A patient presents with anemia. Peripheral smear shows spherocytes. Direct antiglobulin test is negative. Osmotic fragility is positive. Supravital staining reveals blue-speckled red cells. Which hereditary condition is most likely?

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Multiple Choice

A patient presents with anemia. Peripheral smear shows spherocytes. Direct antiglobulin test is negative. Osmotic fragility is positive. Supravital staining reveals blue-speckled red cells. Which hereditary condition is most likely?

Red-cell membrane defects that reduce surface area cause spherocytes and make cells fragile in hypotonic solutions. In hereditary spherocytosis, these membrane-cytoskeleton abnormalities lead to chronic extravascular hemolysis, so you see anemia with spherocytes on the smear. The negative direct antiglobulin test argues against autoimmune hemolysis, which would usually be Coombs positive because antibodies or complement are bound to the red cells. A positive osmotic fragility test specifically reflects the membrane instability of spherocytes, explaining why they lyse more easily in hypotonic solutions. Supravital staining showing blue-speckled red cells indicates increased reticulocytosis as the bone marrow responds to ongoing hemolysis. Put together, these findings point to hereditary spherocytosis. This fits with a nonimmune hemolysis pattern, distinct from autoimmune hemolytic anemia, sickle cell disease, or aplastic anemia.

Red-cell membrane defects that reduce surface area cause spherocytes and make cells fragile in hypotonic solutions. In hereditary spherocytosis, these membrane-cytoskeleton abnormalities lead to chronic extravascular hemolysis, so you see anemia with spherocytes on the smear. The negative direct antiglobulin test argues against autoimmune hemolysis, which would usually be Coombs positive because antibodies or complement are bound to the red cells. A positive osmotic fragility test specifically reflects the membrane instability of spherocytes, explaining why they lyse more easily in hypotonic solutions. Supravital staining showing blue-speckled red cells indicates increased reticulocytosis as the bone marrow responds to ongoing hemolysis. Put together, these findings point to hereditary spherocytosis. This fits with a nonimmune hemolysis pattern, distinct from autoimmune hemolytic anemia, sickle cell disease, or aplastic anemia.

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