Advances in Cellular Therapy for T-Cell Prolymphocytic Leukemia

Varadarajan, Indumathy; Ballen, Karen K.

doi:10.3389/fonc.2022.781479

Cited by 3 publications

(3 citation statements)

References 40 publications

(58 reference statements)

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“…It has shown overall response rates (ORRs) of 90% or higher and complete response of 81% in comparison with traditional chemotherapy. 15,18,19 However, despite the good response rate achieved by alemtuzumab, patients who do not receive hematopoietic stem cell transplantation relapse-with median survival of 17 to 33 months. In patients receiving allogeneic stem cell transplantation, the median survival rate is 48 months.…”

Section: Discussionmentioning

confidence: 99%

“…Both forms of stem cell transplantation—autologous and allogeneic—prolong overall survival when compared with isolated induction with alemtuzumab. 1,5,14,19 In patients who have relapse of T-PLL, the combination of alemtuzumab and pentostatin has been shown to achieve an ORR of 75%. Bendamustine can also be used and has shown an ORR of 53.3.…”

Section: Discussionmentioning

confidence: 99%

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Recurrence of T-Cell Prolymphocytic Leukemia With a Rare Presentation as Diffuse Generalized Skin Lesion

Wasifuddin

Sabzposh

Sun

et al. 2023

Journal of Investigative Medicine High Impact Case Reports

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T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive neoplasm that originates from mature post-thymic T-cells. Cutaneous manifestations are a common presentation in T-PLL but rarely are a presentation in the recurrent setting. Here, we describe the case of a 75-year-old female with a history of T-PLL—who at the time of initial diagnosis did not exhibit any rash—presenting with diffuse rash, facial swelling, sore throat, and dysphagia 7 months later and was found to have recurrent T-PLL. She had diffuse lymphadenopathy and diffuse skin lesions. Biopsy of the skin lesions also confirmed infiltration with T-PLL cells. After review of the literature, no previously reported cases of recurrent T-PLL presented as diffuse skin lesions. This case demonstrates that recurrent T-PLL may present with diffuse rash, respiratory distress, and anasarca. It is important to stay vigilant in patients with history of T-PLL to recognize signs of recurrent disease to allow prompt diagnosis and treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Recurrence of T-Cell Prolymphocytic Leukemia With a Rare Presentation as Diffuse Generalized Skin Lesion

Wasifuddin

Sabzposh

Sun

et al. 2023

Journal of Investigative Medicine High Impact Case Reports

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“…Hematopoietic stem cell transplant (HSCT) is the only potentially curative therapy and should be considered in transplant-eligible patients upon achieving a response to cytoreductive treatment (usually alemtuzumab) (recently reviewed in [65]). Several studies demonstrate the benefit associated with allogeneic hematopoietic stem cell transplant (allo-HSCT), and are summarized in Table 4.…”

Section: Stem Cell Transplantationmentioning

confidence: 99%

T-Cell Prolymphocytic Leukemia: Diagnosis, Pathogenesis, and Treatment

Gutierrez,

Bladek,

Goksu

et al. 2023

IJMS

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T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive neoplasm of mature T-cells. Most patients with T-PLL present with lymphocytosis, anemia, thrombocytopenia, and hepatosplenomegaly. Correct identification of T-PLL is essential because treatment for this disease is distinct from that of other T-cell neoplasms. In 2019, the T-PLL International Study Group (TPLL-ISG) established criteria for the diagnosis, staging, and assessment of response to treatment of T-PLL with the goal of harmonizing research efforts and supporting clinical decision-making. T-PLL pathogenesis is commonly driven by T-cell leukemia 1 (TCL1) overexpression and ATM loss, genetic alterations that are incorporated into the TPLL-ISG diagnostic criteria. The cooperativity between TCL1 family members and ATM is seemingly unique to T-PLL across the spectrum of T-cell neoplasms. The role of the T-cell receptor, its downstream kinases, and JAK/STAT signaling are also emerging themes in disease pathogenesis and have obvious therapeutic implications. Despite improved understanding of disease pathogenesis, alemtuzumab remains the frontline therapy in the treatment of naïve patients with indications for treatment given its high response rate. Unfortunately, the responses achieved are rarely durable, and the majority of patients are not candidates for consolidation with hematopoietic stem cell transplantation. Improved understanding of T-PLL pathogenesis has unveiled novel therapeutic vulnerabilities that may change the natural history of this lymphoproliferative neoplasm and will be the focus of this concise review.

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