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Successful hematopoietic cell transplantation in a child with sickle cell disease and systemic lupus erythematosus

Bone Marrow Transplantation volume 48, pages 151–152 (2013)Cite this article

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Allogeneic hematopoietic SCT (HSCT) remains the only curative but highly successful treatment for sickle cell disease.1 HSCT may be an acceptable treatment for patients with severe systemic lupus erythematosus (SLE).2 We report our experience of matched sibling HSCT in an 11-year-old girl with sickle cell disease complicated by SLE. The post transplant course was complicated by posterior leukoencephalopathy syndrome with status epilepticus secondary to cyclosporine toxicity, gastroduodenitis probably due to mycophenolate mofetil treatment, and mild acute and chronic graft-versus-host disease (GVHD). To the best of our knowledge, this is the first successful HSCT of concurrent sickle cell disease and SLE.

A Bahraini girl with sickle cell-β thalassemia had mild anemia (baseline Hb 8–9 g/dl) and infrequent crisis until the age of 8 years when she presented with recurrent arthralgia, malar rashes, cutaneous vasculitis, hepatosplenomegaly, mild proteinuria and Coombs-positive hemolysis. The diagnosis of SLE was made with the positive findings of anti-dsDNA antibodies. Since then, her clinical course had been complicated by monthly to bimonthly attacks of overlapping sickle cell crises and flaring up of SLE with fever, rashes, bone pains, arthralgias. She was eventually put on regular red cell transfusion or exchange transfusion, intermittent pulses of i.v. methylprednisolone as well as prolonged oral prednisolone therapy, ibuprofen and inhaled bronchodilation for obstructive airway disease. During the first year of her illness with SLE, she had one episode of generalized convulsion associated with posterior leukoencephalothy on magnetic resonance imaging (MRI) but no vascular changes on the magnetic resonance angiography. The abnormalities disappeared a year later on repeat imaging. Treatment with deferasirox was commenced for transfusion hemosiderosis. In view of the frequent veno-occlusive crises and regular blood transfusions associated with sickle cell disease, she was considered a candidate for allogeneic HSCT.

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Authors and Affiliations

  1. Children's Haematology & Cancer Centre, Mount Elizabeth Hospital, Singapore

    A C-W Lee

  2. Department of Pediatric Hematology & Oncology, Salmaniya Medical Complex, Kingdom of Bahrain

    K K Al-Saad

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  1. A C-W Lee
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  2. K K Al-Saad
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Correspondence to A C-W Lee.

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Lee, AW., Al-Saad, K. Successful hematopoietic cell transplantation in a child with sickle cell disease and systemic lupus erythematosus. Bone Marrow Transplant 48, 151–152 (2013). https://doi.org/10.1038/bmt.2012.106

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