Hemophagocytosis
Jump to navigation
Jump to search
Clinic
- Hemophagocytic lymphohistiocytosis (HLH) = Haemophagocytic lymphohistiocytosis = Hemophagocytic = Haemophagocytic syndrome
- It is an uncommon hematologic disorder seen more often in children than in adults.
- It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterised by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines.
- It is classified as one of the cytokine storm syndromes.
- There are inherited and acquired causes
Signs / Symptoms
- Fever
- hepatosplenomegaly
- Lymphadenopathy
- Yellow discoloration of the skin and eyes
- Rash
- Elevated triglyceride levels, low fibrinogen levels, transaminitis, and elevated ferritin levels
Causes
Related disease
- T-cell lymphoma
- B-cell lymphoma
- Acute lymphocytic leukemia
- Acute myeloid leukemia
- Myelodysplastic syndrome
- Juvenile idiopathic arthritis
- Juvenile Kawasaki disease
- SLE
- Still's disease
- Rheumatoid arthritis
- DiGeorge syndrome
- Wiskott–Aldrich syndrome
- Ataxia–telangiectasia
- Dyskeratosis congenita
- ↑ Kalmuk J, Matar S, Feng G, Kilb E, Lim MY. Parvovirus B19-induced hemophagocytic lymphohistiocytosis: Case report and review of the literature. Clin Case Rep. 2019 Sep 27;7(11):2076-2081. doi: 10.1002/ccr3.2401. PMID: 31788255; PMCID: PMC6878072.
- ↑ Bonnecaze AK, Willeford WG, Lichstein P, Ohar J. Acute Cytomegalovirus (CMV) Infection Associated with Hemophagocytic Lymphohistiocytosis (HLH) in an Immunocompetent Host Meeting All Eight HLH 2004 Diagnostic Criteria. Cureus. 2017 Mar 2;9(3):e1070. doi: 10.7759/cureus.1070. PMID: 28409071; PMCID: PMC5376153.
- ↑ Manji F, Wilson E, Mahe E, Gill J, Conly J. Acute HIV infection presenting as hemophagocytic lymphohistiocytosis: case report and review of the literature. BMC Infect Dis. 2017 Sep 20;17(1):633. doi: 10.1186/s12879-017-2732-y. PMID: 28931369; PMCID: PMC5607499.
- ↑ Meazza Prina, M., Martini, F., Bracchi, F. et al. Hemophagocytic syndrome secondary to SARS-Cov-2 infection: a case report. BMC Infect Dis 21, 811 (2021). https://doi.org/10.1186/s12879-021-06532-7