Sickle Cell Anemia and Hematological Neoplasias

Abstract

Sickle cell disease (SCD) is a congenital hemolytic anemia with various clinical findings. In some cases hematological neoplasias and some solid tumors may accompany this disease but these have rarely been reported. Here we report five cases with SCD and accompanying hematological neoplasias including lymphoblastic lymphoma, multiple myeloma and hairy cell leukemia in four cases with sickle cell trait and one case of Hodgkin' disease in sickle cell anemia. All of the cases except one had no previously diagnosed congenital hemolytic anemia and/or family history. Peripheral blood findings suggestive for an underlying hemolytic anemia were the first step and the most important initial lead in the detection of SCD. Severe musculoskeletal signs during the first presentation was seen in the lymphoma case, residual renal dysfunction after remission of multiple myeloma, and areas of infarction in the spleen in CT scans in the patient with sickle cell anemia were the most interesting findings in these cases. A standard therapeutic approach without any additional toxicity was relevant in all cases. Detailed clinical presentation and outcome of these five cases are documented here and the literature has been reviewed.

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

“…The study shows that distortion of cells containing Hb is the result of Hb polymerization. Deoxy HbS polymers in the cell exist in a spectrum of forms from scattered individual fibers to highly ordered fibroid aggregate that essentially filled the cell and distorted it into the classic sickle cell or other elongated form [1][2][3][4][5]. Here, we have reported the association of hematological malignancies and SCD in our region.…”

“…This mutation is not oncogenic directly, sickle cell diseases (SCD), are mostly HbSS and HbC or thalassemia. SCD involves multiple system and manifested by variable degree of anemia, acute vasoocclusive episodes, and chronic organ damage [2]. SCD is associated with high degree of proliferation of bone marrow (BM) cells, chronic inflammation, and immune dysregulation.…”

“…These doubly heterozygous statuses are designated by both aberrant gene products such as HbSC disease or Hbs/β thalassemia. The term SCD is used in a generic sense to refer to all of the stickling syndrome [1,2].…”

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.…”

Hodgkin Lymphoma in a Child With Sickle Cell Anemia

“…There are reports of patients with sickle cell disease who developed hematological malignancies such as acute myeloblastic leukemia, multiple myeloma, malignant histiocytosis, Hodgkin lymphoma, and non-Hodgkin lymphoma [1][2][3][4][5][6]. However, the relationship between these malignancies and sickle cell disease is not yet defined and the number of cases reported so far is few.…”