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Bone marrow plasma cells 20% or greater discriminate presentation, response, and survival in AL amyloidosis.

Abstract：

:We explored the association between bone marrow plasma cells (BMPCs) and disease presentation and outcome among 1574 AL patients. Three BMPC groups were formulated: <5% (n = 231, 15% of study population), 5-19% (n = 1045, 66%), and ≥20% (n = 298, 19%). Heart and renal involvement were more and less prevalent, respectively, with increasing BMPCs. Patients with ≥20% BMPCs had higher likelihood for classic myeloma phenotype with less skewed lambda restriction, a higher rate of intact immunoglobulin secretion, a lower hemoglobin and higher rates of hypercalcemia and bone lytic lesions. High-risk cytogenetic abnormalities were more common in ≥20% BMPCs. Complete hematological response was less frequent with rising BMPCs. The median survival was inversely associated with the BMPC groups (81, 33, 12 months for <5%, 5-19%, and ≥20% BMPCs, respectively; P < 0.001). Survival discrimination was maintained at 1-year landmark and in those who achieved a complete response. Multivariate analysis accounting for known prognostic markers yielded an independent prognostic role for ≥20% BMPCs, but not for the other BMPC groups. AL patients with 20% or greater BMPCs have poorer outcome independent of their cardiac risk category and stem cell transplant eligibility. Distinct interventions in these patients should be explored to improve outcome.

journal_name

Leukemia

journal_title

Leukemia

authors

Muchtar E,Gertz MA,Kourelis TV,Sidana S,Go RS,Lacy MQ,Buadi FK,Dingli D,Hayman SR,Kapoor P,Leung N,Fonder A,Hobbs M,Lisa Hwa Y,Gonsalves W,Warsame R,Russell S,Lust JA,Lin Y,Zeldenrust S,Rajkumar SV,Kyle RA,Kum

doi

10.1038/s41375-019-0655-x

subject

Has Abstract

pub_date

2020-04-01 00:00:00

pages

1135-1143

issue

4

eissn

0887-6924

issn

1476-5551

pii

10.1038/s41375-019-0655-x

journal_volume

34

pub_type

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