Diagnostic Dilemma

Myelofibrosis, jaundice and scrotal swelling


Author: Pediatric Oncall
Question
A 4 years 8 months old boy presented with fever for 6 months, progressive abdominal distension for one month, epistaxis 20 days ago and bilateral swelling of feet since one day. He was diagnosed as myelofibrosis 4 months ago. At that time he had pancytopenia and bone marrow was hypocellular and had myelofibrosis with 9% blasts. He had received 5 blood transfusions and one platelet transfusion since then. There is no fever, jaundice or other bleeding, manifestations currently. On examination, he had icterus, edema feet, with splenohepatomegaly, ecchymotic patches on chest and legs and scrotal swelling with generalized lymphadenopathy. His investigations showed:
• Hemoglobin = 5.8 gm/dl
• WBC count = 15,400/cumm
• Platelet count = 6000/cumm
• Bilirubin = 3.2 mg/dl, SGOT = 78 IU/L, SGPT = 52 IU/L, Albumin = 3.7 gm/dl.
• USG abdomen showed hepatosplenomegaly without ascites.


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