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P-436 Clonal Hematopoiesis of Indeterminate Potential (CHIP) in Patients with Amyloid Light Chain (AL) Amyloidosis

Clinical lymphoma myeloma & leukemia/Clinical lymphoma, myeloma and leukemia(2023)

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摘要
Additional work-up identified then an IgA Lambda monoclonal peak (1.9 g/L), with a discrete marrow infiltration, a situation considered as a MGCS.Treatment with SQ Daratumumab was proposed with the initial disappearance of any angioedema attack after the first 2 months of therapy, but relapses thereafter, persisting after 6 cycles of SQ Daratumumab.The patient was further rechallenged with lanadelumab with success.Conclusions: We report a case of acquired angioedema, a rare form of MGCS, that exemplifies the difficulty to understand the relationship between a monoclonal component and the clinical manifestations of the disease, since a therapy targeting directly the clone was not efficacious in this setting.This case also highlights the spectrum of various disorders associated with MGUS.
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