Two groups of chronic myelomonocytic leukaemia: myelodysplastic and myeloproliferative. Prognostic implications in a series of a single center
Introduction
In two Leukaemia Research recent issues Voglová et al. [1] and Nösslinger et al. [2] try to solve the problem of the classification of chronic myelomonocytic leukaemia (CMML) and they reach two opposite conclusions: Voglová et al. propose that CMML should not be divided in myelodysplastic (MD) and myeloproliferative (MP) types and Nösslinger et al. suggest that segregation into MD and MP types is justified. Here, we will review our data in order to find out the definitive conclusion.
CMML was defined by the French–American–British Co-operative Leukaemia Group (FAB) [3] as a myelodysplastic syndrome (MDS) characterized by peripheral monocytosis >1×109/l, appearance of monocytic cells in the bone marrow, dysplasia in the erythroid, megakaryocytic or granulocytic lines and <5% circulating blasts, and 30% bone marrow blasts. The inclusion into the group of MDS is justified if we bear in mind that CMML presents a cytological expression very similar to refractory anaemia with excess of blasts (RAEB), with promonocytes, promyelocites and many dyshemopoietic morphologic signs [4]. However, compared with other MDS, CMML tends to exhibit a higher count of total white blood cell count (WBC) and myeloid precursors in peripheral blood and shows enlarged liver and spleen; moreover increased levels of lactate dehydrogenase (LDH) and uric acid are frequent features [5]. By these reasons the most recent World Health Organization (WHO) Classification of Neoplastic Diseases of the Hematopoietic and Lymphoid Tissues included this disease in a hybrid MD/MP group [6].
In 1994, the FAB group [7] could distinguish two types of CMML: one of them MP (leukocytes>13×109/l) and the other with MD characteristics (leukocytes<13×109/l).
We present the experience of one single center with a series of 70 patients with CMML, diagnosed and treated in our hospital, in order to test whether this classification into two varieties is useful for diagnosis, treatment and prognosis of this entity.
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Patients and methods
Seventy patients were diagnosed as suffering from CMML in our institution, between 1976 and 1998. We applied the diagnostic criteria proposed by the FAB group [3]. Lost to follow-up patients and incomplete clinical records were excluded and, therefore, the population was reduced to 49 patients. Thirty clinical and biological variables were studied: age, sex, ECOG, spleen and liver size, skin infiltration, pleural and peritoneal effusions, serum LDH level, leukocyte alkaline phosphatase index,
Clinical features
Table 1 shows the most relevant clinical data. Median age was 71 years (range 23–92). Forty-one patients (84%) were older than 60 years. At diagnosis 8 patients presented splenomegaly and 15 patients hepatomegaly.
Laboratory data
Peripheral blood data are presented in Table 1: >51% patients had a very important anaemia (Hb<100 g/l) and 65% patients presented thrombocytopenia (< 120×109/l), but the platelet count was < 25×109/l in only five patients. The proliferative type of CMML (WBC>13×109/l) was seen in 65% of
Discussion
CMML is a disease whose classification presents a lot of problems because of its clinical picture and its evolution [5], [10], [11], [12]. Several patients have minimal leucocytosis with monocytosis, but others show a very important leucocytosis with extramedulary haemopoiesis (reflected in splenomegaly, skin infiltration and serious pleural and peritoneal effusions) that brings the disease closer to myeloproliferative syndromes (MPS). Sometimes the clinical course is slow, with a very long
References (21)
- et al.
Myelodysplastic and myeloproliferative type of chronic myelomonocytic leukaemia. Distinct subgroups or two stages of the same disease?
Leukaemia Res.
(2001) - et al.
Dysplastic versus proliferative CMML. A retrospective analysis of 91 patients from a single institution
Leukaemia Res.
(2001) - et al.
Problems in the classifications of CMML. Dysplastic versus proliferative type
Leukaemia Res.
(1998) - et al.
Chronic myelomonocytic leukaemia: natural history and prognostic determinants
Mayo. Clin. Proc.
(1989) - et al.
Proposals for the classification of the myelodysplastic syndromes
Br. J. Haematol.
(1982) - et al.
La denominada leucemia mielomonocı́tica crónica, una forma de expresión de la anemia refractaria con mieloblastosis parcial
Sangre
(1976) - et al.
Chronic myelomonocytic leukaemia. Clinicobiological characteristics: a multivariate analysis in a series of 70 cases
Eur. J. Haematol.
(1989) - et al.
World Health Organization Classification of Neoplastic Diseases of the Hematopoietic and Lymphoid Tissues: Report of the Clinical Advisory Committee Meeting—Airlie House Virginia, November 1997
J. Clin. Oncol.
(1999) - et al.
The chronic myeloid leukaemias: guidelines for distinguishing chronic granulocytic, atypical chronic myeloid, and chronic myelomonocytic leukaemia. Proposals by the French–American–British Co-operative Leukaemia Group
Br. J. Haematol.
(1994) - et al.
Nonparametric estimation from incomplete observations
J. Am. Stat. Assoc.
(1958)
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2017, Clinical Lymphoma, Myeloma and LeukemiaCitation Excerpt :Because of the recognized heterogeneity in CMML outcomes, risk stratification is pertinent to determining the therapeutic strategy for patients. Multiple risk stratification tools have been developed for CMML in attempts to best prognosticate for this disease.11-18 Although the International Prognostic Scoring System developed for MDS can be used, the system applies only to patients with proliferative CMML.5