Diagnostic Dilemma

Hepatosplenomegaly with bicytopenia

A 2 years old girl presented with lump on left side of abdomen for 6 months. There was no pallor or jaundice. On examination, she had splenohepatomegaly with palmar erythema and cervical adenopathy. Weight was 8 kg.
• She had anemia and thrombocytopenia with peripheral smear suggestive of either blast like cells or atypical lymphocytes.
• Her direct and indirect Coombs test was negative.
• SGOT was 88 IU, L, SGPT = 71 IU, L.
• USG abdomen showed hepatosplenomegaly with intra abdominal lymphnodes and splenic microabscess.
• Bone marrow biopsy showed mature B cell lymphocytosis with relative erythroblastopoiesis and megakaryocytes were seen. There were no blasts or abnormal cells.
• Lymphnode biopsy was suggestive of viral induced lymphadenopathy.
• Epstein Barr Virus {EBV} VCA IgM was negative.
• HIV ELISA was negative.
• S.immunoglobulins showed increased serum IgG.
• Lymphocyte subset analysis showed increased CD19, CD3 and CD4 cells.
• Liver biopsy showed intraepithelial lymphocytosis suggestive of either hepatitis C infiltration or autoimmune hepatitis.
• Her Hepatitis C ELISA was negative and Anti Liver Kidney Microsomal {LKM}, ANA and anti smooth muscle antibody were negative.

What is the diagnosis_?
Expert Opinion :
Lymphnode biopsy in this child - no lymphoma, TB
Bone marrow biopsy - no leukemia
Liver biopsy - suggestive of viral hepatitis.

There is no fever in the child to suspect CGD.

Currently we do not have a diagnosis in this child and she is on follow up to see how the disease evolves.
Answer Discussion :
pawan kumar
11 years ago
majid momin
11 years ago

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