Hypoplastic marrow

Author: Narasimhappa

Question
A 10 years old girl presented to us with jaundice for 10 days and fever for 3 days. On examination, there was icterus, purpura, ecchymoses, anemia and firm liver of 6-7 cm below costal margin {which made us think of acute on chronic liver disease}. There was no lymphadenopathy or splenomegaly. Her hemoglobin was 5.2 g, total count 1000 and platelet count was 3000. KF ring was positive on Slit lamp examination but ceruloplasmin and urinary copper were not suggestive of Wilson’s. Peripheral smear showed no evidence of hemolysis. All hepatitis viral markers including hepatitis A,B,C were negative. ANA – negative. Her bilirubin was 8.2 with direct of 4.2. Liver transaminases were elevated 1200 and 1100. Serum albumin, PT, PTT were normal. Bone marrow aspiration done at 2 centres showed hypoplastic marrow. Bone marrow biopsy was not done. Other autoimmune hepatitis markers awaited and parvovirus serology is awaited. We thought initially as autoimmune hepatitis or parvovirus induced bone marrow suppression or infection induced suppression. She was started on Methylprednisolone but her counts kept on going down. TLC- 750 and platelets – 2000. We stopped steroids and she is on taxim and amikacin. She is too stable for her condition.

What is the diagnosis and what more investigations can be done and any other treatment_?

Answer Discussion :

Ira Shahcas

Since the child has hepatitis and pancytopenia, one would think of EBV, HLH, Hep B and Hep C. However presence of KF ring is unusual. Have you got it rechecked_? What was the 24hours urine copper_?

13 years ago

Vishal Baldua

EBV - SECONDARY HEMOPHAGOCYTOSIS OR BONE MARROW SUPPRESSION

DIRECT BILI IS WELL KNOWN WITH EBV INFECTIONS

13 years ago

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Hypoplastic marrow

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