Primary Systemic Amyloidosis

Watchlist

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

CCND1, LINC02343, CBX7, LINC00457, SMARCD3, TTR, DNAH11, CD38, SCT, GDF15, NPPB, SPP1, IGKV2-29, MIR34A, SMN1, SMN2, ST2, TNF, BMS1P20, KRT20, IGKV1-8, BABAM2, GLIPR1, WDHD1, MAGEC2, SLC1A4, PLXNB2, IGKV3D-15, IGKV1D-8, IGKV3-20, PRAME, ALB, SDC1, FGA, BCR, PRDM1, CAT, MS4A1, CDA, CDH1, CRP, CSF3, CYP1B1, DDT, ETFA, GSN, FAS, IGKV@, LGALS3, LGALS4, MDK, MMP1, MMP2, MUC1, MYD88, NCAM1, PFDN5, RPS27A, SAA1

Drugs

Allogeneic multi-virus specific T lymphocytes targeting BK virus, cytomegalovirus, human herpesvirus-6, Epstein Barr virus and adenovirus, Carboxy pyrrolidine hexanoyl pyrrolidine carboxylate, Chimeric fibril-reactive IgG1k monoclonal antibody 11-1F4, Daratumumab ( DARZALEX ), Eprodisate, Humanised IgG1 kappa antibody against serum amyloid A and AL amyloid, Iodine (123I) Serum Amyloid P, Ixazomib ( NINLARO ), N-(3,4-dihydroxyphenyl)-3,4-dihydroxybenzamide, Recombinant monoclonal antibody to human serum amyloid P component, florbetaben (18F), 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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Primary systemic amyloidosis (PSA) is a form of AL amyloidosis (see this term) caused by the aggregation and deposition of insoluble amyloid fibrils derived from misfolded monoclonal immunoglobulin light chains usually produced by a plasma cell tumor (see this term) and characterized by multiple organ involvement.