期刊论文详细信息
Experimental Hematology & Oncology
Hairy cell leukemia in kidney transplantation: lesson from a rare disorder
Antonio Lupo2  Alberto Zamò3  Gianluigi Zaza2  Paola Tomei2  Fabrizio Vinante1 
[1] Section of Hematology, Department of Medicine, University of Verona, Verona, Italy;Renal Unit, Department of Medicine, University Hospital of Verona, Verona, Italy;Section of Pathological Anatomy, Department of Pathology and Diagnostics, University of Verona, Verona, Italy
关键词: Treatment;    Post-transplant cancer;    Post-transplant lymphoproliferative disease;    Calcineurin inhibitors;    Immunosuppression;    V600E BRAF;    BRAF;    Kidney transplantation;    Hairy cell leukemia;   
Others  :  812088
DOI  :  10.1186/2162-3619-2-22
 received in 2013-06-06, accepted in 2013-08-02,  发布年份 2013
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【 摘 要 】

We report here on the diagnosis and successful treatment of a case of hairy cell leukemia (HCL) that arose 15 years after kidney transplantation in a 51-year-old patient. As soon as the diagnosis was made, HCL was treated with 2-CDA, obtaining complete hematological remission. Immunosuppression with the calcineurin inhibitor cyclosporin was maintained, and the graft was preserved. In kidney transplant recipients supported with immunosuppressive drugs, post-transplant lymphoproliferative diseases (PTLDs) are frequent and typically related to immunosuppression via a loss of control of infectious/EBV-related proliferative stimuli. To date, HCL has not been considered among PTLDs. Recently, however, the oncogenic mutation V600E of the BRAF protein kinase has been found to be a hallmark of HCL, and calcineurin inhibitors have been shown to interfere with signaling downstream of V600E BRAF early on by counteracting senescence-associated mechanisms that protect against the oncogenic potential of the mutated kinase. Such a biochemical link between the oncogene-dependent signaling and calcineurin inhibitor activities suggests that HCL in transplanted patients might be a peculiar type of PTLD based on the presence of a specific mutation. This mechanism might also be involved in other neoplasias bearing the same or similar mutations, such as melanoma and non-melanoma skin cancer.

【 授权许可】

   
2013 Vinante et al.; licensee BioMed Central Ltd.

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