Elsevier

Leukemia Research

Volume 26, Issue 9, September 2002, Pages 821-824
Leukemia Research

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

https://doi.org/10.1016/S0145-2126(02)00021-8Get rights and content

Abstract

The clinical records of 70 patients seen at our hospital between 1976 and 1998 and diagnosed as suffering from chronic myelomonocytic leukaemia (CMML) were reviewed in order to confirm the validity of the classification into two forms of disease that the French–American–British Co-operative Leukaemia Group (FAB) proposed in 1994: myelodysplastic (MD) and myeloproliferative (MP), depending on the peripheral white blood cell count (WBC) (less or more than 13×109/l, respectively). After the rejection of incomplete records and lost to follow up patients, our study population consisted of 49 records. Our results confirm that, even though this classification is useful in order to separate two classes of patients, it is not enough to predict the prognosis in an accurate manner. A lot of studies have tried to find some prognostic factors, but the results have been discordant. The multivariate analysis of our group of patients showed three prognostic factors: serum lactate dehydrogenase (LDH) >1.5 times normal level, blasts in bone marrow >5%, and peripheral blood leukocytes >10×109/l. A second multivariate analysis led us to distinguish two groups: high risk (2–3 risk factors) and low risk (0–1 risk factors) (median survival 7 and 44 months, respectively) with a very high statistic significance (P<0.0001). This score should be applied to other series of CMML patients in order to confirm its validity.

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.

Section snippets

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

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