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Hemophagocytic lymphohistiocytosis
Other Names for this Disease
- Familial erythrophagocytic lymphohistiocytosis
- Familial hemophagocytic lymphohistiocytosis
- Familial histiocytic reticulosis
- Hemophagocytic lymphohistiocytosis familial -1
- HPLH1
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Overview
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition in which certain white blood cells (histiocytes and lymphocytes) build up in organs including the skin, spleen, and liver, and destroy other blood cells. This causes fever and damages the liver and spleen, resulting in enlargement of these organs. HLH may be inherited or it may be caused by certain conditions or diseases including infections and immunodeficiency (inability of the body to fight infections). It most commonly affects young infants and children.[1][2] Treatment includes antibiotics or antiviral medications to treat or prevent infections, and chemotherapy and immunotherapy prior to allogeneic hematopoietic cell transplantation.[3]
References
- Fisman DN. Hemophagocytic Syndromes and Infection. Emerging Infectious Diseases. March 2009. http://www.cdc.gov/ncidod/eid/vol6no6/fisman.htm. Accessed July 21, 2011.
- Familial hemophagocytic lymphohistiocytosis. Genetics Home Reference. http://ghr.nlm.nih.gov/condition/familial-hemophagocytic-lymphohistiocytosis. Accessed July 22, 2011.
- Kejian Zhang, Alexandra H Filipovich, Judith Johnson, Rebecca A Marsh, and Joyce Villanueva. Hemophagocytic Lymphohistiocytosis, Familial. GeneReviews. http://www.ncbi.nlm.nih.gov/books/NBK1444/. Accessed November 26, 2012.
Your Questions Answered
by the Genetic and Rare Diseases Information Center2 question(s) from the public on Hemophagocytic lymphohistiocytosis have been answered. See questions and answers. You can also submit a new question.
General Information
- Genetics Home Reference (GHR) contains information on Hemophagocytic lymphohistiocytosis. Click on the link to go to GHR and review the information.
- The Histiocytosis Association of America has an information page on hemophagocytic syndromes. Click on Histiocytosis Association to view the information page.
- Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Hemophagocytic lymphohistiocytosis. Click on the link to view a sample search on this topic.
- The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Hemophagocytic lymphohistiocytosis. Click on the link to go to OMIM and review these resources.
Selected Full-Text Journal Articles
- Fisman DN. Hemophagocytic Syndromes and Infection. Emerging Infectious Diseases. 2000; 6(6): 601-608.
Insurance Issues
- The Social Security Administration has included this condition in their Compassionate Allowances Initiative. This initiative speeds up the processing of disability claims for applicants with certain medical conditions that cause severe disability. More information about Compassionate Allowances and applying for Social Security disability is available online.