RT Journal Article
SR Electronic
T1 Leukemia and Myelodysplastic Syndrome in Granulomatosis with Polyangiitis: Subtypes, Clinical Characteristics, and Outcome
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 690
OP 694
DO 10.3899/jrheum.141104
VO 42
IS 4
A1 Ann Knight
A1 Karin Hjorton
A1 Christer Sundström
A1 Martin Höglund
A1 Carin Backlin
A1 Karin E. Smedby
A1 Johan Askling
A1 Eva Baecklund
YR 2015
UL http://www.jrheum.org/content/42/4/690.abstract
AB Objective. Previous studies have shown that patients with granulomatosis with polyangiitis (GPA) have an increased risk of hematological malignancies, especially leukemia. Our aim was to assess clinical characteristics and treatment of patients with GPA complicated by hematological malignancies with focus on leukemia and to describe these malignancies in more detail. Methods. From the Swedish population-based patient register, all individuals with a diagnosis of GPA from 1964–2012 were identified (n = 3224). Through linkage with the Swedish Cancer Register, we searched for all cases of leukemia [International Classification of Diseases (ICD) 7: 204–207 and corresponding codes ICD 8–10] registered after the first discharge listing GPA. The GPA diagnosis was evaluated using the European Medical Association classification algorithm. To confirm the hematological malignancy, all diagnostic bone marrow samples were reclassified. Clinical data of both the GPA and hematological malignancy were collected from medical files. Results. Twenty-one cases were identified, all of myeloid origin, including 9 with myelodysplastic syndrome developing to acute myeloid leukemia (MDS-AML), 7 AML, 3 MDS, and 2 chronic myeloid leukemia. The median time from GPA diagnosis to hematological malignancy was 8 years (range 5–21). All patients had severe generalized GPA and had received high doses of cyclophosphamide (CYC; median cumulative dose 96.5 g). Cytopenia occurred in 76% of the patients prior to the hematological malignancy. Conclusion. The findings emphasize the longterm risk of leukemia and MDS in CYC-treated, severely ill patients with GPA. Cytopenia during the course of GPA may be a warning sign and warrants a liberal attitude toward bone marrow examination.