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Mastocytosis among elderly patients: A multicenter retrospective French study on 53 patients.

Abstract：

:Mastocytosis is a heterogeneous group of diseases with a young median age at diagnosis. Usually indolent and self-limited in childhood, the disease can exhibit aggressive progression in mid-adulthood. Our objectives were to describe the characteristics of the disease when diagnosed among elderly patients, for which rare data are available.The French Reference Center conducted a retrospective multicenter study on 53 patients with mastocytosis >69 years of age, to describe their clinical, biological, and genetic features.The median age of our cohort of patients was 75 years. Mastocytosis variants included were cutaneous (n = 1), indolent systemic (n = 5), aggressive systemic (n = 11), associated with a hematological non-mast cell disease (n = 34), and mast cell leukemia (n = 2). Clinical manifestations were predominantly mast cell activation symptoms (75.5%), poor performance status (50.9%), hepatosplenomegaly (50.9%), skin involvement (49.1%), osteoporosis (47.2%), and portal hypertension and ascites (26.4%). The main biological features were anemia (79.2%), thrombocytopenia (50.9%), leucopenia (20.8%), and liver enzyme abnormalities (32.1%). Of the 40 patients tested, 34 (85%), 2 (5%), and 4 (10%) exhibited the KIT D816V mutant, other KIT mutations and the wild-type form of the KIT gene, respectively. Additional sequencing detected significant genetic defects in 17 of 26 (65.3%) of the patients with associated hematological non-mast cell disease, including TET2, SRSF2, IDH2, and ASLX1 mutations. Death occurred in 19 (35.8%) patients, within a median delay of 9 months, despite the different treatment options available.Mastocytosis among elderly patients has a challenging early detection, rare skin involvement, and/or limited skin disease; it is heterogeneous and has often an aggressive presentation with nonfortuitous associated myeloid lineage malignant clones, and thus a poor overall prognosis.

journal_name

Medicine (Baltimore)

journal_title

Medicine

authors

Rouet A,Aouba A,Damaj G,Soucié E,Hanssens K,Chandesris MO,Livideanu CB,Dutertre M,Durieu I,Grandpeix-Guyodo C,Barète S,Bachmeyer C,Soria A,Frenzel L,Fain O,Grosbois B,de Gennes C,Hamidou M,Arlet JB,Launay D,Lavign

doi

10.1097/MD.0000000000003901

subject

Has Abstract

pub_date

2016-06-01 00:00:00

pages

e3901

issue

eissn

0025-7974

issn

1536-5964

pii

00005792-201606140-00050

journal_volume

pub_type

杂志文章,多中心研究

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Mastocytosis among elderly patients: A multicenter retrospective French study on 53 patients.