Acquired hemophagocytic lymphohistiocytosis associated with malignant disease

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.

Disease data

Classification

Particular clinical situation in a disease or syndrome

ORPHA code

158057

OMIM code

ICD10 code

D76.1

ICD11 code

3B62.Y

*Soruce