Is this visceral leishmaniasis or autoimmune hepatitis?
Author:
Pediatric Oncall
Question
A 10-year-old girl born of non-consanguineous marriage resident of Bihar, India presented with fever and recurrent jaundice for 4 years. She has fever and jaundice every 15 days that lasted 2-3 months and subsided on treatment. She had bleeding from the mouth 2 years ago. There was no hematemesis, melena, rash, or altered sensorium and abdominal distension. The birth was normal and milestones were normal. She was unimmunized. On examination, weight was 23 kg and height was 126 cm. There was no jaundice. On systemic examination, she had splenomegaly. Other systems were normal. Investigations showed:
• Hemoglobin = 11.6 gm/dl
• WBC count = 4,500/cumm
• Platelet count = 96,000/cumm
• Bilirubin = 0.6 mg/dl
• SGOT = 97 IU/L, SGPT = 65 IU/L
• Total proteins = 6.8 gm/dl, Albumin = 3.3 gm/dl
• Serum alkaline phosphatase = 845 IU/L
• Ultrasound abdomen = Splenohepatomegaly with coarse echotexture of liver and multiple lieno-renal collaterals
• HIV, HBsAg, Anti HCV = Negative
• Bone marrow aspiration = Erythroid hyperplasia
• ANA = 1:40, Anti smooth muscle Antibody, Anti LKM antibody = Negative
• CMV IgM, IgG, Ebstein Barr Virus nuclear antigen = Negative
• 24 hours urine copper = 20
• Liver biopsy = Fibrosis + LD bodies
Is this visceral leishmaniasis or autoimmune hepatitis?
Expert Opinion :
This child is a resident of Bihar, an area endemic for Kala-azar. She has had recurrent fever and jaundice and pancytopenia suggestive of a disease of the reticuloendothelial system. Her liver biopsy showed the presence of LD bodies, thus confirming the diagnosis of Kala-azar. Regarding autoimmune hepatitis, the only positive finding is an ANA of 1:40 titer which is not very high. In fact, an ANA of 1:80 is considered as positive, and less than that is considered as negative. Thus there is no evidence of autoimmune hepatitis. Though the child has developed collaterals suggestive of portal hypertension due to chronic liver disease, Kalaazar can lead to liver disease over a period of time. Thus in this child, the problem is due to Kala-azar and not due to autoimmune hepatitis. Pancytopenia may be due to hypersplenism or due to bone marrow involvement. A reticulocyte count will help to differentiate the same.