JMML and RALD (Ras-associated autoimmune leukoproliferative disorder): common genetic etiology yet clinically distinct entities.

Abstract:

:Ras-associated autoimmune leukoproliferative disorder (RALD) is a chronic, nonmalignant condition that presents with persistent monocytosis and is often associated with leukocytosis, lymphoproliferation, and autoimmune phenomena. RALD has clinical and laboratory features that overlap with those of juvenile myelomonocytic leukemia (JMML) and chronic myelomonocytic leukemia (CMML), including identical somatic mutations in KRAS or NRAS genes noted in peripheral blood mononuclear cells. Long-term follow-up of these patients suggests that RALD has an indolent clinical course whereas JMML is fatal if left untreated. Immunophenotyping peripheral blood from RALD patients shows characteristic circulating activated monocytes and polyclonal CD10(+) B cells. Distinguishing RALD from JMML and CMML has implications for clinical care and prognosis.

journal_name

Blood

journal_title

Blood

authors

Calvo KR,Price S,Braylan RC,Oliveira JB,Lenardo M,Fleisher TA,Rao VK

doi

10.1182/blood-2014-11-567917

subject

Has Abstract

pub_date

2015-04-30 00:00:00

pages

2753-8

issue

18

eissn

0006-4971

issn

1528-0020

pii

blood-2014-11-567917

journal_volume

125

pub_type

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