Idiopatyczna limfocytopenia CD4

Kod Orpha: 228000Kod OMIM: 615518

Definicja

Idiopathic CD4 lymphocytopenia is a rare primary immunodeficiency disorder characterized by persistent CD4 T-cell lymphopenia (less than 300 cells/µL on multiple occasions) not associated with any other underlying primary or secondary immune deficiency. Patients typically present opportunistic infections (with cryptococcal, mycobacterial, candidal, varicella zoster virus infections and progressive multifocal leukoencephalopathy being the most prevalent), malignancies (mainly lymphoproliferative disorders), or autoimmune disorders. Some individuals are asymptomatic and incidentally diagnosed.

Dane
Klasyfikacja

Wada biologiczna

Kod ORPHA
228000
Kod OMIM
615518
Kod ICD10
D72.8
Kod ICD11
4A00.2

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