Clinicohematological Characteristics and Survival Analysis of Aplastic Anemia in Pakistan; A Single Centre Experience
Authors:   Department of Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan., Anwar Nida , Arshad Aisha ,   Department of Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan., Fatima Naveena ,   Department of Research and Development, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan., Shaheen Sumaira ,   Department of Research and Development, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan., Shamsi Tahir Sultan ,   Department of Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan.
Published: July 2021

Abstract: Objective: Aplastic anemia (AA) is characterized by pancytopenia and hypocellular bone marrow. Several factors like infections, toxins, chemotherapeutics and radiations are known causes for the suppression of hematopoietic cells which results in bone marrow failure but the exact etiology is unknown. The current study was conducted to assess the baseline demographics, laboratory and clinical characteristics of patients presenting with aplastic anemia and evaluate their cytogenetic profile. Materials and Methods: A retrospective cohort study conducted at National Institute of Blood Diseases and Bone Marrow Transplantation after approval by Institutional Ethics Committee. In this study, AA patients were enrolled from January 2010 to December 2018. Data collection included demographic, laboratory and clinical characteristics including age, gender, symptoms, treatment, and blood counts. Cytogenetic analysis was performed on bone marrow samples. Data analysis was done by using SPSS version 23. Results: Based on camitta classification, a total of 362 aplastic anemia patients were enrolled in the study. The frequency of severe aplastic anemia was most common 199(55%). Median and interquartile range (IQR) age of overall patients was 17(11-26) years, for children and adult population it was 12(9-14) years and 28 (21-43) years respectively. Male predominance was observed i.e.251(69%). The most common presenting complaint was fever 202(55.8%). The median and IQR of hemoglobin (Hb) was 7.8(5.8-9.4)g/dl, MCV 90(83-91)fl, total leucocyte count (TLC) 2.6 (1.9-3.6) × 109/l, absolute neutrophil count (ANC) 0.64 (0.27-1.2)×109/l and platelet count 13 (5-27) ×109/l. Bone marrow cytogenetics was done and 76 (67%) patients were found to have normal karyotype. CMV was positive in 24(6.6%). Majority of patients were treated with blood transfusion and supportive care only 230(64%) and the survival rate was 84%. Conclusion: In conclusion, the study represents a large cohort of aplastic anemia in the country. Majority of cases were acquired aplastic anemia predominantly being severe aplastic. Limited numbers of patients opted for standard treatment options probably due to financial reservations to afford standard treatments like immune suppression therapy and hematopoietic stem cell transplant. Further, local studies with larger number of sample size and provision of standard treatment options are needed to explore the treatment response, etiological factors, prognosis and outcomes. Keywords: Aplastic anemia, Cytogenetics, Clinicohematological characteristics, Laboratory analysis, Survival outcome, Pakistan.