Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study

Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder with a short survival duration despite the use of novel therapeutics. PCL remains an understudied disease for which there is no current standard of care treatment. Knowledge on optimal novel drug sequencing, including the role...

Full description

Saved in:
Bibliographic Details
Main Authors: Shagun Singh, Supriya Peshin, Betsy C. Wertheim, Ashley Larsen, Iloabueke Chineke, Douglas W. Sborov, Damian Green, Michaela Liedtke, Marie Okoniewski, Mohammed Wazir, Omar Nadeem, Levanto G. Schachter, Dominique DeGraaff, Victoria A. Vardell, David G. Coffey, Krisstina Gowin
Format: Article
Language:English
Published: Ferrata Storti Foundation 2025-04-01
Series:Haematologica
Online Access:https://haematologica.org/article/view/12031
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850197690147667968
author Shagun Singh
Supriya Peshin
Betsy C. Wertheim
Ashley Larsen
Iloabueke Chineke
Douglas W. Sborov
Damian Green
Michaela Liedtke
Marie Okoniewski
Mohammed Wazir
Omar Nadeem
Levanto G. Schachter
Dominique DeGraaff
Victoria A. Vardell
David G. Coffey
Krisstina Gowin
author_facet Shagun Singh
Supriya Peshin
Betsy C. Wertheim
Ashley Larsen
Iloabueke Chineke
Douglas W. Sborov
Damian Green
Michaela Liedtke
Marie Okoniewski
Mohammed Wazir
Omar Nadeem
Levanto G. Schachter
Dominique DeGraaff
Victoria A. Vardell
David G. Coffey
Krisstina Gowin
author_sort Shagun Singh
collection DOAJ
description Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder with a short survival duration despite the use of novel therapeutics. PCL remains an understudied disease for which there is no current standard of care treatment. Knowledge on optimal novel drug sequencing, including the role and timing of hematopoietic stem cell transplant as primary and salvage therapy is needed. We conducted a large retrospective analysis of 153 PCL patients, using the new definition of ≥ 5% circulating plasma cells, including clinical features and treatment outcomes across seven academic centers in United States. This is, to the best of our knowledge, the largest multicenter study conducted in the US of this rare disease. Disease presentation, clinical and genetic characteristics, and treatment patterns of 93 patients with primary PCL and 57 with secondary PCL were described. Additionally, associations between patient characteristics and mortality were investigated using Cox proportional-hazards regression models. Secondary PCL was associated with a worse prognosis, with a median overall survival of 3.2 months compared to 36.6 months for primary PCL (p
format Article
id doaj-art-d6123ae67daa47f784e0ff1486a25da1
institution OA Journals
issn 0390-6078
1592-8721
language English
publishDate 2025-04-01
publisher Ferrata Storti Foundation
record_format Article
series Haematologica
spelling doaj-art-d6123ae67daa47f784e0ff1486a25da12025-08-20T02:13:03ZengFerrata Storti FoundationHaematologica0390-60781592-87212025-04-01999110.3324/haematol.2024.287158Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort studyShagun Singh0Supriya Peshin1Betsy C. Wertheim2Ashley Larsen3Iloabueke Chineke4Douglas W. Sborov5Damian Green6Michaela Liedtke7Marie Okoniewski8Mohammed Wazir9Omar Nadeem10Levanto G. Schachter11Dominique DeGraaff12Victoria A. Vardell13David G. Coffey14Krisstina Gowin15Internal Medicine, Banner University Medical CenterNorton Community Hospital, Ballad HealthUniversity of Arizona Cancer CenterDepartment of Medicine, University of ArizonaRocky Mountain Oncology, Casper, WYHuntsman Cancer Institute at the University of Utah, Salt Lake City, UTUniversity of Miami, Miller School of Medicine, Miami, FLStandford Medicine, Department of Hematology, Palo Alto, CAUniversity of Kansas Cancer Center, Kansas City, KSUMass Memorial Medical Center, Division of Hematology/Oncology, Worcester, MAHarvard Medical School, Dana-Faber Cancer InstituteUCLA Health, Hematology Oncology, San Luis Obispo, CAUniversity of Washington, Department of Oncology and Hematology, Seattle, WAUniversity of Utah, Huntsman Cancer Institute, Salt Lake City, UTDivison of Myeloma, Sylvester Comprehensive Cancer Center, University of Miami, Miami, FLDepartment of Supportive Care Medicine, Hematology/HCT, City of Hope, CA Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder with a short survival duration despite the use of novel therapeutics. PCL remains an understudied disease for which there is no current standard of care treatment. Knowledge on optimal novel drug sequencing, including the role and timing of hematopoietic stem cell transplant as primary and salvage therapy is needed. We conducted a large retrospective analysis of 153 PCL patients, using the new definition of ≥ 5% circulating plasma cells, including clinical features and treatment outcomes across seven academic centers in United States. This is, to the best of our knowledge, the largest multicenter study conducted in the US of this rare disease. Disease presentation, clinical and genetic characteristics, and treatment patterns of 93 patients with primary PCL and 57 with secondary PCL were described. Additionally, associations between patient characteristics and mortality were investigated using Cox proportional-hazards regression models. Secondary PCL was associated with a worse prognosis, with a median overall survival of 3.2 months compared to 36.6 months for primary PCL (phttps://haematologica.org/article/view/12031
spellingShingle Shagun Singh
Supriya Peshin
Betsy C. Wertheim
Ashley Larsen
Iloabueke Chineke
Douglas W. Sborov
Damian Green
Michaela Liedtke
Marie Okoniewski
Mohammed Wazir
Omar Nadeem
Levanto G. Schachter
Dominique DeGraaff
Victoria A. Vardell
David G. Coffey
Krisstina Gowin
Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study
Haematologica
title Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study
title_full Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study
title_fullStr Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study
title_full_unstemmed Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study
title_short Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study
title_sort outcomes and treatment patterns in primary and secondary plasma cell leukemia insights from a large us cohort study
url https://haematologica.org/article/view/12031
work_keys_str_mv AT shagunsingh outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT supriyapeshin outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT betsycwertheim outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT ashleylarsen outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT iloabuekechineke outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT douglaswsborov outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT damiangreen outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT michaelaliedtke outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT marieokoniewski outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT mohammedwazir outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT omarnadeem outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT levantogschachter outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT dominiquedegraaff outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT victoriaavardell outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT davidgcoffey outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy
AT krisstinagowin outcomesandtreatmentpatternsinprimaryandsecondaryplasmacellleukemiainsightsfromalargeuscohortstudy