» Articles » PMID: 8558932

Refractory Cytopenia with Multilineage Dysplasia: Further Characterization of an 'unclassifiable' Myelodysplastic Syndrome

Overview
Journal Leukemia
Specialties Hematology
Oncology
Date 1996 Jan 1
PMID 8558932
Citations 7
Authors
Affiliations
Soon will be listed here.
Abstract

Myelodysplastic syndromes (MDS) characterized by multilineage cytopenias and dysplasia but lacking an increase in blasts, with no Auer rods or monocytosis, do not exactly fit any of the categories of the French-American-British (FAB) classification of MDS and are often diagnosed as refractory anemia (RA), refractory anemia with ringed sideroblasts (RARS), or 'unclassifiable' MDS. It has been suggested that these 'unclassifiable' cases form a distinct subset with a clinical behavior more like that of refractory anemia with excess of blasts (RAEB) than that of RA or RARS, but few studies have been undertaken that characterize this group. We compared the clinical, hematologic, morphologic and cytogenetic features of 18 such patients - for whose disease we propose the designation 'refractory cytopenia with multilineage dysplasia' (RCMD) - to those of 42 patients meeting the FAB criteria for RA or RARS (14 patients) and RAEB (28 patients). Our results show that cytopenias in RCMD are more severe than those in RA or RARS, but are similar to those in RAEB. Erythroid hyperplasia and dyserythropoiesis are the main findings in bone marrow specimens of RA or RARS, but the major features in RCMD are multilineage proliferation and dysplasia, which, except for the lack of increased blasts resemble the findings in RAEB. Only 1/14 patients (7%) with RA or RARS had an abnormal karyotype, whereas RCMD resembled RAEB in terms of the frequency (41 vs 50%, respectively) and type of karyotypic lesions. Abnormalities of chromosomes 5 and 7 (excluding del(5q) as an isolated finding) or complex aberrations were seen only in RCMD and RAEB. in RCMD, the median survival was 24 months, with a 4-year survival rate of48 +/- 13%, intermediate between the findings in RA/RARS (107 months and 77 +/- 12%, respectively) and RAEB (18 months and 27 +/- 9%, respectively). Our data indicate that RCMD is a distinct subset of MDS, with an unfavorable clinical outcome. The designation 'refractory cytopenia with multilineage dysplasia' emphasizes the differences between such cases and the primarily dyserythropoietic, indolent subgroups of MDS, such as RA or RARS.

Citing Articles

Diagnosis and treatment of primary myelodysplastic syndromes in adults: recommendations from the European LeukemiaNet.

Malcovati L, Hellstrom-Lindberg E, Bowen D, Ades L, cermak J, Del Canizo C Blood. 2013; 122(17):2943-64.

PMID: 23980065 PMC: 3811170. DOI: 10.1182/blood-2013-03-492884.


Prevalence of cirrhosis in patients with thrombocytopenia who receive bone marrow biopsy.

Sheikh M, Raoufi R, Atla P, Riaz M, Oberer C, Moffett M Saudi J Gastroenterol. 2012; 18(4):257-62.

PMID: 22824769 PMC: 3409887. DOI: 10.4103/1319-3767.98431.


Myelodysplastic syndromes/neoplasms: recent classification system based on World Health Organization Classification of Tumors - International Agency for Research on Cancer for Hematopoietic and Lymphoid Tissues.

Gupta G, Singh R, Kotasthane D, Kotasthane V J Blood Med. 2012; 1:171-82.

PMID: 22282696 PMC: 3262332. DOI: 10.2147/JBM.S12257.


Assessment of dysplastic hematopoiesis: lessons from healthy bone marrow donors.

Parmentier S, Schetelig J, Lorenz K, Kramer M, Ireland R, Schuler U Haematologica. 2011; 97(5):723-30.

PMID: 22180437 PMC: 3342975. DOI: 10.3324/haematol.2011.056879.


Myelodysplastic syndromes.

Odenike O, Anastasi J, Le Beau M Clin Lab Med. 2011; 31(4):763-84.

PMID: 22118747 PMC: 4340482. DOI: 10.1016/j.cll.2011.08.005.