Acute leukemia in sickle cell disease patients in a tertiary health facility in Nigeria: a case series

Abstract

Background and objectives: Sickle cell disease(SCD) is a disorder of red cells resulting from the co-inheritance of hae- moglobin S (HbS) with another abnormal haemoglobin. The diagnosis of acute leukaemia is uncommon in our patients with sickle cell disease more so the patients have high morbidity and mortality due to the sickling process.Acute leukemia is a malignant clonal disorder of haemopoietic precursor cells resulting in accumulation of immature blood cells in the bone marrow and blood.The objective of the case series was to highlight the challenges of diagnosis and management of SCD patients with acute leukaemia, the importance of peripheral blood film review and propound a possible risk factor.
 Methods: Records of 58 patients diagnosed and managed for acute leukaemia over a 7 year period at the University College Hospital, Ibadan were reviewed. The diagnosis of acute leukaemia was based on clinical features in addition to peripheral and bone marrow smears findings. Microsoft excel version 2013 was used for statistical analysis.
 Results: Five (8.6%) of the patients with acute leukaemia also had sickle cell disease: 3 males and 2 females were described. Recurrent fever and anaemia were the most consistent presenting features in the patients. All the patients were not on any routine medications meant for SCD patients and had poor history of clinic attendance prior to the diagnosis of acute leu- kaemia. The diagnosis of acute leukaemia was not made until the patients were seen by a haematologist. The principal tool of diagnosis in all the patients was peripheral blood film review. Two patients were discharged against medical advice.The treatment period ranged between one month and one year in the remaining three patients.
 Conclusion: SCD patients are not exempted from developing acute leukaemias and the diagnoses of the two conditions overwhelms the social and economic support of patients and care givers.The study also underscores the relevance of high level of suspicion and prompt review of peripheral blood film of SCD patients particularly when patients present with un- remitting symptoms associated with anaemia and fever.
 Keywords: Acute leukaemia; sickle cell disease; anaemia.

Philadelphia chromosome-positive B-cell acute lymphoblastic leukemia with e1a3 BCR-ABL1 transcript in a Nigerian with sickle cell anemia: a case report

“…The life expectancy of patients with SCA has improved in recent years owing to advances in management that may be associated with the many cases of malignancies now being reported in these patients. In Nigeria, 8.6% of cases of acute leukemia were associated with sickle cell disease, among which there was only one case of ALL [ 2 ]. Brunson et al .…”

“…In addition, this is the first report from Nigeria in which cytogenetic and immunophenotype analyses were done in the diagnosis of acute leukemia in any patient with SCD. There is limited utilization of cytogenetics and flow cytometry in the diagnostic workup of acute leukemia patients in Nigeria owing to lack of facilities in most centers [ 2 , 3 ].…”

“…The abnormal HbS polymerizes when deoxygenated and induces sickling of red blood cells (RBCs). The clinical features include chronic hemolysis, anemia, and periodic episodes of acute pain termed vaso-occlusive crisis (VOC) [ 2 ]. The occurrence of acute leukemia in individuals with SCA is rare [ 2 , 3 ].…”

“…The clinical features include chronic hemolysis, anemia, and periodic episodes of acute pain termed vaso-occlusive crisis (VOC) [ 2 ]. The occurrence of acute leukemia in individuals with SCA is rare [ 2 , 3 ]. This has been attributed to the reduced life expectancy associated with SCA.…”

Twenty years of African Health Sciences as infections continue to plague our continent

“…Nigerian authors 35 report a case series on “Acute leukaemia in sickle cell disease patients in a tertiary health facility.” We end this section with a paper on: “Telepathological evaluation of paediatric histological specimens in support of a hospital in Tanzania” 36 . Quite appropriate because resources and capacity are limited but not innovation!…”