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 cells proliferates in an uncontrolled manner, leading to the clinical sequelae of lymphoid infiltration, autoantibodies, and autoimmune disease.
See: Castleman's Disease
“X-linked lymphoproliferative (XLP) syndrome”
See: Duncan's Syndrome
A chronic, progressive multisystem disorder of obscure aetiology characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes.
See: Castleman's Disease
A condition in which a group of B cells grow out of control after an organ transplant in patients with weakened immune systems. 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 (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.
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