Aplastic Anaemia: Experience with Management in a Resource Limited Setting

Abstract

Background: Aplastic anaemia is syndrome of bone marrow failure characterized by peripheral pancytopenia and loss of haemopoietic stem cells in the bone marrow. It is a relatively rare disease with high risk of death if untreated. Objective: To evaluate the incidence, management and outcome of aplastic anaemia in children, over a four-year period, at the University of Port Harcourt Teaching Hospital(UPTH), Nigeria. Subjects and Methods: This was a retrospective study of case notes of all children with anaemia and bone marrow aspiration reports suggestive of aplastic anaemia in the Haematology and Oncology Units of the Department of Paediatrics, UPTH, from 1st January 2015 to 31stDecember 2018.Data retrieved included bio data, clinical and laboratory profile, and outcome. Data entry and analysis were done using the Statistical Package for Social Sciences (SPSS) version 22. Data analysis were done using descriptive statistics (proportions and frequencies) and presented in prose and frequency tables. Mean and standard deviations of quantitative data were obtained. Results: A total of six children were treated for a plastic anaemia during the period under review, giving an annual incidence of 0.0049 cases per year. Their ages ranged from 3 to 11 years (mean 8.2±3.7), with a male to female ratio of 2:1.At presentation, they had a mean (±SD) packed cell volume of 15± 3.2%, white cell count of 2.72± 0.76x 109/L, Absolute neutrophil count (ANC) of 0.25± 0.17 x 109/L, and platelets count of 17.33± 21.36 x 109/L. They all (100%) had severe aplastic anaemia at diagnosis. Among others, they received transfusion support with unbanked fresh whole blood, platelet concentrates, antibiotics, antifungal and antiviral, oral cyclosporine and intravenous methyl-prednisolone. They were all blood transfusion dependent. The mean survival from diagnosis was 7.3± 3.08 months. None of the patients benefited from bone marrow transplantation and anti-thymocyte immunoglobulin. Mortality rate was 83.3% and major cause of death was sepsis with thrombocytopaenic bleeding. Conclusion: Aplastic anaemia is rare with short survival rate. There is need for the Federal Government to ensure that bone marrow/stem cell transplantation centres are available and accessible across the country, and to make drugs such as cyclosporine and anti-thymocyte immunoglobulin affordable to ameliorate the condition.