Primary plasma cell leukemia: a retrospective multicenter study of 73 patients.
BACKGROUND
Epidemiological and clinical information on primary plasma cell leukemia (pPCL) are rarely reported. The aims are to evaluate the clinical features, prognostic factors, and efficacy of treatments in pPCL.


METHODS
A multicenter retrospective cohort study was carried out from January 2000 to December 2008 in 26 Italian hematology divisions. A total of 128 cases of plasma cell leukemia were collected, and 73 of them (57%) were classified as primary (male/female 43/30).


RESULTS
Sixty-four patients had at least 1 sign of end-organ damage and 10 had extramedullary localization. One patient died early; of the remaining patients, 36 (50%) received anthracycline-based regimens as first-line therapy, 17 (24%) single alkylating agents, and 30 (42%) bortezomib or thalidomide as additional (n = 11) or unique treatments (n = 19). Twenty-three patients (31%) underwent autologous and/or allogeneic hematopoietic stem cell transplantation (HSCT). The median overall survival (OS) was 12.6 months; complete or partial response was achieved in 22 (30%) and 18 patients (25%), respectively; the median duration of response (DOR) was 16.4 months. HSCT patients had a longer OS and DOR (median 38.1 and 25.8 months, respectively) compared with nontransplanted patients (9.1 and 7.3 months, respectively, P < 0.001). OS was influenced by nonresponse to treatment, hypoalbuminemia, and HSCT. DOR was favorably influenced only by HSCT.


CONCLUSIONS
pPCL is an aggressive disease with a poor prognosis and a low response rate to conventional therapy. HSCT is effective, increasing OS and DOR by 69% and 88%, respectively. The use of bortezomib and thalidomide may improve outcomes.
Institute of Hematology, Catholic University, Rome. Electronic address: lpagano@rm.unicatt.it.
L
L
Pagano
Institute of Hematology, Catholic University, Rome.
C G
CG
Valentini
Institute of Hematology, Catholic University, Rome.
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V
De Stefano
Division of Hematology, "Tor Vergata" University, Rome.
A
A
Venditti
Hematology and Hematopoietic Stem Cell Transplant Center, San Salvatore Hospital, Pesaro.
G
G
Visani
Division of Hematology, Department of Cellular Biotechnologies and Hematology, University "La Sapienza," Rome.
M T
MT
Petrucci
Division of Hematology and Bone Marrow Transplantation, University of Udine, Udine.
A
A
Candoni
Hematology Section, University of Bari, Bari.
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Specchia
Department of Hematology, Ospedale San Bortolo, Vicenza.
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C
Visco
Department of Hematology, "San Gerardo" Hospital, Monza.
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EM
Pogliani
Division of Hematology and Stem Cell Transplantation Unit, Cardarelli Hospital, Naples.
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F
Ferrara
Division of Hematology, Ascoli Piceno Hospital, Ascoli Piceno.
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P
Galieni
Department of Medicine and Immunological Sciences, Division of Hematology and Transplants, University of Siena, Siena.
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A
Gozzetti
Institute of Hematology, Catholic University, Rome.
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L
Fianchi
Division of Hematology, Campus Biomedico University, Rome.
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M
De Muro
Institute of Hematology, Catholic University, Rome.
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G
Leone
Unit of Hematology and Stem Cell Transplantation, IRCCS-CROB, Rionero in Vulture, Italy.
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P
Musto
Division of Hematology, Department of Cellular Biotechnologies and Hematology, University "La Sapienza," Rome.
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Pulsoni
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018943:Anthracyclines / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D015331:Cohort Studies; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007952:Leukemia, Plasma Cell / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000628:therapy*; D011719:Pyrazines / Q000008:administration & dosage; D012189:Retrospective Studies; D015996:Survival Rate; D013792:Thalidomide / Q000008:administration & dosage; D014182:Transplantation, Autologous; D016896:Treatment Outcome; D055815:Young Adult
D018943:Anthracyclines; D001897:Boronic Acids; D011719:Pyrazines; D013792:Thalidomide; D000069286:Bortezomib