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Abstract
Extramedullary relapse of acute lymphoblastic leukemia (ALL) is rare and has been primarily reported in pediatric patients or hematopoietic stem cell transplant recipients. We report a case of a 62-year-old woman who presented with relapsed ALL involving her kidneys, pancreas, and bone marrow 2 years after completing chemotherapy with a standard ALL protocol. Unfortunately, her extramedullary disease progressed despite treatment. To the best of our knowledge, this is the first reported case of extramedullary relapse of B-cell ALL to the kidneys and pancreas occurring in an adult patient who had not previously undergone a hematopoietic stem cell transplant. A literature review of kidney and pancreatic extramedullary relapse in ALL is also included.
Highlights
Acute lymphoblastic leukemia carries a poor prognosis in adults
The majority of the literature is in pediatric patients or in the postallogeneic hematopoietic stem cell transplantation setting
The most frequent sites of isolated extramedullary relapse tend to be in the “sanctuary sites” of the central nervous system (CNS) and testes, likely due to selective penetrance and reduced exposure to chemotherapy in these areas [3]
Summary
Acute lymphoblastic leukemia carries a poor prognosis in adults. While the complete remission rate in a patient with a standard risk profile may exceed 90% with initial treatment, the 5-year overall survival is only 30–40% [1]. Data on extramedullary relapses of ALL is limited in the adult population. The majority of the literature is in pediatric patients or in the postallogeneic hematopoietic stem cell transplantation setting. In a review of 9585 pediatric patients with ALL, 42.8% of relapses were found to be extramedullary, either with (13.5%) or without (29.3%) concomitant bone marrow involvement [2]. Extramedullary relapse has been reported in the ovary, breast, eye, bone, and kidneys [4,5,6,7,8]. Our case is the first to describe renal and pancreatic involvement of relapsed B-cell ALL in an adult patient who did not undergo a previous hematopoietic stem cell transplant
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