Familial hemophagocytic lymphohistiocytosis in two brothers

Familial hemophagocytic lymphohistiocytosis in two brothers

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 a...

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Veröffentlicht in:Medical journal of Malaysia 2004-03, Vol.59 (1), p.100-102
Hauptverfasser: Gan, G G, Eow, G I, Teh, A, Ng, S C, Sangkar, J V
Format: Artikel
Sprache:eng
Schlagworte:
Adult
Diagnosis, Differential
Histiocytosis, Non-Langerhans-Cell - diagnosis
Histiocytosis, Non-Langerhans-Cell - drug therapy
Histiocytosis, Non-Langerhans-Cell - genetics
Humans
Male
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Zusammenfassung: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.
ISSN:0300-5283