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Allogeneic hematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a CIBMTR analysis

Hemant S. Murthy, Mei‐Jie Zhang, Karen Chen, Sairah Ahmed, Uday Deotare, Siddhartha Ganguly, Ankit Kansagra, Fotios V. Michelis, Taiga Nishihori, Mrinal M. Patnaik, Muhammad Bilal Abid, Mahmoud Aljurf, Yasuyuki Arai, Ulrike Bacher, Talha Badar, Sherif M. Badawy, Karen K. Ballen, Minoo Battiwalla, Amer Beitinjaneh, Nelli Bejanyan, Vijaya Raj Bhatt, Valerie I. Brown, Rodrigo Martino, Jean‐Yves Cahn, Paul Castillo, Jan Černý, Saurabh Chhabra, Edward A. Copelan, Andrew Daly, Bhagirathbhai Dholaria, Miguel Ángel Díaz, César O. Freytes, Michael R. Grunwald, Shahrukh K. Hashmi, Gerhard Hildebrandt, Omer Jamy, Jacinth Joseph, Christopher G. Kanakry, Nandita Khera, Maxwell M. Krem, Yachiyo Kuwatsuka, Hillard M. Lazarus, Lazaros J. Lekakis, Hongtao Liu, Dipenkumar Modi, Pashna N. Munshi, Alberto Mussetti, Neil Palmisiano, Sagar S. Patel, David A. Rizzieri, Sachiko Seo, Mithun Vinod Shah, Akshay Sharma, Melhm Sohl, Scott R. Solomon, Matthew L. Ulrickson, Celalettin Üstün, Marjolein van der Poel, Leo F. Verdonck, John L. Wagner, Trent Wang, Baldeep Wirk, Amer M. Zeidan, Mark R. Litzow, Partow Kebriaei, Christopher S. Hourigan, Daniel J. Weisdorf, Wael Saber, Mohamed A. Kharfan‐Dabaja

2023Blood Advances24 citationsDOIOpen Access PDF

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with a poor prognosis and considered incurable with conventional chemotherapy. Small observational studies reported allogeneic hematopoietic cell transplantation (allo-HCT) offers durable remissions in patients with BPDCN. We report an analysis of patients with BPDCN who received an allo-HCT, using data reported to the Center for International Blood and Marrow Transplant Research (CIBMTR). We identified 164 patients with BPDCN from 78 centers who underwent allo-HCT between 2007 and 2018. The 5-year overall survival (OS), disease-free survival (DFS), relapse, and nonrelapse mortality (NRM) rates were 51.2% (95% confidence interval [CI], 42.5-59.8), 44.4% (95% CI, 36.2-52.8), 32.2% (95% CI, 24.7-40.3), and 23.3% (95% CI, 16.9-30.4), respectively. Disease relapse was the most common cause of death. On multivariate analyses, age of ≥60 years was predictive for inferior OS (hazard ratio [HR], 2.16; 95% CI, 1.35-3.46; P = .001), and higher NRM (HR, 2.19; 95% CI, 1.13-4.22; P = .02). Remission status at time of allo-HCT (CR2/primary induction failure/relapse vs CR1) was predictive of inferior OS (HR, 1.87; 95% CI, 1.14-3.06; P = .01) and DFS (HR, 1.75; 95% CI, 1.11-2.76; P = .02). Use of myeloablative conditioning with total body irradiation (MAC-TBI) was predictive of improved DFS and reduced relapse risk. Allo-HCT is effective in providing durable remissions and long-term survival in BPDCN. Younger age and allo-HCT in CR1 predicted for improved survival, whereas MAC-TBI predicted for less relapse and improved DFS. Novel strategies incorporating allo-HCT are needed to further improve outcomes.

Topics & Concepts

MedicineHazard ratioInternal medicineTransplantationConfidence intervalTotal body irradiationGastroenterologyHematopoietic stem cell transplantationOncologyHematopoietic cellProportional hazards modelChemotherapyHaematopoiesisCyclophosphamideStem cellBiologyGeneticsCutaneous lymphoproliferative disorders researchFungal Infections and StudiesCAR-T cell therapy research
Allogeneic hematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a CIBMTR analysis | Litcius