Escolar Documentos
Profissional Documentos
Cultura Documentos
1
Projeto Diretrizes
Associação Médica Brasileira e Conselho Federal de Medicina
OBJETIVO:
Oferecer aos pacientes portadores de Anemia Aplástica Grave não constitucional,
que não tenham doadores compatíveis identificados para um transplante alogênico
de medula óssea, o melhor tratamento medicamentoso disponível, fundamentado
em dados disponíveis na literatura.
CONFLITO DE INTERESSE:
Os conflitos de interesse declarados pelos participantes da elaboração desta
diretriz estão detalhados na página 9.
DIAGNÓSTICO DIFERENCIAL
Quadro 1
Anemia aplástica grave Medula óssea com celularidade < 25%, ou 25% a 50% com
< 30% de células hematopoiéticas residuais
Anemia aplástica não grave Pacientes que não preenchem os critérios acima
Quadro 2
Algoritmo
HLA
após 6 meses
após 6 meses
OBS: Os pacientes com resposta parcial ou sem resposta após 6 meses do uso de ATG/ALG poderão realizar, a
critério médico, novo ciclo com ATG/ALG (alternado).
6. Kaufman DW, Kelly JP, Jurgelon JM, 12. Jones-Lecointe A, Murphy A, Charles W,
Anderson T, Issaragrisil S, Wiholm BE, et Daisley H, Pitt-Miller P. Severe aplastic
al. Drugs in the aetiology of agranulocytosis anaemia: complete response to anti-
and aplastic anaemia. Eur J Haematol lymphocyte globulin and cyclosporin. West
Suppl 1996;60:23-30. Indian Med J 1999;48:238-9.
7. Kelly JP, Jurgelon JM, Issaragrisil S, Keisu 13. Frickhofen N, Rosenfeld SJ. Immu-
M, Kaufman DW. An epidemiological nosuppressive treatment of aplastic anemia
with antithymocyte globulin and cyclosporine. 21. Frickhofen N, Heimpel H, Kaltwasser JP,
Semin Hematol 2000;37:56-68. Schrezenmeier H; German Aplastic
Anemia Study Group. German Aplastic
14. Young NS, Calado RT, Scheinberg P. Anemia Study Group. Antithymocyte
Current concepts in the pathophysiology globulin with or without cyclosporine A:
and treatment of aplastic anemia. Blood 11-year follow-up of a randomized trial
2006;108:2509-19. comparing treatments of aplastic anemia.
Blood 2003;101:1236-42.
15. Young N, Griffith P, Brittain E, Elfenbein
G, Gardner F, Huang A, et al. A mul- 22. Rosenfeld S, Follmann D, Nunez O,
ticenter trial of antithymocite globulin in Young NS. Antithymocyte globulin and
aplastic anemia and related diseases. Blood cyclosporine for severe aplastic anemia:
1988;72:1861-9. association between hematologic response
and long-term outcome. JAMA
2003;289: 1130-5.
16. Matloub YH, Bostrom B, Golembe B,
Priest J, Ramsay NK. Antithymocyte
23. Frickhofen N, Kaltwasser JP, Schre-
globulin, cyclosporine and prednisone for
zenmeier H, Raghavachar A, Vogt HG,
the treatment of severe aplastic anemia in
Herrmann F, et al. Treatment of aplastic
children. A pilot study. Am J Pediatr
anemia with antilymphocyte globulin and
Hematol Oncol 1994;16:104-6.
methylprednisolone with or without
cyclosporine. The German Aplastic
17. Rosenfeld SJ, Kimball J, Vining D, Young Anemia Study Group. N Engl J Med
NS. Intensive imunossupression with 1991;324: 1297-304.
antithymocyte globulin and cyclosporine as
treatment for severe acquired aplastic 24. Gluckman E, Esperou-Bourdeau H,
anemia. Blood 1995;85:3058-65. Baruchel A, Boogaerts M, Briere J,
Donadio D, et al. Multicenter randomized
18. Marsh JC. Results of immunosuppression study comparing cyclosporine A alone and
in aplastic anaemia. Acta Haematol antithymocyte globulin with prednisone for
2000;103:26-32. treatment of severe aplastic anemia. Blood
1992;79:2540-6.
19. Socié G, Gluckman E. Cure from severe
aplastic anemia in vivo and late effects. Acta 25. Novitzky N, Wood L, Jacobs P. Treatment
Haematol 2000;103:49-54. of aplastic anaemia with antilymphocyte
globulin and high-dose methylprednisolone.
20. Socié G, Rosenfeld S, Frickhofen N, Am J Hematol 1991;36:227-34.
Gluckman E, Tichelli A. Late clonal
diseases of treated aplastic anemia. Semin 26. Bacigalupo A. Aplastic anemia: patho-
Hematol 2000;37:91-101. genesis and treatment. Hematology Am Soc
Hematol Educ Program 2007;23-8. and Aplastic Anemia Study. Arch Intern Med
1989;149:1036-40.
27. Pongtanakul B, Das PK, Charpentier K,
Dror Y. Outcome of children with aplastic 29. Weinberger M. Approach to management
anemia treated with immunosuppressive of fever and infection in patients with
therapy. Pediatric Blood Cancer primary bone marrow failure and
2008;50:52-7. hemoglobinopathies. Hematol Oncol Clin
North Am 1993;7:865-85.
28. Anti-infective drug use in relation to the risk
of agranulocytosis and aplastic anemia. A 30. Brodsky RA, Jones RJ. Aplastic anaemia.
report from the International Agranulocytosis Lancet 2005;365:1647-56.