Lymphoproliferative disorders ICD-9 | 238.79 eMedicine | ped/1345 MeSH | D008232 WikiDoc Resources for Lymphoproliferative disorders Articles Most recent articles on Lymphoproliferative disorders Most cited articles on Lymphoproliferative disorders Review articles on Lymphoproliferative disorders Articles on Lymphoproliferative disorders in N Eng J Med, Lancet, BMJ Media Powerpoint slides on Lymphoproliferative disorders Images of Lymphoproliferative disorders Photos of Lymphoproliferative disorders Podcasts & MP3s on Lymphoproliferative disorders Videos on Lymphoproliferative disorders Evidence Based Medicine Cochrane Collaboration on Lymphoproliferative disorders Bandolier on Lymphoproliferative disorders TRIP on Lymphoproliferative disorders Clinical Trials Ongoing Trials on Lymphoproliferative disorders at Clinical Trials.gov Trial results on Lymphoproliferative disorders Clinical Trials on Lymphoproliferative disorders at Google Guidelines / Policies / Govt US National Guidelines Clearinghouse on Lymphoproliferative disorders NICE Guidance on Lymphoproliferative disorders NHS PRODIGY Guidance FDA on Lymphoproliferative disorders CDC on Lymphoproliferative disorders Books Books on Lymphoproliferative disorders News Lymphoproliferative disorders in the news Be alerted to news on Lymphoproliferative disorders News trends on Lymphoproliferative disorders Commentary Blogs on Lymphoproliferative disorders Definitions Definitions of Lymphoproliferative disorders Patient Resources / Community Patient resources on Lymphoproliferative disorders Discussion groups on Lymphoproliferative disorders Patient Handouts on Lymphoproliferative disorders Directions to Hospitals Treating Lymphoproliferative disorders Risk calculators and risk factors for Lymphoproliferative disorders Healthcare Provider Resources Symptoms of Lymphoproliferative disorders Causes & Risk Factors for Lymphoproliferative disorders Diagnostic studies for Lymphoproliferative disorders Treatment of Lymphoproliferative disorders Continuing Medical Education (CME) CME Programs on Lymphoproliferative disorders International Lymphoproliferative disorders en Espanol Lymphoproliferative disorders en Francais Business Lymphoproliferative disorders in the Marketplace Patents on Lymphoproliferative disorders Experimental / Informatics List of terms related to Lymphoproliferative disorders Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Synonyms and keywords: B cell proliferation, T cell proliferation, LPD, lymphoproliferation, lymphoproliferative neoplasm ## Overview[edit | edit source] Lymphoproliferative disorders refer to several conditions of lymphatic diseases in which lymphocytes are produced in excessive quantities[1]. They typically occur in patients who have compromised immune systems. They are sometimes equated with "immunoproliferative disorders", but technically lymphoproliferative disorders are a subset of immunoproliferative disorders, along with hypergammaglobulinemia and paraproteinemias. ## Examples of LPDs[edit | edit source] * Chronic lymphocytic leukemia * Acute lymphoblastic leukemia * Castleman disease * Hairy cell leukemia * Lymphomas * Multiple myeloma * Wiskott-Aldrich syndrome * Post-transplant lymphoproliferative disorder * Autoimmune lymphoproliferative syndrome (ALPS) * Plasma cell leukemia ## Types[edit | edit source] Lymphoproliferative disorders are a set of disorders characterized by the abnormal proliferation of lymphocytes into a monoclonal lymphocytosis. The two major types of lymphocytes are B cells and T cells, which are derived from pleuripotent hematopoetic stem cells in the bone marrow. Individuals who have some sort of immunodysfuction are susceptible to developing a lymphoproliferative disorder because when any of the numerous control points of the immune system become dysfunctional, immunodeficiency or deregulation of lymphocytes is more likely to occur. There are several inherited gene mutations that have been identified to cause lymphoproliferative disorders, however there are also acquired and iatrogenic causes.[2] ### X-linked Lymphoproliferative disorder[edit | edit source] Main article: X-linked lymphoproliferative disease There is a mutation on the X chromosome that has been found to be associated with a T and NK cell lymphoproliferative disorder. ### Autosomal lymphoproliferative disorder[edit | edit source] Main article: Autoimmune lymphoproliferative syndrome Some children with autoimmune lymphoproliferative disorders are heterozygous for a mutation in the gene that codes for the Fas receptor, which is located on the long arm of chromosome 10 at position 24.1, denoted 10q24.1.[3] This gene is member 6 of the TNF-receptor superfamily (TNFSF6). The Fas receptor contains a death domain and has been shown to play a central role in the physiological regulation of programmed cell death. Normally, stimulation of recently activated T cells by antigen leads to coexpression of Fas and Fas receptor on the T cell surface. The engagement of Fas by Fas receptor results in apoptosis of the cell and is important for eliminating T cells that are repeatedly stimulated by antigens.[4] As a result of the mutation in the Fas receptor gene, there is no recognition of Fas by Fas receptor, leading to a primitive population of T cells that proliferates in an uncontrolled manner.[2] ### Other inherited causes[edit | edit source] Boys with X-linked immunodeficiency syndrome are at a higher risk of mortality associated with EBV infections, and are predisposed to develop a lymphoproliferative disorder or lymphoma. Children with common variable immune deficiency (CVID) are also at a higher risk of developing a lymphoproliferative disorder. Some disorders that predispose a person to lymphoproliferative disorders are severe combined immuno deficiency (SCID), Chédiak-Higashi syndrome, Wiskott-Aldrich syndrome (an X-linked recessive disorder) and Ataxia telangiectasia. Interestingly, even though Ataxia telangiectasia is an autosomal recessive disorder, people who are heterozygotes for this still have an increased risk of developing a lymphoproliferative disorder.[2] ### Acquired causes[edit | edit source] Viral infection is a very common cause of lymphoproliferative disorders. The most common is congenital HIV infection because it is highly associated with acquired immunodeficiency, which often leads to lymphoproliferative disorders.[2] ### Iatrogenic causes[edit | edit source] There are many lymphoproliferative disorders that are associated with organ transplantation and immunosuppressant therapies. In most reported cases, these cause B cell lymphoproliferative disorders, however some T cell variations have been described.[2] The T cell variations are usually caused by the prolonged use of T cell suppressant drugs, such as sirolimus, tacrolimus belatacept or cyclosporine A.[2] ## See also[edit | edit source] * Evans syndrome * Leukaemia * Lymphoma * Lymphocytosis * Myeloma * Myeloproliferative disease ## References[edit | edit source] 1. ↑ Anonymous (2023), Lymphoproliferative disorders (English). Medical Subject Headings. U.S. National Library of Medicine. 2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Winter, S.S. Lymphoproliferative disorders. Emedicine. December 20, 2006. http://www.emedicine.com/ped/topic1345.htm. Accessed March 2007. 3. ↑ Entrez Gene. FAS Fas (TNF receptor superfamily, member 6). http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=gene&dopt=full_report&list_uids=355. Accessed March 2007. 4. ↑ Abbas, A.K and Lichtman, A.H. Cellular and Molecular Immunology. Fifth Edition. Elsevier Saunders. Philadelphia. 2005 ## External links[edit | edit source] * Sloan-Kettering Template:Hematology * v * t * e Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208) CFU-GM/ and other granulocytes| Template:Navbox subgroup MEP| Template:Navbox subgroup CFU-Mast| Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis) Multiple/unknown| AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) * MP (Myelofibrosis) * Acute biphenotypic leukaemia See also hematology, lymphoid malignancy ar:مرض التكاثر الليمفاوي Template:WH Template:WS *[v]: View this template *[t]: Discuss this template *[e]: Edit this template