Summary
The present study, based upon the retrospective evaluation of 352 patients with primary myelodysplastic syndrome (pMDS), revealed hypoplastic MDS in 42 patients (11.9%). Median age is similar in hypo- and normo-/hypercellular MDS (72.6 versus 70.7 versus 72.4 years). Hypoplastic MDS occurred significantly more often in women compared with normo- and hypercellular MDS. Sequential biopsies were performed in 14 patients, showing a persistence of hypoplasia over a period of up to 43 months. The proportion of patients showing mesenchymal reaction, especially an increase of mast cells, was significantly higher in hypoplastic MDS, whereas dysplastic features of hematopoiesis occurred less frequently and were of lower grade in comparison to normo-/hyperplastic MDS. Among the subgroup with hypoplastic bone marrow, the classification according to FAB criteria revealed 28 patients with RA (66.7%), three with RARS (7.1%), and eight with RAEB (19.0%), as well as one patient each with RAEB-T and CMMol (2.4% each), and one case which had to be reckoned among the category of unclassifiable MDS (2.4%). Median survival was 21.8 months for hypoplastic MDS, 26.9 months for normoplastic MDS, and 14.2 months for hyperplastic MDS. During follow-up, 14 patients (33%) with hypoplastic MDS developed acute nonlymphatic leukemia. Although not a constant finding, karyotype abnormalities involving particularly chromosome 7 seem to be associated with hypoplastic MDS. The results confirm the existence of a hypoplastic variant of MDS which seems to more frequently affect female patients, and which requires bone marrow biopsy for its accurate diagnosis.
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Appelbaum FR, Storb R, Ramberg RE, Shulman HM, Buckner CD, Clift RA, Deeg HJ, Fefer A, Sanders J, Self St, Singer J, Stewart P, Sullivan K, Witherspoon R, Thomas ED (1987) Treatment of preleukemic syndromes with marrow transplantation. Blood 69: 92–96
Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DAG, Gralnick HR, Sultan C (1976) Proposals for the classification of the acute leukemias. Br J Haematol 33: 451–458
Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DAG, Gralnick HR, Sultan C, and the French-American-British (FAB) Co-operative Group (1982) Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 51: 189–199
Bennett JM (1986) Classification of the myelodysplastic syndromes. Clin Haematol 15: 909–923
Coiffier B, Adeleine P, Viala JJ, Byron PA, Fiere D, Gentilhomme O, Vuvan H (1983) Dysmyelopoietic syndromes. A search for prognostic factors in 193 patients. Cancer 52: 8390
Cutler SJ, Ederer F (1958) Maximum utilization of the life table in analyzing survival. J Chronic Dis 55: 699–712
Damashek W (1967) Riddle: What do aplastic anemia, paroxysmal nocturnal hemoglobinuria (PNH) and “hypoplastic” leukemia have in common? Blood 30: 251–254
Delacrétaz F, Schmidt PM, Piguet D, Bachmann F, Costa J (1987) Histopathology of myelodysplastic syndromes. Am J Clin Pathol 87: 180–186
Dezza L, Cazzola M, Bergamaschi G, Burgio VL, Stella CC, Fenoglio C, Ghizza A, Nalli G, Sacchi F, Ascari E (1983) Myelodysplastic syndrome with monosomy 7 in adulthood: A distinct preleukaemic disorder. Haematologica (Pavia) 68: 723
Fohlmeister I, Fischer R, Mödder B, Riester M, Schaefer HE (1985) Aplastic anemia and the hypocellular myelodysplastic syndrome: histomorphological, diagnostic and prognostic features. J Clin Pathol 38: 1218–1224
Georgii A, Vykoupil KF, Buhr T, Choritz H, Döhler U, Kaloutsi V, Werner M (1990) Chronic myeloproliferative disorders in bone marrow biopsies. Pathol Res Pract 186: 3–27
Harnden DG, Klinger HP (eds) (1985) An international system for human cytogenetic nomenclature. Karger, Basel
Kerkhofs H, Hermans J, Haak HL, Leeksma CHW (1987) Utility of the FAB classification for myelodysplastic syndromes: investigation of prognostic factors in 237 cases. Br J Haematol 65: 73–81
Kitagawa M, Kamiyama R, Takemura T, Kasuga T (1989) Bone marrow analysis of the myelodysplastic syndromes: histological and immuno-histological features related to the evolution of overt leukemia. Virchows Arch [B] 57: 47–53
Mangi MH, Mufti GH (1992) Primary myelodysplastic syndromes: diagnostic and prognostic significance of immunohistochemical assessment of bone marrow biopsies. Blood 79: 198–205
Maschek H, Georgii A, Kaloutsi V, Werner M, Bandecar K, Kressel MG, Choritz H, Freund M, Hufnagl D (1992) Myelofibrosis in primary myelodysplastic syndromes: a retrospective study of 352 patients. Eur J Haematol 48: 208–214
Motoji T, Teramura M, Takahashi M, Oshimi K, Okada M, Kusakabe K, Mizoguchi H (1990) Successful treatment of refractory anemia with high-dose methyl-prednisolone. Am J Hematol 33: 8–12
Nand S, Godwin JE (1988) Hypoplastic myeloplastic syndrome. Cancer 62: 958–964
Planque MM de, Kluin-Nelemans HC, van Krieken JHJM, Kluin PhM, Brand A, Beverstock GC, Willemze R, van Rood JJ (1988) Evolution of acquired severe aplastic anemia to myelodysplasia and subsequent leukaemia in adults. Br J Haematol 70: 55–62
Planque MM de, van Krieken JHJM, Kluin-Nelemans HC, Colla LPJM, van der Burgh F, Brand A, Kluin PM (1989) Bone marrow histopathology of patients with severe aplastic anaemia before treatment and at follow-up. Br J Haematol 72: 439–444
Rios A, Canizo MC, Sanz MA, Vallespi T, Sanz G, Torrabadella M, Gomis F, Ruiz C, San Miguel JF (1990) Bone marrow biopsy in myelodysplastic syndromes: morphological characteristics and contribution to the study of prognostic factors. Br J Haematol 75: 26–33
Sanz GF, Sanz MA, Vallespi T, Canizo MC, Sanz G, Torrabadella M, Garcia C, Irriguible D, San Miguel JF (1989) Two regression models and a scoring system for predicting survival and planning treatment in myelodysplastic syndromes: a multivariate analysis of prognostic factors in 370 patients. Blood 74: 395–408
Tomonaga M, Tomonaga Y, Kusano M, Ichimaru M (1984) Sequential karyotypic evolutions and bone marrow aplasia preceding acute myelomonocytic transformation from myelodysplastic syndrome. Br J Haematol 58: 53–60
Tricot G, De Wolf-Peeters C, Hendrickx B, Verwilghen RL (1984) Bone marrow histology in myelodysplastic syndromes. I. Histological findings in myelodysplastic syndromes and comparison with bone marrow smears. Br J Haematol 57: 423–430
Vallespi T, Torrabadello M, Julia A, Irriguible D, Jaen A, Acebedo G, Triginer J (1985) Myelodysplastic syndromes: a study of 101 cases according to the FAB classification. Br J Haematol 61: 83–92
Vykoupil K-F, Thiele J, Georgii A (1976) Histochemical and immunohistochemical techniques on acrylate embedded bone biopsy. Blut 32: 215–218
Weide M van der, Sizoo W, Nauta JJP, Krefft J, Langenhuijsen MMAC (1988) Myelodysplastic syndromes: analysis of clinical and prognostic features in 96 patients. Eur J Haematol 41: 115–122
Yoshida Y, Oguma H, Maekawa T (1988) Refractory myelodysplastic anaemias with hypocellular bone marrow. J Clin Pathol 41: 763–767
Young NS (1992) The problem of clonality in aplastic anemia: Dr. Damashek's riddle, restated. Blood 79: 1385–1392
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Supported by grants from theDeutsche Krebshilfe, Bonn, Germany
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Maschek, H., Kaloutsi, V., Rodriguez-Kaiser, M. et al. Hypoplastic myelodysplastic syndrome: incidence, morphology, cytogenetics, and prognosis. Ann Hematol 66, 117–122 (1993). https://doi.org/10.1007/BF01697619
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DOI: https://doi.org/10.1007/BF01697619