Abstract
IntroductionPost-transplant lymphoproliferative disorder is a serious disorder which occurs post hematopoietic stem cell transplant or solid organ transplantation. T-prolymphocytic leukemia is a T cell type monomorphic post-transplant lymphoproliferative disorder which accounts for only 2% of all mature lymphocytic leukemias in adults over the age of 30.Case presentationA 59-year-old man of Chinese ethnicity presented to our hematology unit with headache, lethargy, and exertional dyspnea for the past 1 month. He underwent an uneventful cadaveric renal transplant 20 years ago for chronic glomerulonephritis-induced end-stage renal disease. He had been on long-term immunosuppressants since then consisting of orally administered prednisolone 10 mg daily and orally administered cyclosporine A 50 mg twice daily. On examination, he was pale with a palpable liver and spleen. He had a functioning renal graft. Marrow flow cytometry confirmed T-prolymphocytic leukemia with lymphocytes expressing CD2, CD3, CD7, CD52, and TCL-1. His human T-cell lymphotropic virus and Epstein–Barr virus serology and deoxyribonucleic acid (DNA) were negative. He was treated with one cycle of cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy to which he failed to respond. In view of his renal allograft, he was not suitable for alemtuzumab due to the risk of nephrotoxicity. He was given orally administered venetoclax but he died on day 17 due to severe auto tumor lysis syndrome.ConclusionThe place of immunophenotyping in the diagnosis and treatment of this disorder is of significant importance. More research needs to be carried out to further comprehend the pathophysiology and treatment modalities for this disorder.
Highlights
Post-transplant lymphoproliferative disorder is a serious disorder which occurs post hematopoietic stem cell transplant or solid organ transplantation
The World Health Organization (WHO) 2016 classification system categorizes Post-transplant lymphoproliferative disorder (PTLD) into six different categories: infectious mononucleosis PTLD, plasmacytic hyperplasia PTLD, florid follicular hyperplasia PTLD, polymorphic PTLD, monomorphic PTLD arising from B and T/natural killer (NK) T cell types, and classical Hodgkin lymphoma PTLD [1]
Prolymphocytic leukemia (PLL) is a rare lymphocytic disorder which accounts for only 2% of all Kasinathan et al Journal of Medical Case Reports (2019) 13:223 mature lymphocytic leukemias in adults over the age of 30 [3]
Summary
Post-transplant lymphoproliferative disorder (PTLD) is a serious disorder which occurs post hematopoietic stem cell transplant (HSCT) or solid organ transplantation. Case presentation A 59-year-old man of Chinese ethnicity presented to our hematology unit with headache, lethargy, and exertional dyspnea for the past 1 month He underwent an uneventful cadaveric renal transplant 20 years ago for chronic glomerulonephritis-induced end-stage renal disease. In view of the bone marrow and flow cytometry findings, he was diagnosed as having T-PLL which is an aggressive form of a lymphoproliferative disorder He was treated with one cycle of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy to which he failed to respond. On day 15 of orally administered venetoclax, he developed severe auto tumor lysis syndrome which required mechanical ventilation and continuous veno-venous hemodialysis His white cell count progressed to 174 × 109/L, predominantly lymphocytosis. He died 2 days later at our intensive care unit
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