Lymphoproliferative Syndrome, X-Linked, 1 (XLP1)

Alias:

Lymphomatoid Papulosis

Purtilo Syndrome

Duncan Disease

X-Linked Lymphoproliferative Syndrome

X-Linked Lymphoproliferative Disease

Xlp

Xlp1

Lyp

X-Linked Lymphoproliferative Disease Due to Sh2d1a Deficiency

X-Linked Lymphoproliferative Syndrome 1

Lymphoproliferative Disease, X-Linked

Immunodeficiency 5

Xlpd

Imd5

Epstein-Barr Virus-Induced Lymphoproliferative Disease in Males

Immunodeficiency, X-Linked Progressive Combined Variable

X-Linked Progressive Combined Variable Immunodeficiency

Infectious Mononucleosis, Severe, Susceptibility to

Severe Susceptibility to Infectious Mononucleosis

Lymphoproliferative Syndrome, X-Linked, Type 1

Epstein-Barr Virus Infection, Familial Fatal

X-Linked Lymphoproliferative Syndrome Type 1

Sh2d1a/slam-Associated Protein Deficiency

Ebv Infection, Severe, Susceptibility to

Severe Susceptibility to Ebv Infection

Lymphoproliferative Syndrome, X-Linked

Familial Fatal Epstein-Barr Infection

X-Linked Lymphoproliferative Disorder

Infectious Mononucleosis, Severe

Ebv Infection, Severe

Sap Deficiency

Ebvs

Sap

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Basic Information

Medical Symptom

Gene & Mutation

Related Drugs

Disease Model

References Literature

Lymphoproliferative Syndrome, X-Linked, 1, also known as lymphomatoid papulosis, is related to primary cutaneous anaplastic large cell lymphoma and mycosis fungoides. An important gene associated with Lymphoproliferative Syndrome, X-Linked, 1 is SH2D1A (SH2 Domain Containing 1A), and among its related pathways/superpathways are Innate Immune System and Signal Transduction. The drugs Cytarabine and Melphalan have been mentioned in the context of this disorder. Affiliated tissues include t cells and skin, and related phenotypes are hypoalbuminemia and pancytopenia

Related ID：

MALACARDS：LYM113

OMIM：308240

MESH：D008232

ICD11：1791207220