Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia.
The natural course of chronic myelomonocytic leukemia (CMML) is highly variable but a widely accepted prognostic scoring system for patients with CMML is not available. The main aim of this study was to develop a new CMML-specific prognostic scoring system (CPSS) in a large series of 558 patients with CMML (training cohort, Spanish Group of Myelodysplastic Syndromes) and to validate it in an independent series of 274 patients (validation cohort, Heinrich Heine University Hospital, Düsseldorf, Germany, and San Matteo Hospital, Pavia, Italy). The most relevant variables for overall survival (OS) and evolution to acute myeloblastic leukemia (AML) were FAB and WHO CMML subtypes, CMML-specific cytogenetic risk classification, and red blood cell (RBC) transfusion dependency. CPSS was able to segregate patients into 4 clearly different risk groups for OS (P < .001) and risk of AML evolution (P < .001) and its predictive capability was confirmed in the validation cohort. An alternative CPSS with hemoglobin instead of RBC transfusion dependency offered almost identical prognostic capability. This study confirms the prognostic impact of FAB and WHO subtypes, recognizes the importance of RBC transfusion dependency and cytogenetics, and offers a simple and powerful CPSS for accurately assessing prognosis and planning therapy in patients with CMML.
Spanish MDS Cooperative Group, and Department of Hematology, Hospital Universitario La Fe, Valencia, Spain.
Esperanza
E
Such
Ulrich
U
Germing
Luca
L
Malcovati
José
J
Cervera
Andrea
A
Kuendgen
Matteo G
MG
Della Porta
Benet
B
Nomdedeu
Leonor
L
Arenillas
Elisa
E
Luño
Blanca
B
Xicoy
Mari L
ML
Amigo
David
D
Valcarcel
Kathrin
K
Nachtkamp
Ilaria
I
Ambaglio
Barbara
B
Hildebrandt
Ignacio
I
Lorenzo
Mario
M
Cazzola
Guillermo
G
Sanz
D000208:Acute Disease; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D017707:Erythrocyte Transfusion / Q000706:statistics & numerical data; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007951:Leukemia, Myeloid / Q000097:blood / Q000175:diagnosis / Q000235:genetics; D015477:Leukemia, Myelomonocytic, Chronic / Q000097:blood / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D017063:Outcome Assessment, Health Care / Q000379:methods* / Q000706:statistics & numerical data; D011379:Prognosis; D016016:Proportional Hazards Models; D015203:Reproducibility of Results; D018570:Risk Assessment / Q000379:methods* / Q000706:statistics & numerical data; D012307:Risk Factors; D016019:Survival Analysis; D055815:Young Adult
D006454:Hemoglobins