Monomorphic Post-transplant Lymphoproliferative Disorder After Kidney Transplantation and Hematopoietic Stem Cell Transplantation: Clinicopathological Characteristics, Treatments and Prognostic Factors

Abstract

Post-transplant lymphoproliferative disorders (PTLDs) are a heterogeneous group of lymphoid neoplasms associated with immunosuppression following transplantation. Among PTLDs, monomorphic PTLD (m-PTLD) is the largest category; however, its characteristics and survival outcome are not fully understood because of low incidence. This study enrolled 30 adult patients with m-PTLD after kidney-transplantation (KT, n = 17) and hematopoietic stem cell transplantation (HSCT, n = 13) from January 1998 to December 2014. The incidence rates of m-PTLD were 0.74 and 3.63% in the KT and HSCT groups, respectively. M-PTLD patients in the HSCT group were younger and showed earlier onset, with EBV-encoded small RNAs (EBER) more frequently identified. Diffuse large B cell lymphoma (DLBCL) was the main pathological type, and the digestive system was the most extranodal involvement site in m-PTLD after KT and HSCT. Among the 28 patients with DLBCL m-PTLD,the complete remission rate after rituximab treatment was higher than in patients not administered rituximab treatment ( = 0.038). With a median follow-up of 46 months after m-PTLD diagnosis, the estimated 5-year overall survival (OS) was 59.2 ± 9.1% in all patients, and 64.2 ± 11.8 and 52.7 ± 14.1% in the KT and HSCT groups, respectively ( = 0.741). ECOG PS, Ann Arbor stage, and CD68 IHC expression were independent prognostic factors for OS. M-PTLD is a rare but serious complication after transplantation. Ongoing efforts to standardize safe and effective treatment protocols would improve the poor overall survival. The independent prognostic factors contributed to risk-stratified treatment, and might be validated by larger studies.

Epstein-Barr virus-positive post-transplant lymphoproliferative disordepresenting as hematochezia and enterobrosis in renal transplant recipients in China: A report of two cases

“…However, in China, the dominant tumors after kidney transplantation are urothelial carcinomas, and experience in the diagnosis and treatment of PTLD is obviously insufficient. PTLD is accompanied by extranodal infiltration in 90% of patients, and PTLD with gastrointestinal involvement accounts for approximately 15%[2], which is probably due to the rich lymphatic system in the gastrointestinal tract. The two cases reported here were both EBV positive, and PTLD was mainly manifested with hematochezia and enterobrosis, the disease was thus highly concealed and easy to be misdiagnosed.…”

Management of Epstein–Barr virus-related post-transplant lymphoproliferative disorder after allogeneic hematopoietic stem cell transplantation

“…1 Extranodal involvement is common, 45,46 and lesions may invade the liver, gastrointestinal tract, spleen, lung, and others. 6,13,24,40 Fever and lymphadenopathy are the most common symptoms and signs. 2,3,24,40,47 If not treated promptly, this rapidly progressive disease may soon lead to multiple organ dysfunction and death.…”

“…11 EBV-PTLD following allo-HSCT is almost exclusively of donor origin and generally develops during the second to fourth month after transplantation. 4,6,13–17 However, no characteristic signature of oncogene expression or mutation in EBV-PTLD has been identified. 8 Sporadically, EBV invades T or NK cells and leads to T-/NK- EBV-PTLD.…”

The Tumor Microenvironment in Post-Transplant Lymphoproliferative Disorders

“…In the PT-DLBCL the infiltration of cytotoxic T cells (CD3+, TIA-1+) and high levels of PD-1 positive lymphocytes [56] and macrophages (CD68+) [116] have been associated with better overall survival [102], while no effect on survival has been found for regulatory T cell (FoxP3+) counts [56,102] or galectin-1+ cytotoxic T cell counts [56]. The EBV+ malignant B cell population is so dominant in monomorphic PTLD that non-malignant B cells are virtually absent and not much is known of their role in the microenvironment.…”