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Acquired hemophagocytic lymphohistiocytosis associated with malignant disease

ORPHA:158057· ICD-10 D76.1

Definition

A rare, secondary hemophagocytic lymphohistiocytosis characterized by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are lukemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings.

Prevalence
Unknown
Age of onset
All ages