Skip to main content
Log in

Idiopathic refractory sideroachrestic anemia (IRSA) progressing to acute mixed lymphoblastic-myelomonoblastic leukemia

Case report and review of the literature

  • Original Articles
  • Published:
Blut Aims and scope Submit manuscript

    We’re sorry, something doesn't seem to be working properly.

    Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

Summary

A longitudinal observation of a patient with idiopathic refractory sideroachrestic anemia (IRSA) progressing to acute mixed lymphoblastic-myelomonoblastic leukemia is reported. The leukemia was characterized by morphology, immunological cell markers, and dissociated clinical responsiveness to vincristine/prednisone and arabinosylcytosine/6-thioguanine. Attention is paid to the hematological changes prior to leukemia development. Acute leukemia was best heralded in this patient by a severe deterioration of dyserythropoiesis and by an increase of blasts in the marrow to more than 5%. The observed preleukemic features are compared to those described in the literature.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. André R, Duhamel G, Naiman A (1970) Anémie sidéroblastique réfractaire acquise et leucémie aigue terminale. Ann Med Interne (Paris) 121: 223–230

    Google Scholar 

  2. Barry WE, Day HJ (1964) Refractory sideroblastic anemia. Clinical and hematologic study of ten cases. Ann Intern Med 61: 1029–1044

    Google Scholar 

  3. Barton JC, Conrad ME, Parmley RT (1980) Acute lymphoblastic leukemia in idiopathic refractory sideroblastic anemia: evidence for a common lymphoid and myeloid progenitor cell. Am J Hematol 9: 109–115

    Google Scholar 

  4. Ben-Bassat I, Simoni FB, Ramot B (1970) Evidence for abnormal red cell population in refractory sideroblastic anemia. Blood 35: 453–461

    Google Scholar 

  5. Bessis MC, Jensen WN (1965) Sideroblastic anemia, mitochondria, and erythroblastic iron. Br J Haematol 11: 49–51

    Google Scholar 

  6. Björkman SE (1956) Chronic refractory anemia with sideroblastic bone marrow. A study of four cases. Blood 11: 250–259

    Google Scholar 

  7. Björkman SE (1963) Prognosis and therapy of the acquired form of sideroachrestic anemia. Proc 9th Congr Europ Soc Haematol, Part II. S Karger, New York, pp 273–276

    Google Scholar 

  8. Cartwright GE, Deiss A (1975) Sideroblasts, siderocytes, and sideroblastic anemia. N Engl J Med 292: 185–193

    Google Scholar 

  9. Cartwright GE, Edwards CQ, Skolnick MH, Amos DB (1980) Association of HLA-linked hemochromatosis with idiopathic refractory sideroblastic anemia. J Clin Invest 65: 989–992

    Google Scholar 

  10. Catovsky D, Shaw MT, Hoffbrand AV, Dacie JV (1971) Sideroblastic anemia and its association with leukemia and myelomatosis: a report of five cases. Br J Haemotol 20: 385–393

    Google Scholar 

  11. Chang AR (1972) Refractory sideroblastic anemia: report on 9 cases. NZ Med J 76: 419–425

    Google Scholar 

  12. Cheng DS, Kushner JP, Wintrobe MM (1979) Idiopathic refractory sideroblastic anemia. Incidence and risk factors for leukemic transformation. Cancer 44: 724–731

    Google Scholar 

  13. Dacie JV, Smith MD, White JC, Mollin DL (1959) Refractory normoblastic anemia: a clinical and haematological study of seven cases. Br J Haematol 5: 56–82

    Google Scholar 

  14. Geschke W, Beutler E (1977) Refractory sideroblastic and non-sideroblastic anemia: a review of 27 cases. West J Med 127: 85–92

    Google Scholar 

  15. Heilmeyer L, Kleiderling W, Bilger R, Bernauer H (1958) Über chronische refraktäre Anämien mit sideroblastischem Knochenmark (Anaemia refractoria sideroblastica). Folia Haematol 2: 49–60

    Google Scholar 

  16. Heimpel H, Drings P, Mitrou P, Queisser W (1979) Verlauf und prognostische Kriterien bei Patienten mit “Präleukämie”. Ergebnisse einer prospektiven Studie. Klin Wochenschr 57: 21–29

    Google Scholar 

  17. Hines JD (1976) Effect of pyridoxine plus chronic phlebotomy on the function and morphology of bone marrow and liver in pyridoxine-responsive sideroblastic anemia. Semin Hematol 13: 133–140

    Google Scholar 

  18. Hines JD, Grasso JA (1970) The sideroblastic anemias. Semin Hematol 7: 86–106

    Google Scholar 

  19. Hoffbrand AV (1977) Sideroblastic anaemia. In: Lewis SM, Verwilghen RL (eds) Dyserythropoiesis. Academic Press, London New York San Francisco, pp 139–147

    Google Scholar 

  20. Khaleeli M, Keane WM, Lee GR (1973) Sideroblastic anemia in multiple myeloma: a preleukemic change. Blood 41: 17–25

    Google Scholar 

  21. Kitahara M, Cosgriff TM, Eyre HJ (1980) Sideroblastic anemia as a preleukemic event in patients treated for Hodgkin's disease. Ann Intern Med 92: 625–627

    Google Scholar 

  22. Koeffler HP, Golde DW (1980) Human Preleukemia. Ann Intern Med 93: 347–353

    Google Scholar 

  23. Kushner JP, Lee GR, Wintrobe MM, Cartwright GE (1971) Idiopathic refractory sideroblastic anemia. Clinical and laboratory investigation of 17 patients and review of the literature. Medicine 50: 139–159

    Google Scholar 

  24. Lewy RI, Kansu E, Gabuzda T (1979) Leukemia in patients with acquired idiopathic sideroblastic anemia: an evaluation of prognostic indicators. Am J Hematol 6: 323–331

    Google Scholar 

  25. Linman JW, Saarni MI (1974) The preluekemic syndrome. Semin Hematol 11: 93–100

    Google Scholar 

  26. MacGibbon BH, Mollin DL (1965) Sideroblastic anaemia in man: Observations on seventy cases. Br J Haematol 11: 59–69

    Google Scholar 

  27. Pierre RV (1974) Preleukemic states. Semin Hematol 11: 73–92

    Google Scholar 

  28. Prchal JT, Throckmorton DW, Carroll AJ III, Fuson EW, Gams RA, Prchal JF (1978) A common progenitor for human myeloid and lymphoid cells. Nature 274: 590–591

    Google Scholar 

  29. Riedler GF, Straub PW (1973) Abnormal iron incorporation, survival, protoporphyrin content and fluorescence of one red cell population in preleukemic sideroblastic anemia. Blood 40: 345–352

    Google Scholar 

  30. Saarni MI, Linman JW (1973) Preleukemia. The hematologic syndrome preceding acute leukemia. Am J Med 55: 38–48

    Google Scholar 

  31. Silberman S, Krmpotic E (1969) Refractory anemia with leukemic transformation and chromosomal change: a case report. Acta Haematol 41: 186–192

    Google Scholar 

  32. Streeter RR, Presant CA, Reinhard E (1977) Prognostic significance of thrombocytosis in idiopathic sideroblastic anemia. Blood 50: 427–432

    Google Scholar 

  33. Thiel E, Rodt H, Huhn D, Netzel B, Grosse-Wilde H, Ganeshaguru K, Thierfelder S (1980) Multimarker classification of acute lymphoblastic leukemia: evidence for further T-subgroups and evaluation of their clinical significance. Blood 56: 759–772

    Google Scholar 

  34. Vavra JD, Poff SA (1967) Heme and porphyrin synthesis in sideroblastic anemia. J Lab Clin Med 69: 904–918

    Google Scholar 

  35. Verloop MC, Panders JT, Ploem JE, Bos CC (1965) Sideroachrestic anemias. Ser Hematol 5: 76–87

    Google Scholar 

  36. Wintrobe MM, Lee GR, Broggs DR, Bihell TC, Athens JW, Foerster J (1982) Clinical hematology, 8 edn. Lea and Febiger, Philadelphia

    Google Scholar 

  37. Zönnchen B, Walther B (1977) Die sideroachrestischen Anämien. Inn Med 4: 235–241

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

Supported in part by the Deutsche Forschungsgemeinschaft (SFB 51)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hehlmann, R., Zönnchen, B., Thiel, E. et al. Idiopathic refractory sideroachrestic anemia (IRSA) progressing to acute mixed lymphoblastic-myelomonoblastic leukemia. Blut 46, 11–21 (1983). https://doi.org/10.1007/BF00320001

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00320001

Key words

Navigation