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
Bloodjournal_title
Bloodauthors
Calvo KR,Price S,Braylan RC,Oliveira JB,Lenardo M,Fleisher TA,Rao VKdoi
10.1182/blood-2014-11-567917subject
Has Abstractpub_date
2015-04-30 00:00:00pages
2753-8issue
18eissn
0006-4971issn
1528-0020pii
blood-2014-11-567917journal_volume
125pub_type
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