Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Prolymphocytic Leukemia

<b>Background:</b> T-prolymphocytic leukemia (T-PLL) is a rare lymphoid neoplasm with particularly poor prognosis. Although it is no longer recognized as a distinct entity by the World Health Organization (WHO), B-prolymphocytic leukemia (B-PLL) comprises conditions with unfavorable outcomes. Both diseases most frequently affect patients in the seventh decade of their lives. Allogeneic hematopoietic stem cell transplantation (alloHSCT) significantly improves outcomes for selected PLL cases, as shown by several, mostly retrospective, analyses. <b>Methods:</b> In this article, we provide a review of existing PLL analyses, followed by a summary of cases treated at our center. We describe outcomes of six T-PLL and three B-PLL cases receiving alloHSCT at our institution between 2015 and 2022. <b>Results:</b> Despite a post-transplant 4-year cumulative relapse incidence of 61% in our T-PLL series, the median OS was 78 months, because relapse therapy was remarkably successful. All B-PLL patients are alive and relapse-free, with a median follow-up of 54 (range of 11-74) months. A poor pre-transplant Karnofsky performance status (KPS) (≤ 80%) and an HCT comorbidity index (HCT-CI) of ≥3 were significantly associated with post-transplant mortality. <b>Conclusions:</b> The comparatively favorable outcomes in our case series underline the increasing value of alloHSCT in PLL in the current era, as it offers a prospect of cure in selected patients with otherwise very poor prognosis.

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Publication Details

Journal
Journal of Clinical Medicine
Published
2025-04-19
DOI
https://doi.org/10.3390/jcm14082816
Primary Topic
Chronic Lymphocytic Leukemia Research
Type
article
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article

Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Prolymphocytic Leukemia

Alexander Nikoloudis, Andreas Petzer, Thomas Bauer, Sigrid Machherndl‐Spandl et al.
Journal of Clinical Medicine
Chronic Lymphocytic Leukemia Research
article

Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Prolymphocytic Leukemia

Alexander Nikoloudis, Andreas Petzer, Thomas Bauer, Sigrid Machherndl‐Spandl, Christoph Aichinger, Emine Kaynak, Stefanie Kreissl, Veronika Buxhofer‐Ausch, Robert Milanov, Irene Straßl, Michael Girschikofsky, Johannes Clausen, Dagmar Wipplinger, Stefanie Nocker, Ansgar Weltermann, Petra Hasengruber, Michaela Binder, Olga Saini, Christina Groiss
article en

Abstract

<b>Background:</b> T-prolymphocytic leukemia (T-PLL) is a rare lymphoid neoplasm with particularly poor prognosis. Although it is no longer recognized as a distinct entity by the World Health Organization (WHO), B-prolymphocytic leukemia (B-PLL) comprises conditions with unfavorable outcomes. Both diseases most frequently affect patients in the seventh decade of their lives. Allogeneic hematopoietic stem cell transplantation (alloHSCT) significantly improves outcomes for selected PLL cases, as shown by several, mostly retrospective, analyses. <b>Methods:</b> In this article, we provide a review of existing PLL analyses, followed by a summary of cases treated at our center. We describe outcomes of six T-PLL and three B-PLL cases receiving alloHSCT at our institution between 2015 and 2022. <b>Results:</b> Despite a post-transplant 4-year cumulative relapse incidence of 61% in our T-PLL series, the median OS was 78 months, because relapse therapy was remarkably successful. All B-PLL patients are alive and relapse-free, with a median follow-up of 54 (range of 11-74) months. A poor pre-transplant Karnofsky performance status (KPS) (≤ 80%) and an HCT comorbidity index (HCT-CI) of ≥3 were significantly associated with post-transplant mortality. <b>Conclusions:</b> The comparatively favorable outcomes in our case series underline the increasing value of alloHSCT in PLL in the current era, as it offers a prospect of cure in selected patients with otherwise very poor prognosis.

Journal of Clinical MedicineVol. 14(8)
Johannes Kepler University of Linz (AT), Krankenhaus der Elisabethinen (AT)
Good health and well-being
Openalex Percentile: Top 88%
Chronic Lymphocytic Leukemia Research
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