Background: Hemopathies were rarely observed in major sickle
cell disease patients some thirty years ago, probably due to the high mortality
rate among the latter as a result of progressive complications. Thanks to
advances in the management of sickle cell disease, patients' life expectancy
has increased considerably, exposing them more frequently to neoplasia,
including hematological malignancies. The increased risk of leukemogenesis is
multifactorial and linked to the pathophysiological mechanisms of the clinical
manifestations of sickle cell disease. Study Setting: The clinical
haematology department of campus teaching hospital and the paediatric onco-haematology unit of Sylvanus Olympio teaching hospital in Lomé were used
as study settings. Observations: Four hematologic malignancies were
collected in a cohort of 5847 major sickle cell syndromes. The median age of
the patients was 31.25 years (extremes: 14
and 58 years) and they were predominantly female (sex ratio M/F = 0.25). Two
were on background therapy with hydroxyurea. Among the four patients,
there were two cases of acute lymphocytic
leukemia, including ALL3 in a 58-year-old SS woman and T-ALL2 in a 12-year-old
SC. Then, a case of lymphocytic lymphoma in a 20-year-old SS man was reported
and finally a case of chronic myelocytic leukemia in a 33-year-old woman of Sβ+thalassaemia
phenotype. Conclusion: To further report this coexistence, it is
therefore essential to systematically consider hematological malignancies
during major sickle cell syndromes even if there are similarities in the
symptomatology of these two serious pathological situations.
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