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Familial hemophagocytic lymphohistiocytosis in two brothers.

Gin Gin Gan, Geok Im Eow, Alan Kee Hean Teh, S C Ng, J. Vijaya Sangkar

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Abstract

Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here, we report the occurrence of this disorder in two brothers in their twenties.

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What this paper is about

Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here, we report the occurrence of this disorder in two brothers in their twenties.

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OpenAlex reports 2 citations for this work. Citation counts describe recorded attention and do not establish research quality.

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Available abstract

Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here, we report the occurrence of this disorder in two brothers in their twenties.

Key concepts: Hemophagocytosis, Pancytopenia, Medicine, Hemophagocytic lymphohistiocytosis, Histiocyte, Liver dysfunction, Histiocytosis, Malignant histiocytosis

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