Myelodysplastic and myeloproliferative type of chronic myelomonocytic leukemia — distinct subgroups or two stages of the same disease?

Voglová, J; Chrobák, L; Neuwírtová, R; Malasková, V; Straka, Libor

doi:10.1016/s0145-2126(00)00159-4

Cited by 53 publications

(31 citation statements)

References 8 publications

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“…[16][17][18][19] Our study confirms that it provides independent prognostic information: patients with CMML-MP had a shorter survival and higher risk of AML than patients with CMML-MD. Years later, the WHO classification moved CMML to a new category of myeloproliferative/myelodysplastic neoplasms and defined CMML-1 and CMML-2 subtypes according to the percentage of blasts in PB and BM.…”

Section: Discussionsupporting

confidence: 78%

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

Such¹,

Germing

Malcovati

et al. 2013

Blood

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Section: Discussionsupporting

confidence: 78%

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

Such¹,

Germing

Malcovati

et al. 2013

Blood

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258

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“…Although splenomegaly is quite common in the myeloproliferative disorders, it is not generally associated with MDS, with the exception of CMML, in which estimates of splenomegaly range from 17-39% [4][5][6]. However, even when splenomegaly is present, the incidence of pathologic splenic rupture in CMML is quite low; we find reports of only five cases [9][10][11][12].…”

Section: Discussionmentioning

confidence: 81%

“…Splenomegaly is thought to be more common in the myeloproliferative form of CMML [6,13], although Nosslinger et al [4] reviewed 91 cases of CMML and found rates of splenomegaly of 38% and 40% in the myelodysplastic form and myeloproliferative form respectively. Given the very low number of case reports of splenic rupture in CMML in the literature to date, it is impossible to say whether splenic rupture is more likely in one form or the other.…”

Section: Discussionmentioning

confidence: 99%

“…CMML shares features with both myelodysplastic and myeloproliferative disorders; although bone marrow dysplasia is a hallmark, myeloproliferative features such as splenomegaly are more common in CMML than in other myelodysplastic diseases [4][5][6]. There is no specific molecular or cytogenetic marker associated with CMML and its clinical features are heterogeneous.…”

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Pathological splenic rupture: A rare complication of chronic myelomonocytic leukemia

et al. 2006

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Chronic myelomonocytic leukemia (CMML) is an uncommon disorder characterized by monocytosis of the peripheral blood, absence of the Philadelphia chromosome, fewer than 20% blasts, and one or more lineages showing dysplastic features. Splenomegaly is frequently seen and may be massive. A 56-year-old man with stable CMML and moderate splenomegaly presented to the emergency department with generalized abdominal pain and abrupt drop in hemoglobin. Abdominal imaging revealed splenic rupture and emergency splenectomy was undertaken, with complete recovery. Atraumatic rupture of the spleen has rarely been reported as a complication of CMML or other myelodysplastic disorders. This report should alert physicians to consider this diagnosis in patients with CMML and acute abdominal pain. Am. J. Hematol. 82:405-408, 2007. V V C 2006 Wiley-Liss, Inc.

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“…26 Due to the variability of this presentation, in 1994 the FAB group distinguished "dysplastic" (MD-CMML) and "proliferative" (MP-CMML) variants, considering a white blood cell (WBC) count of 13x10 9 /L as a cut-off point. 27 In most of the studies investigating the prognostic significance of such a subdivision, [28][29][30][31][32][33][34] median survival of patients with the MP-CMML turned out to be shorter than that of patients with the MD-variant, even though the observed differences were hardly ever statistically significant. On the contrary, the probability of transformation to acute myeloid leukemia (AML) was generally similar between the two subtypes of CMML.…”

Section: Comprehensive Diagnostic Criteriamentioning

confidence: 99%

Management recommendations for chronic myelomonocytic leukemia: consensus statements from the SIE, SIES, GITMO groups

et al. 2013

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MethodsAn expert panel of 8 hematologists with a long experience and scientific interest in CMML was selected. During an initial meeting on December 2011, the areas of major concern in the management of CMML were identified. Clinical key-questions were generated and rank-ordered using the criterion of clinical relevance (Table 1).Each panelist drafted statements that addressed the key questions, and the remaining panelists scored their agreement with those statements providing suggestions for re-phrasing. To exploit this phase of the process, the Delphi questionnaire method was used. 4 Finally, the panel met three times in Bologna, Italy, for consensus conferences. The nominal group technique 5 was used according to which participants were first asked to comment in round-robin fashion on their preliminary votes and then to propose a new vote. Results Patient evaluation at diagnosisThe diagnosis of CMML relies largely on findings of bone With the aim of reviewing critical concepts and producing recommendations for the management of chronic myelomonocytic leukemia, key questions were selected according to the criterion of clinical relevance. Recommendations were produced using a Delphi process and four consensus conferences involving a panel of experts appointed by the Italian Society of Hematology and affiliated societies. This report presents the final statements and recommendations, covering patient evaluation at diagnosis, diagnostic criteria, risk classification, first-line therapy, monitoring, second-line therapy and allogeneic stem cell transplantation. For the first-line therapy, the panel recommended that patients with myelodysplastic-type chronic myelomonocytic leukemia and less than 10% blasts in bone marrow should be managed with supportive therapy aimed at correcting cytopenias. In patients with myelodysplastic-type chronic myelomonocytic leukemia with a high number of blasts in bone marrow (≥10%), supportive therapy should be integrated with the use of 5-azacytidine. Patients with myeloproliferative-type chronic myelomonocytic leukemia with a low number of blasts (<10%) should be treated with cytoreductive therapy. Hydroxyurea is the drug of choice to control cell proliferation and to reduce organomegaly. Patients with myeloproliferative-type chronic myelomonocytic leukemia, and a high number of blasts should receive polychemotherapy. Both in myelodysplastic-type and myeloproliferative-type chronic myelomonocytic leukemia, allogeneic stem cell transplantation should be offered within clinical trials in selected patients. Management recommendations for chronic myelomonocytic leukemia: consensus statements from the SIE, SIES, GITMO groups ABSTRACTmarrow (BM) morphological dysplasia or clonal genetic abnormalities in patients with persistent peripheral blood monocytosis. Conventional cytogenetic analysis can allow clonal abnormalities to be indentifed, even if most patients with CMML exhibit a normal karyotype. 6 The most frequent abnormalities involve chromosome 7, mostly consisting of monosomy, triso...

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Myelodysplastic and myeloproliferative type of chronic myelomonocytic leukemia — distinct subgroups or two stages of the same disease?

Cited by 53 publications

References 8 publications

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

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

Pathological splenic rupture: A rare complication of chronic myelomonocytic leukemia

Management recommendations for chronic myelomonocytic leukemia: consensus statements from the SIE, SIES, GITMO groups

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