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glossary:lymphoproliferative_disorder

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glossary:lymphoproliferative_disorder [2008/05/06 06:37]
Pat O'Connor
glossary:lymphoproliferative_disorder [2012/10/16 14:40] (current)
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 +====== Lymphoproliferative Disorder ======
  
 +A [[disease]] in which [[cell]]s of the [[lymphatic system]] grow excessively. Lymphoproliferative disorders are often treated like [[cancer]]. ​
 +
 +======Types of Lymphoproliferative Disorders======
 +
 +==== Autosomal lymphoproliferative disorder ====
 +Some children with [[autoimmune]] lymphoproliferative syndrome have heterozygous mutations in the Fas receptor (CD95), a key component of a major apoptotic pathway. As a consequence of these mutations, a primitive population of [[T cell]]s proliferates in an uncontrolled manner, leading to the clinical sequelae of lymphoid infiltration,​ autoantibodies,​ and autoimmune disease. ​
 +
 +==== Castleman'​s Disease ====
 +
 +See: [[http://​www.lymphedemapeople.com/​thesite/​lymphedema_castlemans_disease.htm|Castleman'​s Disease]]
 +
 +==== Duncan Syndrome ====
 +"​X-linked lymphoproliferative (XLP) syndrome"​
 +
 +See: [[http://​www.lymphedemapeople.com/​phpBB2/​viewtopic.php?​t=201|Duncan'​s Syndrome]]
 +
 +==== Poems Syndrome ====
 +A chronic, progressive multisystem disorder of obscure aetiology characterised by polyneuropathy,​ organomegaly,​ endocrinopathy,​ monoclonal gammopathy and [[skin]] changes. ​
 +
 +See: [[http://​www.lymphedemapeople.com/​thesite/​lymphedema_castlemans_disease.htm|Castleman'​s Disease]]
 +
 +
 +==== Post-transplant lymphoproliferative disorder ====
 +A condition in which a group of [[B cell]]s grow out of control after an organ transplant in patients with weakened [[immune system]]s. This usually happens if the patient has also been infected with [[Epstein-Barr virus]]. Post-transplant lymphoproliferative disorder may progress to non-Hodgkin lymphoma. Also called PTLD.
 +
 +==== X-Linked Lymphoproliferative Disorder ====
 +X-linked lymphoproliferative (XLP) syndrome is a rare [[immunodeficiency]] [[disease]] characterized by a predilection for fatal or near-fatal Epstein-Barr virus (EBV)–induced infectious mononucleosis (IM) in childhood, subsequent [[hypogammaglobulinemia]],​ and a markedly increased risk of [[lymphoma]] or other lymphoproliferative diseases.
 +
 +X-linked lymphoproliferative (XLP) syndrome is an extremely rare inherited (primary) immunodeficiency disorder characterized by a defective [[immune system]] that is powerfully responsive to [[infection]] with the Epstein-Barr virus (EBV). This [[virus]] is common among the general population and is relatively well-known because it is the cause of [[infectious mononucleosis]] (IM), usually with no long-lasting effects. However, in individuals with XLP, exposure to EBV may result in severe, life-threatening fulminant hepatitis; abnormally low levels of antibodies in the [[blood]] and body secretions (hypogammaglobulinemia),​ resulting in increased susceptibility to various infections; malignancies of certain types of [[lymphoid tissue]] (B-cell lymphomas); and/or other abnormalities. The range of symptoms and findings associated with XLP may vary considerably from case to case. In addition, the range of effects may change in an affected individual over time. In most cases, individuals with XLP experience an onset of symptoms anytime from ages about 6 months to 10 years of age.
 +
 +==== 2008 ICD-9-CM Diagnosis 238.79 ====
 +
 +Other lymphatic and hematopoietic tissues
 +238.79 is a specific code that can be used to specify a diagnosis
 +238.79 contains 12 index entries
 +View the ICD-9-CM Volume 1 238.* hierarchy
 +
 +238.79 also known as:
 +Lymphoproliferative disease (chronic) NOS
 +Megakaryocytic myelosclerosis
 +Myeloproliferative disease (chronic) NOS
 +Panmyelosis (acute)
 +
 +Index entries containing 238.79:
 +Disease, diseased - see also Syndrome
 +lymphoproliferative (chronic) (M9970/1) 238.79
 +
 +myeloproliferative (chronic) (M9960/1) 238.79
 +
 +Disorder - see also Disease
 +lymphoproliferative (chronic) NEC (M9970/1) 238.79
 +
 +myeloproliferative (chronic) NEC (M9960/1) 238.79
 +
 +Myelofibrosis 289.83
 +megakaryocytic 238.79
 +
 +Myeloproliferative disease (M9960/1) 238.79
 +Myelosclerosis 289.89
 +megakaryocytic (M9961/1) 238.79
 +
 +Neoplasm, neoplastic 199.1 199.1 234.9 229.9 238.9 239.9
 +bone (periosteum) 170.9 198.5 - 213.9 238.0 239.2
 +marrow NEC 202.9 198.5 - - - 238.79
 +
 +hematopoietic,​ hemopoietic tissue NEC 202.8 198.89 - - - 238.79
 +
 +marrow (bone) NEC 202.9 198.5 - - - 238.79
 +
 +Panmyelosis (acute) (M9951/1) 238.79
 +Syndrome - see also Disease
 +myeloproliferative (chronic) (M9960/1) 238.79
glossary/lymphoproliferative_disorder.txt · Last modified: 2012/10/16 14:40 (external edit)