Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia

被引：249

作者：

Such, Esperanza ^{[1
,2
]}

Germing, Ulrich ^{[3
]}

Malcovati, Luca ^{[4
,5
]}

Cervera, Jose ^{[1
,2
]}

Kuendgen, Andrea ^{[3
]}

Della Porta, Matteo G. ^{[4
,5
]}

Nomdedeu, Benet ^{[1
,6
]}

Arenillas, Leonor ^{[1
,7
,8
]}

Luno, Elisa ^{[1
,9
]}

Xicoy, Blanca ^{[1
,10
,11
]}

Amigo, Mari L. ^{[1
,12
]}

Valcarcel, David ^{[1
,13
]}

Nachtkamp, Kathrin ^{[3
]}

Ambaglio, Ilaria ^{[4
,5
]}

Hildebrandt, Barbara ^{[14
]}

Lorenzo, Ignacio ^{[1
,2
]}

Cazzola, Mario ^{[4
,5
]}

Sanz, Guillermo ^{[1
,2
]}

机构：

[1] Hosp Univ La Fe, Spanish MDS Cooperat Grp, Valencia 46026, Spain

[2] Hosp Univ La Fe, Dept Hematol, Valencia 46026, Spain

[3] Univ Dusseldorf, Dept Hematol Oncol & Clin Immunol, D-40225 Dusseldorf, Germany

[4] Policlin San Matteo, Fdn Ist Ricovero & Cura Carattere Sci, Dept Hematol Oncol, I-27100 Pavia, Italy

[5] Univ Pavia, I-27100 Pavia, Italy

[6] Hosp Clin Barcelona, Dept Hematol, Barcelona, Spain

[7] Hosp del Mar, Serv Patol, Lab Citol Hematol, Barcelona, Spain

[8] Hosp del Mar, Res Inst, Barcelona, Spain

[9] Univ Oviedo, Hosp Cent Asturias, Dept Hematol, E-33080 Oviedo, Spain

[10] Hosp Badalona Germans Trias & Pujol, Dept Hematol, Badalona, Spain

[11] Jose Carreras Leukemia Res Inst, Inst Catala Oncol, Badalona, Spain

[12] Hosp Morales Meseguer, Dept Hematol, Murcia, Spain

[13] Hosp Univ Vall DHebron, Dept Hematol, Barcelona, Spain

[14] Univ Dusseldorf, Inst Human Genet, Dusseldorf, Germany

来源：

BLOOD | 2013年 / 121卷 / 15期

关键词：

FRENCH-AMERICAN-BRITISH; MYELODYSPLASTIC SYNDROMES; MYELODYSPLASTIC/MYELOPROLIFERATIVE NEOPLASMS; MUTATIONS; CLASSIFICATION; CMML; PROPOSALS; MACHINERY; SURVIVAL; FEATURES;

D O I：

10.1182/blood-2012-08-452938

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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, Dusseldorf, 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.

引用

页码：3005 / 3015

页数：11

共 31 条

[1] Spliceosomal gene mutations are frequent events in the diverse mutational spectrum of chronic myelomonocytic leukemia but largely absent in juvenile myelomonocytic leukemia [J].

Abu Kar, Sarah ;

Jankowska, Anna ;

Makishima, Hideki ;

Visconte, Valeria ;

Jerez, Andres ;

Sugimoto, Yuka ;

Muramatsu, Hideki ;

Traina, Fabiola ;

Afable, Manuel ;

Guinta, Kathryn ;

Tiu, Ramon V. ;

Przychodzen, Bartlomiej ;

Sakaguchi, Hirotoshi ;

Kojima, Seiji ;

Sekeres, Mikkael A. ;

List, Alan F. ;

McDevitt, Michael A. ;

Maciejewski, Jaroslaw P. .

HAEMATOLOGICA, 2013, 98 (01) :107-113

[2]

[Anonymous], 2008, WHO CLASSIFICATION T

[3] THE CHRONIC MYELOID LEUKEMIAS - GUIDELINES FOR DISTINGUISHING CHRONIC GRANULOCYTIC, ATYPICAL CHRONIC MYELOID, AND CHRONIC MYELOMONOCYTIC LEUKEMIA - PROPOSALS BY THE FRENCH-AMERICAN-BRITISH-COOPERATIVE-LEUKEMIA-GROUP [J].

BENNETT, JM ;

CATOVSKY, D ;

DANIEL, MT ;

FLANDRIN, G ;

GALTON, DAG ;

GRALNICK, H ;

SULTAN, C ;

COX, C .

BRITISH JOURNAL OF HAEMATOLOGY, 1994, 87 (04) :746-754

[4]

BENNETT JM, 1982, BRIT J HAEMATOL, V51, P189, DOI 10.1111/j.1365-2141.1982.tb08475.x

[5] Prognostic factors and risk assessment in chronic myelomonocytic leukemia: Validation study of the M. D. Anderson prognostic scoring system [J].

Beran, Miloslav ;

Wen, Sijin ;

Shen, Yu ;

Onida, Francesco ;

Jelinek, Jaroslav ;

Cortes, Jorge ;

Giles, Francis ;

Kantarjian, Hagop .

LEUKEMIA & LYMPHOMA, 2007, 48 (06) :1150-1160

[6] Myelodysplastic/Myeloproliferative Neoplasms [J].

Cazzola, Mario ;

Malcovati, Luca ;

Invernizzi, Rosangela .

HEMATOLOGY-AMERICAN SOCIETY OF HEMATOLOGY EDUCATION PROGRAM, 2011, :264-272

[7] Problems in the classification of CMML - dysplastic versus proliferative type [J].

Germing, U ;

Gattermann, N ;

Minning, H ;

Heyll, A ;

Aul, C .

LEUKEMIA RESEARCH, 1998, 22 (10) :871-878

[8] To the editor: New prognostic parameters for chronic myelomonocytic leukemia? [J].

Germing, U ;

Strupp, C ;

Alvado, M ;

Gattermann, N .

BLOOD, 2002, 100 (02) :731-732

[9] Concordance probability and discriminatory power in proportional hazards regression [J].

Gönen, M ;

Heller, G .

BIOMETRIKA, 2005, 92 (04) :965-970

[10] Two groups of chronic myelomonocytic leukaemia:: myelodysplastic and myeloproliferative.: Prognostic implications in a series of a single center [J].

González-Medina, I ;

Bueno, J ;

Torrequebrada, A ;

López, A ;

Vallespí, T ;

Massagué, I .

LEUKEMIA RESEARCH, 2002, 26 (09) :821-824

← 1 2 3 4 →