Peripheral T-Cell Lymphoma, not Otherwise Specified (PTCL-NOS)

Oluwasanjo, Adetokunbo; Kartan, Saritha; Johnson, W. Thomas; Alpdoğan, Önder; Gru, Alejandro A.; Mishra, Anjali; Haverkos, Bradley M.; Gong, Jerald Z.; Porcu, Pierluigi

doi:10.1007/978-3-319-99716-2_4

Cited by 29 publications

(28 citation statements)

References 68 publications

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“…There is a small subgroup of TBX21 ‐expressing PTCL with a poor outcome that express cytotoxic markers and specific cytokine transcripts including CXCR3 , CXCL12 , and were associated with CD8+ cytotoxic cells . Other mutations include the TCR pathways, NOTCH signaling, mutation in RHOA , and hyperactivation of PI3K‐AKT1 pathways …”

Section: Molecular Pathogenesis Of Ptcl Subtypesmentioning

confidence: 99%

Aggressive T‐cell lymphomas: 2019 updates on diagnosis, risk stratification, and management

Zain

2019

American J Hematol

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Introduction Aggressive T‐cell lymphomas continue to have a poor prognosis. There are over 27 different subtypes of peripheral T‐cell lymphoma (PTCL) and we are now beginning to understand the differences between the various subtypes beyond histologic variations. Molecular pathogenesis of various subtypes of PTCL Gene expression profiling can help in diagnosis and prognostication of various subtypes including PTCL‐nos and anaplastic large cell lymphoma. In addition, mutational analysis is now being incorporated in clinical trials of novel agents to evaluate various biomarkers of response to allow better therapeutic choices for patients. Targeted therapies There are many targeted agents currently in various stages of clinical trials for PTCL that take advantage of the differential expression of specific proteins or receptors in PTCL tumors. The most promising is the CD30 directed antibody drug conjugate brentuximab vedotin. This has recently been approved by the Food and Drug Administration for the upfront treatment of CD30 expressing PTCLs in combination with cyclophosphamide, doxorubicin, and prednisone chemotherapy. Other notable targets are CD25, CCR4 tag, PI3kinase inhibitors, and JAK/STAT inhibitors. Anaplastic lymphoma kinase (ALK) inhibitors are promising for ALK expressing tumors. Immunotherapies The use of checkpoint inhibitors in the treatment of PTCL is still controversial. The most promising results have been seen in cases of extranodal natural killer cell/T‐cell lymphomas and cutaneous T‐cell lymphomas. For all other subtypes, immune checkpoint inhibitors should be used with extreme caution and only in the context of a clinical trial. Allogeneic stem cell transplant continues to be the curative therapy for most aggressive subtypes of PTCL.

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Section: Molecular Pathogenesis Of Ptcl Subtypesmentioning

confidence: 99%

Aggressive T‐cell lymphomas: 2019 updates on diagnosis, risk stratification, and management

Zain

2019

American J Hematol

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“…5 The 5-year overall survival rate is 20%-30%. 6 F I G U R E 5 HE staining and CD 3 immunohistochemistry. Circumscribed infiltrates of the tumor next to regular salivary gland tissue.…”

Section: Discussionmentioning

confidence: 99%

“…If the diagnosis of a T‐cell lymphoma is confirmed, the oncologic therapy should be initiated immediately although standard treatment and outcome still remain poor 5 . The 5‐year overall survival rate is 20%‐30% 6 …”

Section: Discussionmentioning

confidence: 99%

Facial nerve paralysis caused by a T‐cell lymphoma

Nachtsheim

Wittersheim

Kreissl

et al. 2020

Clinical Case Reports

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“…Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is a heterogeneous category of nodal and mature T-cell lymphomas that do not correspond to any of the specifically defined entities of mature Tcell lymphoma in the current classification. 1,2 PTCL-NOS is the most common PTCL subtype, accounting for at least 25% of all cases of PTCL. 3 Although PTCL-NOS is a clinically rare disease, it has various clinical manifestations, including impaired immune surveillance, [1][2][3] secondary malignancies, 4 Epstein-Barr virus (EBV) infection, [1][2][3][4] and/or peripheral eosinophilia.…”

Section: Introductionmentioning

confidence: 99%

CCR4-positive peripheral T-cell lymphoma presenting as eosinophilic pneumonia and developing from prolonged pustular psoriasis

et al. 2021

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A 29-year-old woman with chronic, prolonged pustular psoriasis was admitted to our hospital because of high-grade fever and a systemic skin rash. General examination revealed a whole-body skin rash and superficial lymphadenopathy. Peripheral blood examination showed unclassified cells positive for CD3, CD4, and T-cell receptor αβ, and negative for CD20 and CD56. Soon after administration, she developed acute respiratory failure and required artificial ventilation. Bronchoalveolar lavage fluid showed increased numbers of eosinophils and abnormal lymphocytes of the same phenotype in peripheral blood and skin. She was diagnosed with eosinophilic pneumonia, and her respiratory failure was improved by corticosteroid therapy. Based on the histological findings of skin, lymph node, and bone marrow biopsies, a diagnosis of peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), with positivity for CC chemokine receptor 4 was made. She received chemotherapy followed by allogeneic stem cell transplantation, which resulted in complete remission of her PTCL-NOS. She remained alive and disease-free 6 years later. This is the first reported case of PTCL-NOS developing during the clinical course of pustular psoriasis. The clinical manifestations of PTCL-NOS are complex, but an accurate diagnosis and appropriate therapy may produce a good clinical outcome in patients with PTCL-NOS.

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Peripheral T-Cell Lymphoma, not Otherwise Specified (PTCL-NOS)

Cited by 29 publications

References 68 publications

Aggressive T‐cell lymphomas: 2019 updates on diagnosis, risk stratification, and management

Aggressive T‐cell lymphomas: 2019 updates on diagnosis, risk stratification, and management

Facial nerve paralysis caused by a T‐cell lymphoma

CCR4-positive peripheral T-cell lymphoma presenting as eosinophilic pneumonia and developing from prolonged pustular psoriasis

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