Hematologic malignancy in sickle cell disease: Report of four cases and review of the literature

Abstract

Hematologic malignancy has rarely been reported in adults with sickle cell disease. We describe four sickle cell patients (two with hemoglobin SC, two with hemoglobin SS) who developed hematologic malignancy (acute myeloblastic leukemia, multiple myeloma, malignant histiocytosis, and Hodgkin's disease). Three of the cases represent the first adult association between SC or SS hemoglobinopathy and the particular malignancy involved. Sickle hemoglobin does not appear to exert a protective effect against childhood hematologic malignancies, suggesting that better survival in sickle cell disease may be accompanied by an increased incidence of hematologic neoplasms in adulthood. Karyotypic analysis revealed alterations of chromosome 5 in two sickle cell patients with leukemia, raising the possibility of a chromosomal link between the two diseases. Further epidemiologic and cytogenetic studies are needed to define the relationship between hematologic malignancy and sickle cell disease.

Incidence of Hematological Malignancies in Sickle Cell Patients From an Indian Tertiary Care Teaching Hospital

“…In 1986, Stricker et al [13] reported four adult cases of cancer in SCD and summarized 13 previously reported cases of leukemia and lymphoma. They concluded that SCD likely not only provided no increased risk of cancer developing but also conferred no protection.…”

“…The development of malignancy in persons with SCD has been documented by numerous individual cases [8][9][10][11][12][13][14][15] and several small series [13][14][15] over the past 50 years. These scattered reports document that malignancy occurs in both children and adults with SCD but do not provide complete data on the types of cancer or attempt to define the incidence of cancer in this patient population [5,6].…”

“…IASCNAPA sent a survey to its members requesting information about cases of malignancy in patients with SCD followed at their institutions. The results of this survey are summarized in this report [13,14].…”

Cytotoxicity and DNA damage in the neutrophils of patients with sickle cell anaemia treated with hydroxyurea

“…Some studies have shown that HU is genotoxic (Flanagan et al, 2010;Juul et al, 2010;Santos et al, 2011) while other studies suggest that HU has low mutagenicity in vivo (Stricker et al, 1986;Dawkins et al, 1997;Montalembert et al, 1999;Hanft et al, 2000;Montalembert, Davies, 2001;Moschovi et al, 2001). In this study, patients with SCA treated with HU had significantly elevated DI values when compared with the patients with SCA who were not treated with HU.…”

Successful mobilization and transplantation of filgrastim mobilized hematopoietic stem cells in sickle cell-hemoglobin C disease

“…[2][3][4] Many of these malignancies may benefit from autologous hematopoietic stem cell transplantation. However, filgrastim administered to mobilize hematopoietic stem cells may precipitate severe and sometimes fatal sickle crisis in patients with sickle-cell anemia or complex sickle-cell hemoglobinopathies.…”