HLH (Hemophagocytic lymphohistiocytosis)
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- For the protein structural motif, see Basic-helix-loop-helix.
Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis. This is phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors in bone marrow and other tissues.
HLH may be associated with malignant, genetic, or autoimmune diseases. It is also prominently linked with Epstein-Barr virus (EBV) infection.
Hyperproduction of cytokines, including interferon-g and tumor necrosis factor-a, by EBV-infected T lymphocytes may play a role in the pathogenesis of HLH. EBV-associated HLH may mimic T-cell lymphoma and is treated with cytotoxic chemotherapy. In contrast, hemophagocytic syndromes associated with nonviral pathogens often respond to treatment of the underlying infection.
HLH comprises familial (primary) hemophagocytic lymphohistiocytosis (FHL) and secondary HLH (SHLH), both clinically characterized by the features described above, in particular fever, hepatosplenomegaly, and cytopenia.
FHL, an autosomal recessive disease, is invariably fatal when untreated. It is associated with defective triggering of apoptosis and reduced cytotoxic activity, resulting in a widespread accumulation of T lymphocytes and activated macrophages.
References
- Emerging Infectious Diseases Vol. 6, No. 6, November–December 2000
- Blood, 1 October 2002, Vol. 100, No. 7, pp. 2367-2373
External links
- http://www.hrtrust.org/library/HLHSymptomsSignsAndDiagnosis.html
- Histiocytosis at eMedicine
- http://www.histio.org
- http://www.jonahchuang.com
- http://www.elyseyu.com
- http://www.ethanmichaelsmith.com
- http://www.savejonah.com
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
