Skip to main content
NCBI home page
Journal of Clinical Pathology logoLink to Journal of Clinical Pathology
. 1983 Oct;36(10):1129–1135. doi: 10.1136/jcp.36.10.1129

Haematological features of primary myelodysplastic syndromes (PMDS) at initial presentation: a study of 118 cases.

S K Juneja, M Imbert, H Jouault, J Y Scoazec, F Sigaux, C Sultan
PMCID: PMC498488  PMID: 6619310

Abstract

The haematological features of 118 cases of primary myelodysplastic syndromes (PMDS) were reviewed to see how these could be related and classified according to the recent FAB proposals. A majority of the cases were elderly who presented with a macrocytic or normocytic anaemia and reticulocytopenia. Cases of acquired idiopathic sideroblastic anaemia (AISA) usually had normal leucocyte and platelet counts, erythroid hyperplasia, marked dyserythropoiesis and more than 20% ringed sideroblasts. Cases of refractory anaemia with excess of blasts (RAEB) had frequent neutropenia and thrombopenia usually with prominent dysgranulopoiesis and dysthrombopoiesis. Refractory anaemia or refractory cytopenia appeared morphologically to be a heterogeneous group. Leukaemic transformation did not occur in any of these 16 cases of AISA whereas six of the 34 cases of RAEB transformed into acute leukaemia. It appears that the cases of PMDS present with well defined haematological features which permit recognition of different groups; these latter groups appear to be morphologically and prognostically distinct.

Full text

PDF
1129

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BJORKMAN S. E. Chronic refractory anemia with sideroblastic bone marrow; a study of four cases. Blood. 1956 Mar;11(3):250–259. [PubMed] [Google Scholar]
  2. BLOCK M., JACOBSON L. O., BETHARD W. F. Preleukemic acute human leukemia. J Am Med Assoc. 1953 Jul 11;152(11):1018–1028. doi: 10.1001/jama.1953.03690110032010. [DOI] [PubMed] [Google Scholar]
  3. BOWMAN W. D., Jr Abnormal ("ringed") sideroblasts in various hematologic and non-hematologic disorders. Blood. 1961 Dec;18:662–671. [PubMed] [Google Scholar]
  4. Bennett J. M., Catovsky D., Daniel M. T., Flandrin G., Galton D. A., Gralnick H. R., Sultan C. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol. 1982 Jun;51(2):189–199. [PubMed] [Google Scholar]
  5. Bottomley S. S. Sideroblastic anaemia. Clin Haematol. 1982 Jun;11(2):389–409. [PubMed] [Google Scholar]
  6. Butler W. M., Taylor H. G., Viswanathan U. Idiopathic acquired sideroblastic anemia terminating in acute myelosclerosis. Cancer. 1982 Jun 15;49(12):2497–2499. doi: 10.1002/1097-0142(19820615)49:12<2497::aid-cncr2820491215>3.0.co;2-x. [DOI] [PubMed] [Google Scholar]
  7. Cheng D. S., Kushner J. P., Wintrobe M. M. Idiopathic refractory sideroblastic anemia: incidence and risk factors for leukemic transformation. Cancer. 1979 Aug;44(2):724–731. doi: 10.1002/1097-0142(197908)44:2<724::aid-cncr2820440245>3.0.co;2-w. [DOI] [PubMed] [Google Scholar]
  8. Classification of acute leukemia. Ann Intern Med. 1977 Dec;87(6):740–753. doi: 10.7326/0003-4819-87-6-740. [DOI] [PubMed] [Google Scholar]
  9. Coiffier B., Adeleine P., Viala J. J., Bryon P. A., Fière D., Gentilhomme O., Vuvan H. Dysmyelopoietic syndromes. A search for prognostic factors in 193 patients. Cancer. 1983 Jul 1;52(1):83–90. doi: 10.1002/1097-0142(19830701)52:1<83::aid-cncr2820520117>3.0.co;2-9. [DOI] [PubMed] [Google Scholar]
  10. DACIE J. V., SMITH M. D., WHITE J. C., MOLLIN D. L. Refractory normoblastic anaemia: a clinical and haematological study of seven cases. Br J Haematol. 1959 Jan;5(1):56–82. doi: 10.1111/j.1365-2141.1959.tb04013.x. [DOI] [PubMed] [Google Scholar]
  11. Dohy H., Genot J. Y., Imbert M., D'Agay M. F., Sultan C. Myelodysplasia and leukaemia related to chemotherapy and/or radiotherapy--a haematological study of 13 cases. Value of macrocytosis as an early sign of bone marrow injury. Clin Lab Haematol. 1980;2(2):111–119. doi: 10.1111/j.1365-2257.1980.tb00814.x. [DOI] [PubMed] [Google Scholar]
  12. Dreyfus B. Preleukemic states. I. Definition and classification. II. Refractory anemia with an excess of myeloblasts in the bone marrow (smoldering acute leukemia). Nouv Rev Fr Hematol Blood Cells. 1976;17(1-2):33–55. [PubMed] [Google Scholar]
  13. Dreyfus B., Sultan C., Rochant H., Salmon C., Mannoni P., Cartron J. P., Boivin P., Galand C. Anomalies of blood group antigens and erythrocyte enzymes in two types of chronic refractory anaemia. Br J Haematol. 1969 Mar;16(3):303–312. doi: 10.1111/j.1365-2141.1969.tb00404.x. [DOI] [PubMed] [Google Scholar]
  14. Juneja S. K., Imbert M., Sigaux F., Jouault H., Sultan C. Prevalence and distribution of ringed sideroblasts in primary myelodysplastic syndromes. J Clin Pathol. 1983 May;36(5):566–569. doi: 10.1136/jcp.36.5.566. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Kushner J. P., Lee G. R., Wintrobe M. M., Cartwright G. E. Idiopathic refractory sideroblastic anemia: clinical and laboratory investigation of 17 patients and review of the literature. Medicine (Baltimore) 1971 May;50(3):139–159. doi: 10.1097/00005792-197105000-00001. [DOI] [PubMed] [Google Scholar]
  16. Lewy R. I., Kansu E., Gabuzda T. Leukemia in patients with acquired idiopathic sideroblastic anemia: an evaluation of prognostic indicators. Am J Hematol. 1979;6(4):323–331. doi: 10.1002/ajh.2830060404. [DOI] [PubMed] [Google Scholar]
  17. Linman J. W., Bagby C., Jr The preleukemic syndrome: clinical and laboratory features, natural course, and management. Nouv Rev Fr Hematol Blood Cells. 1976;17(1-2):11–31. doi: 10.1007/978-3-642-66312-3_2. [DOI] [PubMed] [Google Scholar]
  18. Lourenço G., Embury S., Schrier S. L., Kedes L. H. Decreased ribosomal RNA content and in vitro RNA synthesis in purified bone marrow erythroblasts of patients with idiopathic ineffective erythropoiesis and DiGuglielmo disease. Am J Hematol. 1978;5(3):169–182. doi: 10.1002/ajh.2830050302. [DOI] [PubMed] [Google Scholar]
  19. Pierre R. V. Preleukemic states. Semin Hematol. 1974 Jan;11(1):73–92. [PubMed] [Google Scholar]
  20. Prchal J. T., Throckmorton D. W., Carroll A. J., 3rd, Fuson E. W., Gams R. A., Prchal J. F. A common progenitor for human myeloid and lymphoid cells. Nature. 1978 Aug 10;274(5671):590–591. doi: 10.1038/274590a0. [DOI] [PubMed] [Google Scholar]
  21. RHEINGOLD J. J., KAUFMAN R., ADELSON E., LEAR A. Smoldering acute leukemia. N Engl J Med. 1963 Apr 11;268:812–815. doi: 10.1056/NEJM196304112681504. [DOI] [PubMed] [Google Scholar]
  22. Streeter R. R., Presant C. A., Reinhard E. Prognostic significance of thrombocytosis in idiopathic sideroblastic anemia. Blood. 1977 Sep;50(3):427–432. [PubMed] [Google Scholar]
  23. Sultan C., Sigaux F., Imbert M., Reyes F. Acute myelodysplasia with myelofibrosis: a report of eight cases. Br J Haematol. 1981 Sep;49(1):11–16. doi: 10.1111/j.1365-2141.1981.tb07191.x. [DOI] [PubMed] [Google Scholar]
  24. Wickramasinghe S. N., Hughes M. Capacity of ringed sideroblasts to synthesize nucleic acids and protein in patients with primary acquired sideroblastic anaemia. Br J Haematol. 1978 Mar;38(3):345–352. doi: 10.1111/j.1365-2141.1978.tb01053.x. [DOI] [PubMed] [Google Scholar]
  25. Yeung K., Trowbridge A. A. Idiopathic acquired sideroblastic anemia terminating in acute myelofibrosis: case report and review of leterature. Cancer. 1977 Jan;39(1):359–365. doi: 10.1002/1097-0142(197701)39:1<359::aid-cncr2820390155>3.0.co;2-c. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Clinical Pathology are provided here courtesy of BMJ Publishing Group

RESOURCES