Multiple isolated extramedullary relapse of acute promyelocytic leukemia after allogeneic hematopoietic stem cell transplant: a case report and review of literature

Shalin Kothari,Roger Herzig,Geoffrey Herzig,Stephen Slone

doi:10.1186/2193-1801-2-49

Shalin Kothari, Roger Herzig + Show 2 more

Open Access

https://doi.org/10.1186/2193-1801-2-49

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Abstract

Isolated extramedullary disease (EMD) is uncommon, especially in acute promyelocytic leukemia (APL) after allogeneic hematopoietic stem cell transplantation (HSCT). We review the literature and present a 32 year old woman with APL who developed multiple EMDs after allogeneic HSCT within the calvarium, and later found to have various isolated lesions including femur, humerus and thoraco lumbar vertebrae. She was treated with local radiotherapy (XRT) to EMD lesions, all-trans retinoic acid, arsenic trioxide and donor lymphocyte infusion at different time points in her clinical course, without success. Out of reported cases in clinical setting as ours, average onset of isolated EMD is 25 months and median survival 14 months. Effective treatment of isolated EMD after HSCT is not yet clear, but ATO in combination with local XRT, tamibarotene and second HSCT have shown good results in some reported cases, but accumulation of more cases is needed to elucidate optimal therapy in such setting.Electronic supplementary materialThe online version of this article (doi:10.1186/2193-1801-2-49) contains supplementary material, which is available to authorized users.

Highlights

Among the more treatable subtypes of acute myeloid leukemia (AML) is acute promyelocytic leukemia (APL)
We present a case of multiple skull lesions of myeloid sarcoma in a patient with APL that occurred seven months
She maintained complete remission (CR) status for 18 months with 2 courses of consolidation therapy consisting of idarubicin and cytarabine. She relapsed in BM and CNS while being on all-trans retinoic acid (ATRA) as maintenance therapy. She had a successful re-induction with arsenic trioxide (ATO) and intrathecal cytarabine followed by high-dose busulfan and etoposide with autologous promyelocytic leukemia (PML)/RARα PCR-negative hematopoietic cells rescue

Summary

Introduction

Among the more treatable subtypes of acute myeloid leukemia (AML) is acute promyelocytic leukemia (APL). She relapsed in BM and CNS while being on ATRA as maintenance therapy She had a successful re-induction with arsenic trioxide (ATO) and intrathecal cytarabine followed by high-dose busulfan and etoposide with autologous PML/RARα PCR-negative hematopoietic cells rescue. She relapsed in 7 months and was referred to our institution for an allogeneic HSCT from her HLA matched sister after cyclophosphamide and total body irradiation (TBI) conditioning regimen. After radiation therapy (a total of 20 Gy in 10 fractions), she received two months of ATO, but had progressive disease evident on whole body PET/CT imaging She was treated with high-dose cytarabine and idarubicin with infusion of stored donor hematopoietic cells (DLI), but died of complications 16 days after starting therapy.

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