Question of the Week

Question :

Posted On : 22 Apr 2006

How do you treat hemophagocytic syndrome and how do you follow up such patients?

Expert Answer :

No expert answer available.

Answer Discussion :

nikhil agrawal

Chemotherapy ,immunotherapy,intravenous immunoglobulin and allogeneic bone marrow transplantation

18 years ago

pediatriconcall

Hemophagocytosis can occur as a result of multiple etiologies: viral, bacterial, parasitic {Infectious}, malignant, autoimmune, and familial. Hemophagocytic syndromes are associated with a high mortality {30-45 Percent} dependent on etiology and degree of associated organ failure. Malignancy associated and familial hemophagocytic syndromes have a worse prognosis than infection associated.

In general therapy is supportive with treatment of the underlying disorder. High dose corticosteroids, immunoglobulins, plasmapheresis and other immue-modulating treatments such as etoposide are used in treatment. One would need to monitor CBC, liver function tests, renal function tests and clinical examination serially to assess improvement. Occasionally a bone marrow may be required to check for hemophagocytes in marrow. Other parameters such as fibrinogen, triglycerides, cholesterol, ferrtin etc should also be monitored.

18 years ago

RICHARD ZAMMIT

blood exchange perfusion

18 years ago

H C BHATT

it is of two types familial and acquired usually secondary to viral infections,high level of suscpicion is required,presents with fever hsm irritability ,cns symptomps as well,bone marrow establishes the diagnosis with accumulation of t cells,usually treatment is immunoglobulins.steroids,cyclosporin a.-definte treatment is allogenic BMT.

18 years ago

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