<prompt> Hemoglobin electrophoresis in SC A sickle cell anemiHbAHbFHbA2None  <response>: Ans. a Hb A Ref. William haemat 7thpg. 670,678; Wintrobes haemat 11th pg. 1288.Williams 680 ... Because there are no p-polypeptide chain genes, patients with SCA have no NORMAL Hb A.Electrophoresis in Sickle cell anaemia Hb S -50 Hb F -Variable Hb A2 -Normal Hb A - AbsentSummary: Sickle cell anaemia HbS point mutation causes a single amino acid replace in a chain substitution of glutamic acid with valine at position 6. Pathogenesis: low O2, dehydration, or acidosis precipitates sickling deoxygenated HbS polymerizes, which results in anemia and vaso-occlusive disease. Newborns are initially asymptomatic because of HbF andHbS. In SC A, mean MCHC Increase, Hb is between 5-10 g Heterozygotes sickle cell trait have resistance to malaria. Crew cut on skull x-ray due to marrow expansion fromerythropoiesis also in thalassemias. Sickling is seen only when sickle cells are present by 50 Complications in sickle cell disease SS: Aplastic crisis due to parvovirus B19; Autosplenectomy Howell-Jolly bodies risk of infection with encapsulated organisms; Splenic sequestration crisis; Salmonella osteomyelitis; Painful crisis vaso-occlusive: dactylitis; acute chest syndrome most common cause of death in adults, avascular necrosis, stroke; Renal papillary necrosis due to low O2 in papilla; also seen in heterozygotes and microhematuria medullary infarcts. Diagnosis: hemoglobin electrophoresis. Treatment: a. To increase HbF with drugs like: 5- azitidine and Hydroxyurea HbF. b. To increase water content of RBCs or to prevent dehydration of cells: Gordos channel inhibitors, Cotrimoxazole and Mg. 