homogeneous and rim - like pattern . in addition , associated lymphadenopathy is noted in most patients with primary or secondary hepatic lymphomas.11 the patient in our case had multiple , ill - defined nonenhancing areas in the hepatic lobes that had a homogeneous appearance . these findings could aid in the differential diagnosis of ivbcl from other forms of primary and secondary hepatic lymphomas . other liver diseases of diffuse pattern including malignant or non - malignant infiltrative diseases can be considered as differential diagnosis . the most common malignant lesion is hepatic metastasis of diffuse infiltrative pattern that can be differentiated from ivbcl on the bases of clinical context and imaging findings . hemochromatosis typically shows diffuse high attenuation at ct and hypointensity at t2 weighted mri whereas ivbcl shows low attenuation on ct and hyperintensity on t2 weighted image . an anthracycline - based chemotherapy regimen such as r - chop is recommended in cases with a b - cell immunophenotype.1 we presented a rare case of ivbcl of the liver without known radiologic features . however , when a patient presents with fever of unknown origin and elevated liver enzymes with infiltrative mass lesions with no enhancement on liver ct or mri , as in our case , ivbcl involvement of the liver should be considered for prompt diagnosis and treatment .
intravascular large b - cell lymphoma ( ivlbcl ) is a rare subtype of extranodal diffuse large b - cell lymphoma that most commonly involves the central nervous system and skin . to our knowledge , 
 no state - of - the art imaging findings have been reported for hepatic ivlbcl in the english literature . 
 we report the first case of hepatic involvement of ivlbcl along with a literature review .