Abstract
Background: Multiple myeloma (MM) rarely coexists with Sickle Cell Anemia (Hb-SS-Disease). Whereas MM usually affects those older than 50, Sickle Cell Anemia is a congenital disease with a median life expectancy of 42-47 years. In one recent survey by Zerdan et al (Am J Case reports 2021), there have been 12 cases of coexisting MM with Sickle Cell Disease (including 4 cases of compound sickle cell syndrome). Pareseval et al (J Clin Investigation 2008) has previously shown that the thalidomide analogues Lenalidomide and particularly Pomalidomide are effective inducers of Hgb F in ex-vivo erythroid cells from both healthy donors and patients with SCD. Kutlar et al (Blood 2013) reported on a phase I dose-escalation trial of Pomalidomide given for 12 weeks in patients with SCD, which demonstrated an increase in Hgb F. However, there has not been further progress in exploring this modality of treatment since that time. Here, we report a rare case of a patient with Sickle Cell Anemia who was diagnosed with MM at the age of 38, and is benefiting from Lenalidomide for the treatment of both conditions.
Case: 38 y/o M with a history of Sickle Cell Anemia complicated by pulmonary embolism and recurrent deep vein thrombosis, priapism and thrombotic strokes at the ages of 6 and 18 years and follows at hematology clinic for SCD management. Patient was maintained on Hydroxyurea and partial exchange transfusions every 2 weeks with a goal sickle cell hemoglobin (HbS) of 30%. He developed another stroke at the age of 35, and was found to have Moyamoya disease and intracranial bleed which required craniotomy and evacuation. Later, he was incidentally detected to have smoldering multiple myeloma confirmed by bone marrow biopsy which then clinically progressed to MM. He was started on treatment for MM, received a total of 11 cycles of Lenalidomide + Bortezomib + Dexamethasone (RVD regimen) and later transitioned to Lenalidomide alone as maintenance. Around this time, patient was switched to full exchange transfusion, with close monitoring of Hgb S to determine the timing of each exchange. In this context, it was noted that the Hgb F level was steadily rising and peaked at 21% in January 2020, with Hgb S of 41% and transfusion-induced Hgb A of 35%. Once the COVID-19 pandemic set it, the patient stayed at home as a precaution against infection, but the Lenalidomide maintenance was continued, along with Hydroxyurea. He was followed remotely with televisits and home blood draws for the following 2 years. His Hgb F stayed between 25-30% and he had no sickle cell crises and did not require any blood transfusions. He has not had significant side effects from Lenalidomide and the MM has remained controlled, as well.
Conclusion: It is interesting to note that our patient with severe Sickle Cell Anemia, with a history of multiple strokes, has had sustained control of his disease for 2 years while on Hydrea and maintenance Lenalidomide (used primarily for prevention of MM relapse), without the need for exchange transfusions. This outcome supports the pre-clinical findings of Parseval et al, and may provide additional basis for advancing clinical research of thalidomide analogues in the management of Sickle Cell Anemia.
Disclosures
No relevant conflicts of interest to declare.
Author notes
∗Asterisk with author names denotes non-ASH members.
