Abstract
Introduction: Hypoplastic myelodysplastic syndrome (hMDS) is morphologically difficult to be distinguished from acquired aplastic anaemia (AA). Response to treatment of AA varies based on the treatment used and duration of follow up. we conducted a prospective study to estimate bone marrow CD 34 cells on hypoplastic marrows (AA and hMDS) and evaluated the haematologic response to treatment in aplastic anaemia.
Methods: We conducted a prospective study in patients with hypocellular BM due to either acquired AA or hMDS. The CD34+cells in the bone marrow were estmated by immunohistochemistry (IHC) prior to treatment. We assessed treatment response to immunosuppressive therapy in patients with AA.
Results: We followed up 53 patients with AA for a minimum of 3 months. Out of 53 patients with AA, 34 (64%) had low CD34+ level, 17 (32%) had normal and 2 (4%) had high CD34+ levels. 34 (64%) patients were on cyclosporine, 14 (26%) patients were on androgens, 3(5.6%) patients received ATG regime and 2(4.4%) patients received blood and blood products alone. At the end of 3 months, 20 (39%) patients showed partial response while the remaining 31 (61%) patients were nonresponders, irrespective of the treatment category.
Conclusion: In conclusion, majority of the patients with AA had low CD34 cells at baseline in our study. The response to treatment varies widely based on the type of treatment. There was no difference in treatment response, irrespective of treatment category based on the CD34 cells at baseline among patients with low and normal CD 34 cells.
Keywords: Aplastic anemia, hypocellular myelodysplastic syndrome (hMDS), CD 34+, immunosuppressive therapy, treatment response.
References
- Longo DL, et al. Harrison’s Principles of Internal Medicine.18th edition.McGraw-Hill Companies.2012.
- Orazi A, Albitar M, Heerema NA, Haskins S, Neiman RS. Hypoplastic myelodysplastic syndromes can be distinguished from acquired aplastic anemia by CD34 and PCNA immunost-aining of bone marrow biopsy specimens. Am J Clin Pathol 1997;107:268-74.
- Orazi A, Czader MB. Myelodysplastic syndromes. Am J Clin Pathol 2009;132: 290-305.
- Greaves MF, Brown J. Molecular features of CD34: a haematopoietic progenitor cell associated molecule.Leukemia,6:31-36,1992.
- Appelbaum FR, Barrall J, Storb R, Ramberg R, Doney K, Sale GE et al. Clonal cytogenetic abnormalities in patients with otherwise typical aplastic anemia. Exp Hematol 1987; 15: 1134–1139.
- Mikhailova N, Sessarego M, Fugazza G, Caimo A, De Filippi S,Van Lint MT et al. Cytogenetic abnormalities in patients with severe aplastic anemia. Haematologica 1996; 81: 418–422.
- Socie G, Henry-Amar M, Bacigalupo A, Hows JM, Tichelli A, Ljungman P et al. Malignant tumors occuring after treatment of aplastic anemia. N Engl J Med 1993; 329: 1152–1157.
- Bacigalupo A, Bruno B, Saracco P, Di Bona E, Locasciulli A, Locatelli F et al. Antilymphocyte globulin, cyclosporine, prednisolone, and granulocyte colony-stimulating factor for severe aplastic anemia: an update of the GITMO/EBMT study on 100 patients. European Group for Blood and Marrow Transplantation (EBMT) Working Party on Severe Aplastic Anemia and the Gruppo Italiano Trapianti di Midolio Osseo (GITMO). Blood 2000; 95:1931–1934.
- Baur AS, Meugé-Moraw C, Schmidt PM, Parlier V, Jotterand M, Delacrétaz F. CD34/QBEND10 immunostaining in bone marrow biopsies: an additional parameter for the diagnosis and classification of myelodysplastic syndromes. Eur J Haematol 2000;64:71-9.
- Matsui WH, Brodsky RA, Smith BD, Borowitz MJ, Jones RJ. Quantitative analysis of bone marrow CD34 cells in aplastic anemia and hypoplastic myelodysplastic syndromes. Leukemia Mar 20(3):458-462,2006.
- Cha CH, Park CJ, Chi HS, Seo EJ, Jang S, Cho YU et.al. CD34 and p53 Immunohistochemical Stains Differentiate Hypocellular Myelodysplastic Syndrome (hMDS) from Aplastic Anemia and a CD34 Immunohistochemical Stain Provides Useful Survival Information for hMDs. Annals of Laboratory Medicine.2014;34:426-432.
- Orazi A, Albitar M, Heerema NA, Haskins S, Neiman RS. Hypoplastic myelodys-plastic syndromes can be distinguished from acquired aplastic anemia by CD34 and PCNA immunostaining of bone marrow biopsy specimens. Am J ClinPathol1997;107:268-74.
- The International Agranulocytosis and Aplastic Anemia. Incidence of aplastic anemia: The relevant diagnostic criteria. Blood. 1987; 70: p. 1718.
- Foucar K, Langdon RM II, Armitage JO, et al. Myelodysplastic syndromes: a clinical and pathologic analysis of 109 cases. Cancer. 1985;56:553-561.
- Camitta B.M., Rappeport, J.M., Parkman, R. & Nathan, D.G. Selection of patients for bonemarrow transplantation in severe aplastic anemia. Blood, 1975, 45,355-363.
- Camitta B.M. What is the defination of cure for aplastic anemia? Acta hematologica, 2000, 103, 16-18.
- Marsh JC, Chang J, Testa NG, Hows JM, Dexter TM. In vitro assessment of marrow ‘stem cell’ and stromal cell function in aplastic anaemia. Br J Haematol 1991; 78: 258–267.
- Manz CY, Nissen C, Wodnar-Filipowicz A. Deficiency of CD34+ c-kit+ and CD34+38 hematopoietic precursors in aplastic anemia after immunosuppressive treatment. Am J Hematol 1996; 52: 264–274.
- Amy E DeZernand Robert A Brodsky, Clinical management of aplastic anemia, Expert Rev Hematol. 2011 April ; 4(2): 221–230. doi:10.1586/ehm.11.
- José Carlos Jaime-Pérez,Perla R. Colu-nga-Pedraza, Cynthia D. Gómez-Ramírez. et al Danazol as first-line therapy for aplastic anemia Annals of Hematology May 2011, Volume 90, Issue 5, pp 523–527.
- Locasciulli A, Oneto R, Bacigalupo A, et al. Outcome of patients with acquired aplastic anemia given first line bone marrow transplantation or immunosuppressive treatment in the last decade: a report from the European Group for Blood and Marrow Transplantation (EBMT). Haematologica.2007; 92(1):11–18. [PubMed: 17229630.
- Marsh J, Schrezenmeier H, Marin P, et al. Prospective randomized multicenter study comparing cyclosporin alone versus the combination of anti-thymocyte 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. 1999; 93(7):2191–2195.[PubMed: 10090926]
Corresponding Author
Shruthi Kulkarni
Assistant Professor, Dept of Medicine,
St. John’s Medical College Hospital, Sarjapur road, Bangalore-34
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