Idiopathic Cd4 Lymphocytopenia

Watchlist

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

UNC119, FANCD2, IL7, CD27, TP53BP1, E2F7, MUS81, GGCT, FANCI, UHRF1, BABAM1, EXO1, STAT5B, CD34, STAT5A, RAD51, IL7R, IL2, DUSP4, ATN1, CD40LG, LINC02605

Drugs

Allogeneic multi-virus specific T lymphocytes targeting BK virus, cytomegalovirus, human herpesvirus-6, Epstein Barr virus and adenovirus, Recombinant human interleukin-7 fused to a hybrid crystallisable fragment region of a human antibody (IL-7-hyFc), haematopoietic stem cells and blood progenitors umbilical cord-derived expanded with (1R, 4R)-N1-(2-benzyl-7-(2-methyl-2H-tetrazol-5-yl)-9H-pyrimido[4,5-b]indol-4-yl)cyclohexane-1,4-diamine dihydrobromide dihydrate

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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.