Thal/Dex Superior to VAD Before Transplant
Thal/Dex Superior to VAD Before Transplant
Abstract & Commentary
By Andrew S. Artz, MD, Section of Hematology/Oncology, University of Chicago. Dr. Artz reports no financial relationship to this field of study.
Synopsis: In this retrospective review of patients with MDS reclassified according to the WHO, prognostic grouping using the WHO was found to have important prognostic value. Specifically, patients with RA and RARS fare significantly better than those with multi-lineage dysplasia (RCMD). Cytogenetics, independent of WHO classification, significantly influenced survival. The development of iron overloaded among low-risk patients was associated with worse outcome. This study points to the value of both the WHO classification system and cytogenetics for MDS. RA and RARS with normal cytogenetics may identify a particularly low-risk subset of patients although monitoring for iron overload may be important.
Source: Malcovati L, et al. Prognostic Factors and Life Expectancy in Myelodysplastic Syndromes Classified According to WHO Criteria: A Basis for Clinical Decision Making. J Clin Oncol. 2005 Sep 26; [Epub ahead of print]
Myelodysplastic syndromes (MDS) encompass a heterogeneous group of clonal hematopoietic stem cell diseases characterized by dysplasia and ineffective hematopoiesis often evolving into acute myeloid leukemia. The marked prognostic variability represents a formidable challenge for clinicians weighing treatment options from supportive care to hematopoietic stem cell transplantation. In 1982, the French-American-British (FAB) cooperative group published a revised system based upon morphology.1 Subsequent efforts to refine prognostication led to the widely used International Prognostic Scoring System (IPSS).2 More recently the World Health Organization (WHO) published morphologic criteria offering refined morphologic classification, although the prognostic value of the different groups has not been well validated.
Malcovati and colleagues offer a large retrospective review evaluating prognostic value of the WHO classification system as well as other variables. From 1992 to 2002, 467 patients diagnosed with MDS using the FAB system were reclassified using the WHO criteria. Three-hundred eighty-six patients had evaluable cytogenetic data.
After reclassifying using the WHO, 16% of patients had refractory anemia (RA) and 24% had either RA or RA with ringed sideroblasts (RARS). By IPSS, 34% had low-risk disease and 39% had intermediate-1 risk disease. Refractory cytopenia with multilineage dysplasia had significantly worse survival than RA (P < .001). The IPSS continued to hold prognostic value, especially for blast count and cytogenetics. Furthermore, cytogenetics showed independent value from the WHO classification. Patients who developed iron overload had inferior survival. Even in patients with RA or RARS, survival was inferior to the general population, except for patients 70 years and older.
Commentary
Clinicians face a challenging task in patients with MDS, both in diagnosis and treatment options. This study provides additional support for the value of employing the WHO classification rather than FAB. We must work with pathology to ensure that reporting occurs using classification systems most meaningful for clinical decision making. The available data suggest that incorporating cytogenetics with the WHO classification may offer optimal prognostic use and hence direct treatment options.
While most oncologists recognize the importance of increased blast count in the marrow and the need to refer eligible patients for hematopoietic transplantation, the most important task may be separating patients with low blast count into prognostic categories. Refractory cytopenia with multi-lineage dysplasia (RCMD) and/or adverse cytogenetics identify a higher risk subgroup. Alternatively, RA or possibly RARS without adverse cytogenetics in general have more indolent disease, and in older patients suggest best supportive care represents a viable option. A caveat exists however that in lower risk patients, the development of iron overload confers a worse prognosis. In addition to minimizing transfusion, hopefully new more convenient oral iron chelating agents will offer easier options for treatment of iron overload.
References
1. Bennett JM, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol. 1982;51:189-199.
2. Greenberg P, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997;89:2079-2088; Erratum in: Blood. 1998;91:1100.
In this retrospective review of patients with MDS reclassified according to the WHO, prognostic grouping using the WHO was found to have important prognostic value. Specifically, patients with RA and RARS fare significantly better than those with multi-lineage dysplasia (RCMD). Cytogenetics, independent of WHO classification, significantly influenced survival. The development of iron overloaded among low-risk patients was associated with worse outcome. This study points to the value of both the WHO classification system and cytogenetics for MDS.Subscribe Now for Access
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