medigraphic.com

2016, Number 2

<< Back Next >>

Rev Hematol Mex 2016; 17 (2)

Acquired aplastic anemia: a demographic, clinical, and therapeutic survey of a single institution in Mexico City

Gutiérrez-Serdán R, López-Karpovitch X

Language: English
References: 28
Page: 81-89
PDF size: 400.31 Kb.


ABSTRACT

Background: Acquired aplastic anemia (AA) pathophysiology involves immune mechanisms. Risk classifications to stratify AA severity are employed to define treatment.
Objetive: To analyze therapeutic response and survival in patients with AA.
Patients and Method: A retrospective study was done in which diagnosis and therapy response were establish following 2009 AA British guidelines. Data collected from patients admitted between January 1998 and December 2007 were analyzed.
Results: In the study period 51 patients were identified. At diagnosis 2 of 19 cases had paroxysmal nocturnal hemoglobinuria clones. Median age in the remainder patients (22 females and 27 males) was 35 years (range 17 to 78 years). Eleven, 28 and 10 patients had non-severe, severe, and very severe AA, respectively. Seven patients with severe AA received bone marrow transplantation (BMT). All of them remain in complete response (CR) with a median follow-up of 1,675 days. Median survival in non-BMT patients (n=42) with nonsevere, severe, and very severe AA was 1,253, 895, and 447 days, respectively (p‹0.001). Forty patients received immunosuppressive therapy and androgens. Overall response (CR+PR; partial response) with immunosuppressive therapy and androgens was 51% and 38.5%, respectively. Overall response was significantly higher in BMT patients than in those treated with immunosuppressive therapy and androgens (p=0.002). No statistically significant difference in overall response was recorded between patients who received immunosuppression and androgens. Median survival in non-BMT patients with CR (1,577 days), PR (1,213 days) and no response (408 days) was statistically significant different (p‹0.02).
Conclusions: Long standing classifications are still useful to stratify survival and therapy response in AA. BMT remains the best therapeutic option, and seemingly immunosuppression and androgens render similar response rates in AA.


REFERENCES

  1. Marsh JCW, Ball SE, Cavenagh J, et al. Guidelines for the diagnosis and management of aplastic anemia. Br J Haematol 2009;147:43-70.

  2. Wanachiwanawin W, Siripannyaphinyo U, Piyawattanasakul N, Kinoshita T. A cohort study of the nature of paroxysmal nocturnal hemoglobinuria clones and PIG-A mutations in patients with aplastic anemia. Eur J Haematol 2006;76:502-509.

  3. Szklo M, Sensenbrenner L, Markowitz J, Weida S, et al. Incidence of aplastic anemia in metropolitan Baltimore: a population-based study. Blood 1985;66:115-119.

  4. International Agranulocytosis and Aplastic Anaemia study. Incidence of aplastic anaemia: the relevance of diagnostic criteria. Blood 1987;70:1718-1721.

  5. Cartwright RA, McKinney PA, Williams L, et al. Aplastic anaemia incidence in parts of the United Kingdom in 1985. Leuk Res 1988;12:459-463.

  6. Mary JY, Baumelou E, Guiquet M. Epidemiology of aplastic anemia in France: a prospective multicentric study. The French Cooperative Group for Epidemiological Study of Aplastic Anemia. Blood 1990;75:1646-1653.

  7. Clausen N, Kreuger A, Salmi T, Storm-Mathisen I, Johannesson G. Severe aplastic anaemia in the Nordic countries: a population based study of incidence, presentation, course, and outcome. Arch Dis Child 1996;74:319-322.

  8. Maluf EMCP, Pasquini R, Eluf JN, Kelly J, Kaufman DW. Aplastic anemia in Brazil : Incidence and risk factors. Am J Hematol 2002;71:268-274.

  9. Montané E, Ibáñez L, Vidal X, et al. The Catalan Group for the Study of Agranulocytosis and Aplastic Anemia. Epidemiology of aplastic anemia: a prospective multicenter study. Haematologica 2008;93:518-523.

  10. Yang C, Zhang X. Incidence survey of aplastic anemia in China. Chin Med Sci J 1991;6:203-207.

  11. Yong AS, Goh AS, Rahman M, Menon J, Purushothaman V. Epidemiology of aplastic anemia in the state of Sabah, Malaysia. Med J Malaysia 1998;53:59-62.

  12. Benítez-Aranda H, Vélez-Ruelas MA, Díaz-Cárdenas S, et al. Incidence of aplastic anemia in a defined subpopulation from Mexico City. Hematology 2002;7:229-232.

  13. Issaragrisil S, Kaufman DW, Anderson T, et al. The epidemiology of aplastic anemia in Thailand. Blood 2006;107:1299-1307.

  14. Davies SM, Walker DJ. Aplastic anaemia in the Northern Region 1971-1978 and follow-up of long term survivors. Clin Lab Haematol 1986;8:307-313.

  15. Young NS, Calado RT, Scheinberg P. Current concepts in the pathophysiology and treatment of aplastic anemia. Blood 2006;108:2509-2519.

  16. Camitta BM, Thomas ED, Nathan DG, et al. A prospective study of androgens and bone marrow transplantation for treatment of severe aplastic anemia. Blood 1979;53:504-514.

  17. Shahidi NT, Diamond LK. Testosterone-induced remission in aplastic anemia of both acquired and congenital types: further observations in 24 cases. N Engl J Med 1961;264:953-967.

  18. Sánchez-Medal L, Gómez-Leal A, Duarte L, Rico MG. Anabolic androgenic steroids in the treatment of acquired aplastic anemia. Blood 1969;34:283-300.

  19. Bacigalupo A, Hows J, Gluckman E, et al. Bone marrow transplantation (BMT) versus immunosuppression for the treatment of severe aplastic anemia (SAA): A report of the EBMT SAA working party. Br J Haematol 1988;70:177-182.

  20. Piedras J, López-Karpovitch X. Flow cytometric analysis of glycosylphosphatidil-inositol-anchored proteins to assess paroxysmal nocturnal hemoglobinuria clone size. Cytometry 2000:42:234-238.

  21. Camitta BM. What is the definition of cure for aplastic anemia? Acta Haematol 2000;103:16-18.

  22. Maciejewski JP, Rivera C, Kook H, Dunn D, Young NS. Relationship between bone marrow failure syndromes and the presence of glycophosphatidyl inositol-anchored proteindeficient clones. Br J Haematol 2001;115:1015-1022.

  23. Scheinberg P, Marte M, Nunez O, Young NS. Paroxysmal nocturnal hemoglobinuria clones in severe aplastic anemia patients with horse anti-thymocyte globulin plus cyclosporine Haematologica 2010;95:1075-1080.

  24. Delgado‑Lamas JL, López‑Karpovitch X, Marín‑López A, et al. Low‑dose of high‑potency antithymocyte globulin (ATG) in severe aplastic anemia: Experience with the Mexican ATG. Acta Haematol 1989;81:70‑74.

  25. Gluckman E, Esperou-Bordeau H, Baruchel A, et al. Multicenter randomized study comparing cyclosporine-A alone and antithymocyte globulin with prednisone for treatment of sever aplastic anemia. Blood 1992;79:2540- 2546.

  26. Chuhjo T, Yamazaki H, Mitsushiro O, Nakao S. Danazol therapy for aplastic anemia refractory to immunosuppessive therapy. Am J Hematol 2008;83:387-389.

  27. Jaime-Pérez JC, Colunga-Pedraza PR, Gómez-Ramírez CD, et al. Danazol as first-line therapy for aplastic anemia. Ann Hematol 2011;50:523-527.

  28. Sheinber P, Wu C, el al. Predicting response to immunosuppressive therapy and survival in severe aplastic anemia. Br J Hematol 2006;144;206-216.




2020     |     www.medigraphic.com