title: Hematological malignancy associated with polymyositis and dermatomyositis.
abstract: The aims of this present study were to: 1) assess the characteristics of hematological malignancies in polymyositis/polymyositis (PM/DM) patients; and 2) determine predictive variables of hematological malignancies in PM/DM patients. We retrospectively reviewed the medical records of 32 patients (14 PM, 18 DM) associated with hematological malignancies. In our 32 PM/DM patients, hematological malignancy was concurrently identified (18.8%) or occurred during the course of PM/DM (31.2%); although, PM/DM more often preceded hematological malignancy onset (50%). We observed that the types of hematological malignancies varied, consisting of: B-cell lymphoma (n=20), T-cell lymphoma (n=4), Hodgkin's disease (n=2), multiple myeloma (n=1), myelodysplastic syndrome without excess of blasts (n=3), hairy cell (n=1) and acute lymphocytic leukemia (n=1). In 21 patients of our 32 patients with PM/DM-associated hematological malignancy (65.6% of cases), PM/DM paralleled the course of hematological malignancy. Finally, we observed that patients with PM/DM-associated hematological malignancies had a poor prognosis, the survival status ranging from 96.9%, 78.1% and 51.4% at 1, 3 and 5years, respectively. Interestingly, we found that patients with hematological malignancies, compared with those without were older and more frequently had DM; on the other hand, these patients less commonly exhibited: joint involvement (p=0.017), interstitial lung disease (p=0.06) and anti-Jo1 antibody (p=0.001). Taken together, our study underscores that the association between PM/DM and hematological malignancy, especially lymphoma, should not be ignored. Our findings also suggest that antisynthetase syndrome may be a protective factor of hematological malignancy in PM/DM patients.
authors.affiliation: Department of Internal medicine, CHU Rouen, Rouen, France. isabelle.marie@chu-rouen.fr
authors.forename: I
authors.initials: I
authors.lastname: Marie
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
authors.forename: J-F
authors.initials: JF
authors.lastname: Menard
authors.forename: P Y
authors.initials: PY
authors.lastname: Hatron
authors.forename: P
authors.initials: P
authors.lastname: Cherin
authors.forename: Z
authors.initials: Z
authors.lastname: Amoura
authors.forename: P
authors.initials: P
authors.lastname: Cacoub
authors.forename: H
authors.initials: H
authors.lastname: Bachelez
authors.forename: A
authors.initials: A
authors.lastname: Buzyn
authors.forename: G
authors.initials: G
authors.lastname: Le Roux
authors.forename: J M
authors.initials: JM
authors.lastname: Ziza
authors.forename: P
authors.initials: P
authors.lastname: Brice
authors.forename: J N
authors.initials: JN
authors.lastname: Munck
authors.forename: F
authors.initials: F
authors.lastname: Sarrot-Reynauld
authors.forename: J C
authors.initials: JC
authors.lastname: Piette
authors.forename: C
authors.initials: C
authors.lastname: Larroche
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003882:Dermatomyositis / Q000150:complications / Q000401:mortality / Q000473:pathology*; D005260:Female; D019337:Hematologic Neoplasms / Q000150:complications / Q000401:mortality / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000473:pathology; D017285:Polymyositis / Q000150:complications / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate