Question of the Week

Question :
Posted On : 10 Apr 2023
A male 4-month-old male infant presented with recurrent otitis media. he has pallor and splenomegaly. on investigations hemoglobin 6 percent, platelets 52000, ESR 35, TLC 14500, retics 3 percent, anisopoikilocytosis, tear drop cells, and pencil cells.
Expert Answer :
Recurrent infection in a Male child of 4 months age with splenomegaly makes me think of Wiskott Aldrich Syndrome. But, before we jump on that it will be worthwhile knowing the platelet size and platelet transfusion requirement. spleen size again will be important. Parental CBCs may also give us a clue. I suggest doing a Coomb test. Anemia may be due to iron deficiency also.
Answer Discussion :
lusungu muchelenganga
This infant is presenting with symptoms suggesting bone marrow infiltration or failure I am thinking of Wiskott-Aldrich Syndrome but it would be great to rule out any inherited bone marrow failure syndrome. The reticulocyte count is not very convincing for a hereditary hemolytic anemia like HS and hes not Jaundiced.The recurrent OMs are worrying for a primary immunodeficiency syndrome but could just be from the bone marrow failure.He needs genetic testing,DNA breakage tests ,Bone marrow aspirations,sickling test,Hb electrophoresis,HIV testing,Blood cultures,Urine and stool cultures,TB tests including checking of the adults in the home for TB,but it will be nice to rule out a neuroblastoma at that age(MS may present as anything at that age).He needs to be isolated and treated with broad spectrum antibiotics,monitor vitals closely and a crossmatch for blood transfusion with packed red cells.
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