<unk>  is a genetically transmitted form of hemolysis, characterized by hemolytic anemia, jaundice and splenomegaly. It was first described by Vanlair and Masius in 1871, where they described chronically icteric patients who had no bile in urine, no evidence of liver disease and often splenomegaly and family history of jaundice.<unk>  is classified in 05 subtypes on the basis of underlying protein defect including; ankyrin1, spectrin beta chain (erythrocytic), spectrin alpha chain (erythrocytic1), band 3 and protein 4.2. The defects in<unk>  lie in the cell membrane. The cell membrane proteins essential for the integrity of cell membrane structure includes; spectrin, ankyrin, band 3 and protein 4.1 and 4.2, and their deficiency can produce varying degree of severity of the disease. it should be differentiated from autoimmune hemolysis, congenital dyserythropoietic anemia type II, thermal injury and hemoglobinopathies.<unk>  can present at any age, having a positive family history is an important risk factor for the disease. Mean corpuscular hemoglobin concentration (MCHC) and erythrocyte distribution width (RDW) tests can be used for the screening of<unk> . Complications of the disease include; jaundice, kernicterus, pigment gallstones, splenomegaly, hemolytic, aplastic and megaloblastic crises. It can present with yellowing of skin, fatigue, irritability, shortness of breath or it can be asymptomatic altogether. Physical examination findings include scleral icterus, jaundice and splenomegaly. Laboratory testing includes CBC, MCHC, blood smear, hemolysis testing and coombs test. There is no specific medical therapy for the<unk> , however surveillance is needed to help detect and manage the complications. Folic acid supplementation, blood transfusions and erythropoietin may also be tried. Splenectomy is very effective in reducing the hemolysis. Partial splenectomies are tried in children to control hemolysis and preserve splenic function as well. Administration of vaccines (pneumococcal, hemophilus influenzae, meningococcal and influenza), antibiotics (penicillin) and folic acid should be prescribed for postsplenectomy patients.
Hereditary spherocytosis
Paroxysmal nocturnal hemoglobinuria
Paroxysmal cold hemoglobinuria
Familial erythrocytosis
Hereditary elliptocytosis
Congenital hemolytic anemia
Sideroblastic anemia
Beta-thalassemia