Options for patients with relapsed or refractory (R/R) classical Hodgkin lymphoma (cHL) after brentuximab vedotin (Bv) and autologous stem cell transplantation (ASCT) are limited. Immune checkpoint inhibitors (ICI) are active in this population but rarely induce complete response (CR). Ten patients with R/R cHL after ASCT and Bv received pembrolizumab (n = 8) or nivolumab (n = 2). Five had been previously exposed to 5-azacitidine on a phase 1 study. Among nine evaluable patients, seven (78%) achieved CR, one partial response, and one reduction of tumor burden. All five patients who had received 5-azacitidine prior to ICI achieved CR, while only two of four who did not receive prior 5-azacitidine achieved CR. At a median follow-up of 9.9 months [0.5–14.3], eight patients are alive and five are still receiving treatment. We documented an unprecedented CR rate after ICI in patients with R/R cHL. We hypothesize that hypomethylating agents might have an immune priming effect and enhance the efficacy of ICI.Electronic supplementary materialThe online version of this article (doi:10.1186/s13045-016-0363-1) contains supplementary material, which is available to authorized users.
It has been nearly 8 years since pralatrexate became the first drug approved by the U.S. Food and Drug Administration for the treatment of relapsed or refractory peripheral T-cell lymphoma (PTCL). Like most drugs approved for a particular clinical indication, as much or more is learned once it enters mainstream use as in the years leading up to regulatory approval. Over the past several years, many diverse lines of research have shed new insight into both the agent, and the diseases it treats. In this review, we will bring the reader up to date on the many new aspects related to pralatrexate's pharmacology, activity across the panoply of T-cell lymphoproliferative malignancies, as well as some new and emerging guidelines that are likely to improve its safety profile. Finally, the review will close with the many new lines of evidence building a rationale for the combination of these novels: novel combination, and the vision for new platforms in PTCL care.
Systemic Castleman's disease is a lymphoproliferative disorder which has been associated with autoimmune phenomena. We report a case of the plasma cell variant of Castleman's disease that was associated with autoimmune hemolytic anemia and autoimmune thrombocytopenia (Evan's syndrome), and vitiligo, the association of which has not been previously reported.
The case history of a 71-year-old woman with three episodes of a microangiopathic hemolytic anemia over a 22-year span is detailed. During the last episode a possible response of her thrombotic thrombocytopenic purpura (TTP)-like syndrome to the administration of intravenous immunoglobulin is documented. In retrospect it became apparent that she only improved in her prior episodes after receiving plasma-containing blood products. Prior case reports of TTP responses to intravenous immunoglobulin are reviewed with specific attention to the dosage used. Since a prospectively randomized series is unlikely to be reported, investigators should be encouraged to report their experience with intravenously administered gamma globulin.
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