B cell lymphoproliferative disorders following hematopoietic stem cell transplantation: risk factors, treatment and outcome

Abstract

Summary:Twenty-six cases of B cell lymphoproliferative disorder (BLPD) were identified among 2395 patients following hematopoietic stem cell transplants (HSCT) for which an overall incidence of BLPD was 1.2%. The true incidence was probably higher, since 9/26 of the diagnoses were made at autopsy. No BLPD was observed following autologous HSCT, so risk factor analyses were confined to the 1542 allogeneic HSCT. Factors assessed were HLA-mismatching (у1 antigen), T cell depletion (TCD), presence of acute GvHD (grades II-IV), donor type (related vs unrelated), age of recipient and donor, and underlying disease. Factors found to be statistically significant included patients transplanted for immune deficiency and CML, donor age у18 years, TCD, and HLA-mismatching, with recipients of combined TCD and HLA-mismatched grafts having the highest incidence. Factors found to be statistically significant in a multiple regression analysis were TCD, donor age and immune deficiency, although 7/8 of the patients with immunodeficiencies and BLPD received a TCD graft from a haploidentical parent. The overall mortality was 92% (24/26). One patient had a spontaneous remission, but subsequently died Ͼ1 year later of chronic GVHD. Thirteen patients received therapy for BLPD. Three patients received lymphocyte infusions without response. The only patients with responses and longterm survival received alpha interferon (␣IFN). Of seven patients treated with ␣IFN there were four responses (one partial and three complete). These data demonstrate that ␣IFN can be an effective agent against BLPD following HSCT, if a timely diagnosis is made.

Monitoring of Epstein‐Barr virus load after hematopoietic stem cell transplantation for early intervention in post‐transplant lymphoproliferative disease

“…In several studies the median time from stem cell transplantation to diagnosis lymphoproliferative disease was 76 days [Simon et al, 1991], 71.5 days [Micallef et al, 1998] and 86 days [Gross et al, 1999], respectively. According to these data, in the presented study first clinical signs of post-transplant lymphoproliferative disease occurred on days +50, +66, and +90, respectively.…”

“…According to these data, in the presented study first clinical signs of post-transplant lymphoproliferative disease occurred on days +50, +66, and +90, respectively. Earlyonset forms often show a rapid disease progression with a median survival of 0.6 months [Gross et al, 1999]. Micallef et al [1998] A disadvantage at present is that EBV polymerase chain reaction is not yet standardized.…”

Features of Epstein-Barr Virus (EBV) reactivation after reduced intensity conditioning allogeneic hematopoietic stem cell transplantation

“…Following allo-HSCT, EBV reactivation and EBV-related proliferations are well recognized complications. [7][8][9][10][11][12] EBV reactivation may be associated with a spectrum of clinical presentations, going from fever to lymphoproliferative diseases (LPDs), which arise as a consequence of an outgrowth of B cells latently infected with EBV in the setting of loss or suppression of normal cytotoxic T-cell surveillance. Established LPD post-allo-HSCT is associated with a significant mortality and morbidity.…”

Donor cell leukemia: insight into cancer stem cells and the stem cell niche

“…[13][14][15] PTLDs have a reported cumulative incidence of 1% to 2% after HCT and most occur within the first 6 months after HCT. The majority of PTLDs after HCT are donor in origin, although host-derived PTLDs after HCT are described.…”