Multiple myeloma in sickle cell syndromes

Area of Science:

• Hematology
• Oncology

Background:

• Multiple myeloma (MM) is an uncommon malignancy in patients with sickle cell syndromes (SCS).
• Understanding the co-occurrence of these conditions is crucial for patient management and research.

Observation:

• Six Greek patients with SCS (five HbSbeta+thalassaemia, one sickle cell anaemia) aged 56-65 developed MM.
• The cases included various MM subtypes (IgGkappa, IgGlambda, IgAkappa, biclonal IgGK-IgAK).
• All patients had cholelithiasis, and two progressed from monoclonal gammopathy of undetermined significance (MGUS) to MM.

Findings:

• This study presents the first reported cases of MM associated with HbSbeta+thalassaemia.
• Clinical manifestations included bleeding diathesis, stroke, seizures, and bone marrow necrosis, exacerbated by sickle cell vaso-occlusion.
• Treatment involved plasmapheresis and exchange blood transfusions for hyperviscosity complications.

Implications:

• Increased survival in SCS patients may lead to a higher incidence of MM if a pathogenic link exists.
• Cholelithiasis may be a predisposing factor for MM in SCS patients.
• Further research is necessary to elucidate the relationship between SCS and MM.