Plasma Cell Leukemia: Impact of Cytogenetic Profile on Prognosis
Tariq Muzzafar, Surabhi Kaul, Jose M Gonzalez-Berjon, Jatin Shah. Universitiy of Texas MD Anderson Cancer Center, Houston, TX
Background: Plasma cell leukemia (PCL) is a rare, highly aggressive plasma cell (PC) neoplasm that can present de novo (primary PCL) or as transformation of PC myeloma (secondary PCL). WHO criteria require a > 2000/μL or 20% plasma cells in peripheral blood. Overall median survival is 7 - 11 months. We aimed to characterize the cytogenetic features of this disease and analyze their impact on survival.
Design: We identified 37 cases of PCL in our institutional files from 01/2003 to 09/2012. Demographic, cytogenetic (karyotypes and fluorescent in situ hybridization [FISH]) studies and survival data were collected.
Results: Karyotypes were available for 29 cases out of 37 cases. Of these 16/33 (48%) 13/33were hypodiploid, (39%) were pseudo/diploid and 4/33 (12%) were hyperdiploid. Of cases with FISH studies available, 5/15 (33%) had TP53 deletion, 11/15 (73%) had RB1 deletion, 3/7 (43%) had CCND1 rearrangement. Nine patients were treated with hyper CVAD (cyclophosphamide, vincristine, doxorubicin and dexamethasone) and 12 with regimen including bortezomib. Overall median survival was 106 weeks. TP53 deletion, RB deletion, hypodiploidy, hyperdiploidy or diploid/pseudodiploidy had no impact on survival.
Conclusions: Cases with PCL in our cohort appear to have better survival as compared to patients reported in literature previously. Newer treatment regimens may be responsible for the improved prognosis. TP53 deletion, RB deletion, hypodiploidy, hyperdiploidy or diploid/pseudodiploidy had no impact on survival.
Wednesday, March 6, 2013 1:00 PM
Poster Session VI # 223, Wednesday Afternoon