Aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and bone marrow failure. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, outcomes have improved considerably, with 5-year survival reported to be 70% - 90%. In Congo, contemporary data on survival are lacking. We performed a retrospective study to describe the epidemiological, diagnostic, and therapeutic characteristics of patients with AA diagnosed in the clinical hematology department of the University Hospital of Brazzaville from 2017 to 2023. Chemically induced aplasia was excluded from the study. The CAMITTA criteria were used to classify the severity of AA. In total, 45 confirmed cases were identified, and 35 files provided sufficient data for the descriptive study. The median age was 26 years (range: 1 - 50). Adults made up 75% of the study population. The sex ratio was 1.05 (0.5 in children and 1.17 in adults). One case of AA was secondary to treatment with imatinib mesylate; the other cases (97.1%) were idiopathic. Pancytopenia was present in all patients. Moderate, severe, and very severe AA represented 11.4%, 74.3%, and 14.3% of cases, respectively. Severe and very severe forms were more frequent in adults (77.4% vs. 22.6%) and in men. Nine patients (26%) received cyclosporine monotherapy. Only one received treatment regularly and obtained the only favorable response. No patient received ATG or eltrombopag. Hemorrhagic syndrome was the most common cause of death (4 out of 6 cases) due to the unavailability of platelet concentrates. Eighteen patients (51.4% of cases) were lost to follow-up. The median follow-up was 28.7 (1 - 96) months. In conclusion, the prognosis of AA remains poor and could be improved with affordable immunosuppressive treatments, availability of platelet concentrates, and implementation of allogeneic bone marrow transplantation.
References
[1]
Scheinberg, P. (2022) A New Standard Immunosuppression Regimen in Severe Aplastic Anemia. New England Journal of Medicine, 386, 89-90. https://doi.org/10.1056/nejme2118143
[2]
Scheinberg, P. (2021) Acquired Severe Aplastic Anaemia: How Medical Therapy Evolved in the 20th and 21st Centuries. British Journal of Haematology, 194, 954-969. https://doi.org/10.1111/bjh.17403
[3]
Georges, G.E. and Storb, R. (2016) Hematopoietic Stem Cell Transplantation for Acquired Aplastic Anemia. Current Opinion in Hematology, 23, 495-500. https://doi.org/10.1097/moh.0000000000000281
[4]
Scheinberg, P. (2018) Recent Advances and Long-Term Results of Medical Treatment of Acquired Aplastic Anemia: Are Patients Cured. Hematology/Oncology Clinics of North America, 32, 609-618. https://doi.org/10.1016/j.hoc.2018.03.003
[5]
Scheinberg, P. (2018) Activity of Eltrombopag in Severe Aplastic Anemia. Hematology, 2018, 450-456. https://doi.org/10.1182/asheducation-2018.1.450
[6]
Protocole national de diagnostic et de soins (2023) Aplasies médullaires acquises et constitutionnelles. https://www.has-sante.fr/upload/docs/application/pdf/2023-04/pnds_aplasies-medullaires-acquises_et_constitutionnelles_20130301.pdf
[7]
Arewa, O.P. and Akinola, N.O. (2009) Survival in Primary a Plastic Anaemia; Experience with 20 Cases from a Tertiary Hospital in Nigeria. African Health Sciences, 9, 290-293.
[8]
Aken’ova, Y.A. and Okunade, M.A. (1992) Aplastic Anaemia: A Review of Cases at the University College Hospital, Ibadan, Nigeria. Central African Journal of Medicine, 38, 362-367.
[9]
Riyat, M.S., Kasili, E.G. and Mwanda, W.O. (1990) Childhood Aplastic Anemia in Kenya. East African Medical Journal, 67, 264-272.
[10]
Tolo-Diebkilé, A., Koffi, K.G., Nanho, D.C., Sékongo, Y.M., Kouakou, B., Méité, N., Ayémou, R. and Sanogo, I. (2009) Les aplasies médullaires: Profils épidémiologique, clinique, étiologique et évolutif. A propos de 34 cas colligés au CHU de Yopougon. Mali Médical, 24, 7-11.
[11]
Yao, T., Abissey, A., Danho, B., Moumouni, K. and Téa, D. (1990) Etude des aplasies médullaires: À propos de 12 cas diagnostiqués au CHU de Treichville. Publications Médicales Africaines, 110.
[12]
Galiba Atipo Tsiba, F.O., Kocko, I., Kambourou, J., Nziengui Mboumba, J.V., Ocko, T., Malanda, F., Atanda, H.L. and Elira Dockekias, A. (2016) Aplasies médullaires de l’enfant au CHU de Brazzaville. Medecined’Afrique Noire, 63, 607-612.
[13]
World Health Organization (2023) Le Congo a presque doublé ses dons en trois ans. https://www.afro.who.int/fr/photo-story/le-congo-presque-double-les-dons-de-sang-en-trois-ans
[14]
Camitta, B., Thomas, E., Nathan, D., Santos, G., Gordon-Smith, E., Gale, R., et al. (1976) Severe Aplastic Anemia: A Prospective Study of the Effect of Early Marrow Transplantation on Acute Mortality. Blood, 48, 63-70. https://doi.org/10.1182/blood.v48.1.63.63
[15]
Ally, M., Magesa, P. and Luzzatto, L. (2019) High Frequency of Acquired Aplastic Anemia in Tanzania. American Journal of Hematology, 94, E86-E88. https://doi.org/10.1002/ajh.25388
[16]
Norasetthada, L., Wongkhantee, S., Chaipokam, J., Charoenprasert, K., Chuncharunee, S., Rojnuckarin, P., et al. (2021) Adult Aplastic Anemia in Thailand: Incidence and Treatment Outcome from a Prospective Nationwide Population-Based Study. Annals of Hematology, 100, 2443-2452. https://doi.org/10.1007/s00277-021-04566-0
[17]
Li, S., Hsu, Y., Chang, C., Lee, S., Yen, C., Cheng, C., et al. (2018) Incidence and Treatment Outcome of Aplastic Anemia in Taiwan Region—Real-World Data from Single-Institute Experience and a Nationwide Population-Based Database. Annals of Hematology, 98, 29-39. https://doi.org/10.1007/s00277-018-3486-3
[18]
Peffault de Latour, R., Kulasekararaj, A., Iacobelli, S., Terwel, S.R., Cook, R., Griffin, M., et al. (2022) Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation. Eltrombopag Added to Immunosuppression in Severe Aplastic Anemia. The New England Journal of Medicine, 386, 11-23.
[19]
Mandal, P.K., Baul, S., Dolai, T.K., De, R. and Chakrabarti, P. (2016) Outcome of Cyclosporine Monotherapy in Patients of Aplastic Anemia: Experience of a Tertiary Care Hospital in Eastern India. Indian Journal of Hematology and Blood Transfusion, 33, 144-147. https://doi.org/10.1007/s12288-016-0706-7
[20]
Dolai, T.K., Jain, M. and Mahapatra, M. (2022) Idiopathic Aplastic Anemia: Indian Perspective. Indian Journal of Hematology and Blood Transfusion, 39, 357-370. https://doi.org/10.1007/s12288-022-01592-4
[21]
Marsh, J.C.W., Zomas, A., Hows, J.M., Chapple, M. and Gordon‐Smith, E.C. (1993) Avascular Necrosis after Treatment of Aplastic Anaemia with Antilymphocyte Globulin and High‐Dose Methylprednisolone. British Journal of Haematology, 84, 731-735. https://doi.org/10.1111/j.1365-2141.1993.tb03153.x
[22]
Vaht, K., Göransson, M., Carlson, K., Isaksson, C., Lenhoff, S., Sandstedt, A., et al. (2017) Incidence and Outcome of Acquired Aplastic Anemia: Real-World Data from Patients Diagnosed in Sweden from 2000-2011. Haematologica, 102, 1683-1690. https://doi.org/10.3324/haematol.2017.169862
[23]
Giammarco, S., Peffault de Latour, R., Sica, S., Dufour, C., Socie, G., Passweg, J., et al. (2018) Transplant Outcome for Patients with Acquired Aplastic Anemia over the Age of 40: Has the Outcome Improved? Blood, 131, 1989-1992. https://doi.org/10.1182/blood-2017-09-807859
[24]
Sachdev, M., Bansal, M., Chakraborty, S., Jain, S., Bhargava, R. and Dua, V. (2020) Allogeneic Hematopoietic Stem Cell Transplant Offer Good Outcomes in Pediatric Aplastic Anemia: Experience from Developing World. Journal of Pediatric Hematology/Oncology, 43, e677-e681. https://doi.org/10.1097/mph.0000000000001981