Diagnostic Dilemma

Fever an jaundice


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Case Report:- A 9 year old boy hailing from Jaunpur, UP presented with fever for 6 months and jaundice with high coloured urine for 3 months. For the same, he was investigated and fine needle aspiration of cervical lymph node was done which was suggestive of tuberculosis and was started on antituberculous therapy {ATT} but fever did not respond. There was no skin rash, previous exposure to drugs, blood transfusion or altered sensorium. He had now developed frequent epistaxis and malena. On examination, he was deeply icteric, had pallor, petechiae, hepatosplenomegaly and ascitis. Other systems were normal.
Investigations showed:
• Hemoglobin = 7 gm percent {which persistently remained low}
• WBC count = 8,600, cumm {64 percent polymorphs, 30 percent lymphocytes, 6 percent eosinophils}
• Platelet count = 17,000, cumm {which persistently remained low}
• Bilirubin = 13.6 mg, dl {Direct = 10.8 mg, dl} which showed a gradual rise
• SGOT = 50 IU, L, SGPT = 40 IU, L
• Total proteins = 6.2 gm, dl, Albumin = 2.3 gm, dl
• Alkaline phosphatase = 823 IU, L {elevated}
• BUN, Creatinine, electrolytes, calcium, phosphorus, blood gas, blood sugar = Normal
• ESR = 127 mm at end of 1 hour
• LDH = 746 IU, L, Uric Acid = 2.8 mg, dl
• Serum ceruloplasmin = Normal
• GGTP = 20 IU, L
• Prothrombin time and Partial thromboplastin time = Constantly deranged
• Bone marrow aspiration = Normocellular marrow with few hemophagocytes
• Blood culture, HIV ELISA, HBsAg, Anti Hepatitis C, Anti Hepatitis A, Dengue, Leptospira = Negative
• CMV IgG = Positive
• EBV Early nuclear antigen IgG = Positive
• ANA, dsDNA, Anti smooth muscle antibody, Anti LKM = Negative
• Ultrasound abdomen = Hepatosplenomegaly with minimal asctis
• Fibrinogen = 143 {Normal}
• FDP and d-dimers = elevated
• Ferritin = 5,800 mg, dl

A differential diagnosis of the following was considered:
1. Kalaazar
2. CMV, EBV infection
3. Autoimmune hepatitis
4. Hemophagocytosis
5.
The child was given regular blood transfusion and plasma. He also received a short course of steroids for a suspected macrophage activation syndrome. A liver biopsy could not be done due to clinical condition and the child continued to remain febrile.

What is the diagnosis _?


What is the diagnosis_?
Expert Opinion :
We would like to thank all viewers for their inouts. Regarding HLH, though bone marrow showed a few hemophagocytes, it was not conclusive of HLH and even serym ferritin was normal. We suspect the child had EBV induced problem as the only thing positive for EBNA in the child. He died subsequently due to his illness and post mortem liver biopsy was inconclusive
Answer Discussion :
S
shanthi shanthi
bubble
hemophagocytic lymphohistiocytosis chemotherapy can be tried
15 years ago
N
Niyaz Ahmad Buch
bubble
infectious mononucliosis with aplastic anemia
15 years ago

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