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hemophagocytic lymphohistiocytosis; hemophagocytic syndrome
Etiology:
- several inherited immune deficiencies may predispose children
- most adults have no underlying immune disorder
- triggers include Herpesvirus & less frequently other infectious agents
- malignant lymphomas may predispose, especially in adults
- complication of dengue
- lamotrigine
Pathology:
- severe inflammation
- hemophagocytosis by activated, morphologically benign macrophages
- impaired function of NK cells & cytotoxic T-cells
Clinical manifestations:
- prolonged fever
- cytopenia
- hepatosplenomegaly
- lymphadenopathy
- bleeding
- neurologic manifestations: seizures, ataxia, visual disturbances
Laboratory:
- serum ferritin is elevated & is used as a marker for macrophage activation during treatment [1,2]
- serum triglycerides are low
- plasma fibrinogen is low
- elevated CD25 cells in blood
Special laboratory:
- hemophagocytosis identified through
- bone marrow biopsy
- spleen biopsy
- lymph node biopsy
Differential diagnosis:
- infection
Complications:
- may be fatal
Management:
- low risk patients:
- cyclosporine, glucocorticoids, or intravenous immunoglobulin
- intravenous immunoglobulin within hours of disease onset
- high risk:
- etoposide plus dexamethasone for 8 weeks
- cyclosporine may be added at the beginning
- intrathecal methotrexate is used only with persistently abnormal CSF analysis or progressive neurologic symptoms
- alemtuzumab may be effective for refractory disease [2]
- treat associated malignancy
- cure with bone marrow transplantation
Specific
familial histiocytic reticulosis; familial hemophagocytic reticulosis; familial hemophagocytic lymphohistiocytosis; familial erythrophagocytic lymphohistiocytosis
General
lymphohistiocytosis
macrophage activation syndrome
References
- Janka GE
Hemophagocytic syndromes.
Blood Rev. 2007 Sep;21(5):245-53. Epub 2007 Jun 21.
PMID: 17590250
- Schwartz RA and Coppes MJ
Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis) Treatment
& Management
MedScape: eMedicine
http://emedicine.medscape.com/article/986458-treatment