scholarly journals Zebras and hen's teeth: recognition and management of rare T and NK lymphomas

Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 545-549 ◽  
Author(s):  
Neha Mehta–Shah ◽  
Steven Horwitz

Abstract Although all the peripheral T-cell lymphomas are uncommon, there are some entities that are truly rare. Subtypes, such as enteropathy-associated T-cell lymphoma, hepatosplenic T-cell lymphoma, extranodal NK/T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma, have an approximate annual incidence in United States of <500 each. In these very rare subtypes, there is limited data to guide clinical decision-making. As such, our treatment decisions are often based on extrapolation, case series, personal experience, and biases. We summarize the existing data regarding initial management of these entities and compare how that management follows paradigms established for the more common T-cell lymphomas.

2015 ◽  
Vol 8 (2) ◽  
pp. 78-84 ◽  
Author(s):  
Philippa Ashmore ◽  
Moosa Patel ◽  
Jenifer Vaughan ◽  
Tracey Wiggill ◽  
Pascale Willem ◽  
...  

2015 ◽  
Vol 9 ◽  
pp. CMO.S35120 ◽  
Author(s):  
Feryal A. Ibrahim ◽  
Vignesh Shanmugam ◽  
Aliaa Amer ◽  
Halima El-Omri ◽  
Ahmad Al-Sabbagh ◽  
...  

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδT-cell receptor (TCR), but recently, a few cases have been shown to express the αß TCR. Comparison of these two subtypes (αβ and γδ) shows similar clinicopathologic and cytogenetic features; however, due to the paucity of reported cases, it is not clear whether they are prognostically distinct entities. We report a case of αβ HSTCL with a rather unusual presentation of Coombs'-negative hemolytic anemia. Diagnosis proved challenging due to an unusual blastoid morphology with the absence of typical intrasinusoidal distribution of tumor cells in the bone marrow. This unique case adds to the growing list of this rare subtype of T-cell lymphomas, which warrant urgent attention due to the lack of effective treatment options and dismal prognosis.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4960-4960
Author(s):  
Elizabeth J Heller ◽  
Sarah L Williams ◽  
Keira P Smith ◽  
Steven M. Horwitz

Abstract Background: Therapeutic advances have made the treatment of T-cell lymphomas (TCLs) more effective, less toxic, and more individualized, but research indicates that the multidisciplinary team faces challenges in maintaining a working knowledge of evolving data that can inform clinical decision making. This study was conducted to determine if an online, case-based continuing medical education (CME)/nursing continuing professional development (NCPD) activity could improve clinicians' skills regarding the individualized treatment of patients with TCLs. The educational intervention was intended for all members of the multidisciplinary team: hematologist-oncologists, oncology advanced practitioners and nurses, and other health care professionals involved in the treatment of patients with TCLs. Methods: The CME/NCPD-approved live webinar series titled Update on Therapeutic Advances in T-Cell Lymphoma was presented on March 11, 2021 and March 15, 2021, and was made accessible as a CME/NCPD-approved enduring webinar archive starting on March 18, 2021. Learners participated in a 1-hour online activity regarding current challenges and emerging opportunities in the management of newly diagnosed and relapsed/refractory TCLs. The presentation highlighted mechanisms of action, efficacy, and tolerability of newer therapeutic agents, along with adverse event management strategies. Learners engaged in active learning during case studies that explored patient and disease characteristics that inform care, risk stratification, and treatment. Prior to the activity and following its completion, learners were given a repeated pairs pre- and post-activity assessment consisting of case-based questions that gauged their ability to apply emerging data and guideline recommendations to clinical decision making. Each learner served as their own control. The percentages of learners obtaining correct responses on the pre-activity assessment were compared to those on the post-activity assessment using a chi-squared test. Additionally, learners completed an evaluation of the educational content and self-reported the influence that they judged the activity would have on their future practice. Results: As of July 29, 2021, 218 clinicians had completed the activity for credit. Online education had a significant impact on gains in knowledge and competence in the selection of personalized treatment for patients with TCLs (Figure 1). Learners demonstrated substantial improvements in knowledge of the superior efficacy of mogamulizumab versus vorinostat for a patient with previously treated stage IVB mycosis fungoides (55.67% mean improvement on the post-activity assessment, P &lt; .000001); of the association of the TET2 mutation with angioimmunoblastic T-cell lymphoma (AITL) (54.35% improvement, P &lt; .000001); of the predicted efficacy of oral azacitidine/CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) for an older patient with peripheral T-cell lymphoma-T follicular helper (PTCL-TFH) (31.28% improvement, P &lt; .000001); of the appropriateness of granulocyte colony-stimulating factor (G-CSF) prophylaxis for a patient with CD30-positive PTCL commencing treatment with brentuximab vedotin/CHP (cyclophosphamide/doxorubicin/prednisone) (19.90% improvement, P &lt; .000001); and of the superior efficacy of brentuximab vedotin versus methotrexate for CD30-positive primary cutaneous anaplastic large cell lymphoma (pcALCL) (17.33% improvement, P &lt; .000001). The case-based assessment questions and answer choices can be seen in Table 1. Learners' responses on the activity evaluation can be seen in Figures 2 and 3. Conclusions: These data indicate that online, case-based CME/NCPD-approved activities can result in statistically significant improvements in clinicians' knowledge of therapeutic advances and can increase their sense of competence and confidence in personalizing treatment plans and managing treatment-related adverse events for patients with TCLs. Acknowledgements: This activity was supported by independent educational grants from Kyowa Kirin and Seagen. Figure 1 Figure 1. Disclosures Horwitz: Affimed: Research Funding; Aileron: Research Funding; ADC Therapeutics, Affimed, Aileron, Celgene, Daiichi Sankyo, Forty Seven, Inc., Kyowa Hakko Kirin, Millennium /Takeda, Seattle Genetics, Trillium Therapeutics, and Verastem/SecuraBio.: Consultancy, Research Funding; Acrotech Biopharma, Affimed, ADC Therapeutics, Astex, Merck, Portola Pharma, C4 Therapeutics, Celgene, Janssen, Kura Oncology, Kyowa Hakko Kirin, Myeloid Therapeutics, ONO Pharmaceuticals, Seattle Genetics, Shoreline Biosciences, Inc, Takeda, Trillium Th: Consultancy; Celgene: Research Funding; C4 Therapeutics: Consultancy; Crispr Therapeutics: Research Funding; Daiichi Sankyo: Research Funding; Forty Seven, Inc.: Research Funding; Kura Oncology: Consultancy; Kyowa Hakko Kirin: Consultancy, Research Funding; Millennium/Takeda: Research Funding; Myeloid Therapeutics: Consultancy; ONO Pharmaceuticals: Consultancy; Seattle Genetics: Consultancy, Research Funding; Secura Bio: Consultancy; Shoreline Biosciences, Inc.: Consultancy; Takeda: Consultancy; Trillium Therapeutics: Consultancy, Research Funding; Tubulis: Consultancy; Verastem/Securabio: Research Funding.


2003 ◽  
Vol 127 (3) ◽  
pp. e119-e122
Author(s):  
Jingyu Dong ◽  
Yap-Yee Chong ◽  
Howard J. Meyerson

Abstract Hepatosplenic T-cell lymphoma is an uncommon neoplasm characterized by a lymphoid infiltrate within the sinusoids of the liver, spleen, and bone marrow, without significant nodal involvement. The majority of cases express the γδ T-cell receptor and are associated with an isochromosome 7q cytogenetic abnormality. Recently, a small number of cases have been reported that express the αβ T-cell receptor. Here, we report our findings of a case of an S100-positive hepatosplenic αβ T-cell lymphoma in a 20-year-old woman who presented with pancytopenia and hepatosplenomegaly. The case adds to the growing literature of hepatosplenic αβ T-cell lymphomas.


2019 ◽  
Vol 15 (6) ◽  
pp. 307-312 ◽  
Author(s):  
Mridula Krishnan ◽  
Matthew Lunning

Hepatosplenic γ-δ T-cell lymphoma, an exceptionally uncommon subtype of peripheral T-cell lymphomas, commonly presents with advanced-stage disease manifesting with hepatosplenomegaly, cytopenias, and constitutional symptoms. Management of this subset is challenging as a result of the unique presentation and refractory nature to conventional treatment approaches. There is a lack of consensus guidelines for up-front induction strategies, and the role of consolidative autologous or allogeneic stem-cell transplantation is controversial. Prospective studies are lacking, and treatment is often guided by literature on the basis of case series or single-institution studies, lending to expert opinions influencing treatment paradigms.


2020 ◽  
Vol 18 (11) ◽  
pp. 1460-1467
Author(s):  
Steven M. Horwitz ◽  
Stephen Ansell ◽  
Weiyun Z. Ai ◽  
Jeffrey Barnes ◽  
Stefan K. Barta ◽  
...  

Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of T-cell lymphoma associated with an aggressive clinical course and a worse prognosis. HSTCL develops in the setting of chronic immune suppression or immune dysregulation in up to 20% of cases and is most often characterized by spleen, liver, and bone marrow involvement. Diagnosis and management of HSTCL pose significant challenges given the rarity of the disease along with the absence of lymphadenopathy and poor outcome with conventional chemotherapy regimens. These Guidelines Insights focus on the diagnosis and treatment of HSTCL as outlined in the NCCN Guidelines for T-Cell Lymphomas.


2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Laura Alder ◽  
Scott Graupner ◽  
Guanhua Lai ◽  
Victor Yazbeck

Hepatosplenic T-cell lymphomas (TCLs) are a rare, aggressive subset of TCLs, accounting for less than 5% of all peripheral T-cell and natural killer (NK) cell lymphomas. We report the case of a CD3 negative hepatosplenic T-cell lymphoma in a 42-year-old female, who presented with left-sided abdominal pain. She underwent a liver biopsy that showed marked abnormal sinusoidal lymphoid infiltration. PET scan revealed increased splenic and pharyngeal lymph node uptake. Immunophenotype was remarkable for negative CD3, gamma delta T-cell receptor, and alpha beta-T-cell receptor expression. She received 6 cycles of DA-EPOCH, had primary refractory disease and then underwent palliative splenectomy secondary to painful necrosis. Then, she was started on pralatrexate as a single agent and then in combination with romidepsin as a potential bridge to an allogeneic stem cell transplantation from her sibling.


2016 ◽  
Vol 50 ◽  
pp. 109-117 ◽  
Author(s):  
Mariko Yabe ◽  
L. Jeffrey Medeiros ◽  
Guilin Tang ◽  
Sa A. Wang ◽  
Keyur P. Patel ◽  
...  

Blood ◽  
2003 ◽  
Vol 102 (6) ◽  
pp. 2213-2219 ◽  
Author(s):  
Marcel W. Bekkenk ◽  
Maarten H. Vermeer ◽  
Patty M. Jansen ◽  
Ariënne M. W. van Marion ◽  
Marijke R. Canninga-van Dijk ◽  
...  

Abstract In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30– peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30– large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30– large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3+CD4+CD8– phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4+ or CD8+ phenotype. The only exception was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3+CD4+CD8– phenotype and presenting with localized skin lesions.


2009 ◽  
Vol 2 (6) ◽  
pp. 611-614 ◽  
Author(s):  
Jenny Vu Pozadzides ◽  
Barbara Pro

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