MEDizzy - A 19-year-old primigravida of Southeast Asian ancestry gives

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Combined Hematology II

A 19-year-old primigravida of Southeast Asian ancestry gives birth at 35 weeks gestation a male infant. On physical examination the infant is markedly hydropic. Laboratory studies show his hematocrit is 17% and the peripheral blood smear reveals numerous nucleated red blood cells and even a few erythroblasts. The red blood cells display marked anisocytosis and poikilocytosis. Which of the following diseases is most likely to be present in this infant?

ExplanationB. This patient presented with anemia at birth with microcytosis, nRBCs and anisopoikilocytosis in peripheral blood. Out of the options mentioned, Hemoglobin E disease and sickle cell disease do not present at birth. G6PD and hereditary elliptocytosis can rarely present at birth. Hereditary elliptocytosis is uncommon in the neonatal period. Typically, elliptocytosis does not appear on the blood film until the patient is 4 to 6 months old. Occasionally, severe forms of hereditary elliptocytosis present in the neonatal period with severe, hemolytic anemia with marked poikilocytosis and jaundice. Alpha thalassemia is a frequent cause of stillbirth in Southeast Asia. Infants either are stillborn at 34 to 40 weeks of gestation or are born alive but die within the first few hours. Pallor, edema and hepatosplenomegaly are seen.