ORPHA:158057
Acquired hemophagocytic lymphohistiocytosis associated with malignant disease
- Body system
- Immunological diseases
- Inheritance pattern
- Not documented in Orphadata
- Typical age of onset
- All ages
- Estimated prevalence
- Unknown (Worldwide)
- Rarity class
- Unknown
About this disease
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.
Definition from Orphanet.
Common signs
- Abnormal circulating interleukin concentration
- Splenomegaly
- Pancytopenia
- Fever
- Immune dysregulation
- Increased circulating ferritin concentration
Learn more
Source: Orphanet (Orphadata), ORPHA:158057. Data release 2026-06-23. www.orphadata.com — CC BY 4.0.