eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Aplastic Anemia: Follow-up

Author: Sameer Bakhshi, MD, Associate Professor of Pediatric Oncology, Department of Medical Oncology, Dr BRA Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, India
Coauthor(s): Esteban Abella, MD, Consulting Staff, Arizona Pediatric Hematology/Oncology, PLLC
Contributor Information and Disclosures

Updated: Oct 4, 2009

Follow-up

Further Inpatient Care

  • Inpatient care for patients with aplastic anemia may be needed during periods of infection and for specific therapies, such as ATG or BMT.

Further Outpatient Care

  • Frequent outpatient follow-up of patients with aplastic anemia is needed to monitor blood counts and adverse effects of various drugs.
  • Transfusions of packed RBCs and platelets are administered on an outpatient basis.

Inpatient & Outpatient Medications

  • The specific medications administered depend on the choice of therapy and whether it is supportive care only, immunosuppressive therapy, or BMT.

Transfer

  • Patients with aplastic anemia should be treated by physicians who are experts in the care of immunocompromised patients and in consultation with a BMT physician for patients younger than 65 years.

Complications

  • Complications of aplastic anemia
    • Infections
    • Bleeding
  • Complications of BMT
    • GVHD
    • Graft failure

Prognosis

  • The outcome of patients with aplastic anemia has substantially improved because of improved supportive care. The natural history of aplastic anemia suggests that as many as one fifth of patients may spontaneously recover with supportive care; however, observational and/or supportive care therapy alone is rarely indicated.
  • The estimated 5-year survival rate for the typical patient receiving immunosuppression is 75%. The rate for those receiving a BMT from a matched sibling donor is greater than 90%. However, in case of immunosuppression, relapse and late clonal disease are risks.

Patient Education

  • Patients should maintain hygiene to reduce the risks of infection.
  • Clinicians must stress the need for compliance with therapy.

Miscellaneous

Medicolegal Pitfalls

  • Failure to correctly diagnose aplastic anemia and initiate appropriate treatment is a pitfall.
  • Aplastic anemia has a >70% mortality rate with supportive care alone. It is a hematologic emergency, and care should be instituted promptly.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Dr Roy Baynes to the development and writing of this article.



More on Aplastic Anemia

Overview: Aplastic Anemia
Differential Diagnoses & Workup: Aplastic Anemia
Treatment & Medication: Aplastic Anemia
Follow-up: Aplastic Anemia
Multimedia: Aplastic Anemia
References
Further Reading
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References

  1. Wu Y, Yu J, Zhang L, et al. [Hematopoiesis support of mesenchymal stem cells in children with aplastic anemia] [Chinese]. Zhongguo Dang Dai Er Ke Za Zhi. Aug 2008;10(4):455-9. [Medline].

  2. Scopes J, Daly S, Atkinson R, et al. Aplastic anemia: evidence for dysfunctional bone marrow progenitor cells and the corrective effect of granulocyte colony-stimulating factor in vitro. Blood. Apr 15 1996;87(8):3179-85. [Medline]. [Full Text].

  3. Young NS. Pathophysiologic mechanisms in acquired aplastic anemia. Hematology Am Soc Hematol Educ Program. 2006;72-7. [Medline]. [Full Text].

  4. Liu H, Mihara K, Kimura A, Tanaka K, Kamada N. Induction of apoptosis in CD34+ cells by sera from patients with aplastic anemia. Hiroshima J Med Sci. Jun 1999;48(2):57-63. [Medline].

  5. Solomou EE, Keyvanfar K, Young NS. T-bet, a Th1 transcription factor, is up-regulated in T cells from patients with aplastic anemia. Blood. May 15 2006;107(10):3983-91. [Medline]. [Full Text].

  6. Socié G, Rosenfeld S, Frickhofen N, Gluckman E, Tichelli A. Late clonal diseases of treated aplastic anemia. Semin Hematol. Jan 2000;37(1):91-101. [Medline].

  7. Nakao S. Immune mechanism of aplastic anemia. Int J Hematol. Aug 1997;66(2):127-34. [Medline].

  8. Frickhofen N, Kaltwasser JP, Schrezenmeier H, et al. Treatment of aplastic anemia with antilymphocyte globulin and methylprednisolone with or without cyclosporine. The German Aplastic Anemia Study Group. N Engl J Med. May 9 1991;324(19):1297-304. [Medline].

  9. Passweg JR, Pérez WS, Eapen M, et al. Bone marrow transplants from mismatched related and unrelated donors for severe aplastic anemia. Bone Marrow Transplant. Apr 2006;37(7):641-9. [Medline]. [Full Text].

  10. Bacigalupo A, Brand R, Oneto R, et al. Treatment of acquired severe aplastic anemia: bone marrow transplantation compared with immunosuppressive therapy--The European Group for Blood and Marrow Transplantation experience. Semin Hematol. Jan 2000;37(1):69-80. [Medline].

  11. Solomou EE, Gibellini F, Stewart B, et al. Perforin gene mutations in patients with acquired aplastic anemia. Blood. Jun 15 2007;109(12):5234-7. [Medline]. [Full Text].

  12. Chan KW, McDonald L, Lim D, et al. Unrelated cord blood transplantation in children with idiopathic severe aplastic anemia. Bone Marrow Transplant. Aug 11 2008;epub ahead of print. [Medline].

  13. Bunin N, Aplenc R, Iannone R, et al. Unrelated donor bone marrow transplantation for children with severe aplastic anemia: minimal GVHD and durable engraftment with partial T cell depletion. Bone Marrow Transplant. Feb 2005;35(4):369-73. [Medline].

  14. Kang HJ, Shin HY, Choi HS, Ahn HS. Fludarabine, cyclophosphamide plus thymoglobulin conditioning regimen for unrelated bone marrow transplantation in severe aplastic anemia. Bone Marrow Transplant. Dec 2004;34(11):939-43. [Medline].

  15. Brodsky RA, Sensenbrenner LL, Smith BD, et al. Durable treatment-free remission after high-dose cyclophosphamide therapy for previously untreated severe aplastic anemia. Ann Intern Med. Oct 2 2001;135(7):477-83. [Medline].

  16. Horowitz MM. Current status of allogeneic bone marrow transplantation in acquired aplastic anemia. Semin Hematol. Jan 2000;37(1):30-42. [Medline].

  17. Kaito K, Kobayashi M, Katayama T, et al. Long-term administration of G-CSF for aplastic anaemia is closely related to the early evolution of monosomy 7 MDS in adults. Br J Haematol. Nov 1998;103(2):297-303. [Medline].

  18. Piaggio G, Podestà M, Pitto A, et al. Coexistence of normal and clonal haemopoiesis in aplastic anaemia patients treated with immunosuppressive therapy. Br J Haematol. Dec 1999;107(3):505-11. [Medline].

  19. Rosti V. The molecular basis of paroxysmal nocturnal hemoglobinuria. Haematologica. Jan 2000;85(1):82-7. [Medline]. [Full Text].

  20. Orazi A, Czader MB. Myelodysplastic syndromes. Am J Clin Pathol. Aug 2009;132(2):290-305. [Medline].

  21. Breakey VR, Meyn S, Ng V, Allen C, Dokal I, Lansdorp PM, et al. Hepatitis-associated Aplastic Anemia Presenting as a Familial Bone Marrow Failure Syndrome. J Pediatr Hematol Oncol. Sep 23 2009;[Medline].

  22. Gonzalez-Casas R, Garcia-Buey L, Jones EA, Gisbert JP, Moreno-Otero R. Systematic review: hepatitis-associated aplastic anaemia--a syndrome associated with abnormal immunological function. Aliment Pharmacol Ther. Sep 1 2009;30(5):436-43. [Medline].

  23. Camitta B, O'Reilly RJ, Sensenbrenner L, et al. Antithoracic duct lymphocyte globulin therapy of severe aplastic anemia. Blood. Oct 1983;62(4):883-8. [Medline]. [Full Text].

  24. Valdez JM, Scheinberg P, Young NS, Walsh TJ. Infections in patients with aplastic anemia. Semin Hematol. Jul 2009;46(3):269-76. [Medline].

  25. Jancel T, Penzak SR. Antiviral therapy in patients with hematologic malignancies, transplantation, and aplastic anemia. Semin Hematol. Jul 2009;46(3):230-47. [Medline].

  26. Di Bona E, Rodeghiero F, Bruno B, et al. Rabbit antithymocyte globulin (r-ATG) plus cyclosporine and granulocyte colony stimulating factor is an effective treatment for aplastic anaemia patients unresponsive to a first course of intensive immunosuppressive therapy. Gruppo Italiano Trapianto di Midollo Osseo (GITMO). Br J Haematol. Nov 1999;107(2):330-4. [Medline].

  27. Bacigalupo A, Broccia G, Corda G, et al. Antilymphocyte globulin, cyclosporin, and granulocyte colony-stimulating factor in patients with acquired severe aplastic anemia (SAA): a pilot study of the EBMT SAA Working Party. Blood. Mar 1 1995;85(5):1348-53. [Medline]. [Full Text].

  28. Dufour C, Ferretti E, Bagnasco F, Burlando O, Lanciotti M, Ramenghi U, et al. Changes of cytokine profile pre and post immunosuppression in acquired aplastic anemia. Haematologica. Jul 7 2009;[Medline].

  29. Maschan A, Bogatcheva N, Kryjanovskii O, et al. Results at a single centre of immunosuppression with cyclosporine A in 66 children with aplastic anaemia. Br J Haematol. Sep 1999;106(4):967-70. [Medline].

  30. Marsh J, Schrezenmeier H, Marin P, et al. Prospective randomized multicenter study comparing cyclosporin alone versus the combination of antithymocyte globulin and cyclosporin for treatment of patients with nonsevere aplastic anemia: a report from the European Blood and Marrow Transplant (EBMT) Severe Aplastic Anaemia Working Party. Blood. Apr 1 1999;93(7):2191-5. [Medline]. [Full Text].

  31. Scheinberg P, Wu CO, Nunez O, Young NS. Long-term outcome of pediatric patients with severe aplastic anemia treated with antithymocyte globulin and cyclosporine. J Pediatr. Jul 29 2008;epub ahead of print. [Medline].

  32. Jiang S, Wang Y, Shi W, Shao Y, Qiao X, Lin J, et al. The benefit of ATG in immunosuppressive therapy of children with moderate aplastic anemia. Pediatr Hematol Oncol. Jul-Aug 2009;26(5):313-20. [Medline].

  33. Locatelli F, Bruno B, Zecca M, et al. Cyclosporin A and short-term methotrexate versus cyclosporin A as graft versus host disease prophylaxis in patients with severe aplastic anemia given allogeneic bone marrow transplantation from an HLA-identical sibling: results of a GITMO/EBMT randomized trial. Blood. Sep 1 2000;96(5):1690-7. [Medline]. [Full Text].

  34. George B, Mathews V, Viswabandya A, et al. Fludarabine and cyclophosphamide based reduced intensity conditioning (RIC) regimens reduce rejection and improve outcome in Indian patients undergoing allogeneic stem cell transplantation for severe aplastic anemia. Bone Marrow Transplant. Jul 2007;40(1):13-8. [Medline].

  35. Maury S, Balère-Appert ML, Chir Z, et al. Unrelated stem cell transplantation for severe acquired aplastic anemia: improved outcome in the era of high-resolution HLA matching between donor and recipient. Haematologica. May 2007;92(5):589-96. [Medline]. [Full Text].

  36. Kojima S, Matsuyama T, Kato S, et al. Outcome of 154 patients with severe aplastic anemia who received transplants from unrelated donors: the Japan Marrow Donor Program. Blood. Aug 1 2002;100(3):799-803. [Medline]. [Full Text].

  37. Ades L, Mary JY, Robin M, et al. Long-term outcome after bone marrow transplantation for severe aplastic anemia. Blood. Apr 1 2004;103(7):2490-7. [Medline]. [Full Text].

  38. de Planque MM, Bacigalupo A, Würsch A, et al. Long-term follow-up of severe aplastic anaemia patients treated with antithymocyte globulin. Severe Aplastic Anaemia Working Party of the European Cooperative Group for Bone Marrow Transplantation (EBMT). Br J Haematol. Sep 1989;73(1):121-6. [Medline].

  39. Koh LP, Koh MB, Ng HY, et al. Allogeneic hematopoietic stem cell transplantation for patients with severe aplastic anemia following nonmyeloablative conditioning using 200-cGy total body irradiation and fludarabine. Biol Blood Marrow Transplant. Aug 2006;12(8):887-90. [Medline].

  40. Kröger N, Zabelina T, Renges H, et al. Long-term follow-up of allogeneic stem cell transplantation in patients with severe aplastic anemia after conditioning with cyclophosphamide plus antithymocyte globulin. Ann Hematol. Nov 2002;81(11):627-31. [Medline].

  41. Stein RS, Means RT Jr, Krantz SB, Flexner JM, Greer JP. Treatment of aplastic anemia with an investigational antilymphocyte serum prepared in rabbits. Am J Med Sci. Dec 1994;308(6):338-43. [Medline].

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Keywords

aplastic anemia; anemia; anemia, aplastic; hypoplastic anemia; bone marrow disease; bone marrow failure; bone marrow failure syndrome; severe aplastic anemia; SAA; progressive hypocythemia; aregeneratory anemia; aleukia hemorrhagica; panmyelophthisis; toxic paralytic anemia; peripheral pancytopenia; myelodysplastic syndrome; MDS

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Contributor Information and Disclosures

Author

Sameer Bakhshi, MD, Associate Professor of Pediatric Oncology, Department of Medical Oncology, Dr BRA Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, India
Disclosure: Nothing to disclose.

Coauthor(s)

Esteban Abella, MD, Consulting Staff, Arizona Pediatric Hematology/Oncology, PLLC
Esteban Abella, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and West Virginia State Medical Association
Disclosure: Nothing to disclose.

Medical Editor

David Aboulafia, MD, Medical Director, Bailey-Boushay House; Clinical Professor, Department of Medicine, Division of Hematology, University of Washington
David Aboulafia, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Medical Directors Association, American Society of Hematology, Infectious Diseases Society of America, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Troy H Guthrie, Jr, MD, Director of Cancer Institute, Baptist Medical Center
Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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