HLH and autoimmune hepatitis

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Author: Pediatric Oncall

Question
A 2½ years old presented with jaundice for 1 month and fever for 15 days. He had been started on anti TB treatment {ATT} 7 months ago following which he had jaundice after a month and ATT was modified following which jaundice disappeared. ATT was given for total 6 months of duration. For the current episode of jaundice, he was investigated and found to have pancytopenia. Bone marrow examination was suggestive of hemophagocytosis and ferritin was 8737 with low Fibrinogen and high triglyceride. His ANA and anti-smooth muscle antibody were positive. Thus he was suspected to have HLH with autoimmune hepatitis. He was treated with cyclosporin and Dexamethasone for his HLH for 8 weeks.

Does he need treatment with steroids for longer duration for autoimmune hepatitis_?

Answer Discussion :

M

mahmoud magdy

0

Yes he need steroid for ttt

4 years ago

D

Daffaallah Soba

0

yeas

4 years ago

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Answer 1

The diagnosis of autoimmune hepatitis is not clear though ANA and anti-smooth muscle antibody are positive. A liver biopsy has not been done to check for interface hepatitis nor have been the total serum IgG. One also should rule out whether the HLH is primary or secondary. If primary, then HSCT will be required.

HLH and autoimmune hepatitis

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