Peripheral T-cell lymphoma (PTCL-NOS)

chłoniak z obwodowych komórek T, gdzie indziej niesklasyfikowany, stanowi ok. połowy wszystkich chłoniaków T-komórkowych. Praca prezentuje jego dominującą lokalizację węzłową oraz dość agresywny przebieg kliniczny. opracowany dla niego wskaźnik prognostyczny PIT dość dobrze stratyfikuje pacjentów na grupy o różnych szansach uzyskania 5-letniego przeżycia. Ustalenie rozpoznania bywa trudne ze względu na heterogenność obrazu histopatologicznego. często opiera się ono bardziej na wykluczeniu innych nowotworów T-komórkowych niż stwierdzeniu charakterystycznego obrazu. Niemniej obowiązuje analiza wielu markerów fenotypowych limfocytów oraz genetycznych. w leczeniu nie ustalono dotąd specyficznych standardów postępowania i sprowadza się ono do stosowania schematów dla chłoniaków agresywnych. SłowaPeripheral T-cell lymphoma unspecified represents approx. 50% of all T-cell lymphoma. The paper presents its mostly nodal localization and aggressive clinical course. Its prognostic index PIT well stratificates patients to different 5-year survival groups. The diagnosis is sometimes difficult because of heterogeneity of the histopathological picture. For this reason exclusion of other distinct T-cell malignancies is very important for final PTCL-NOS diagnosis. Nevertheless analysis of many fenotypic as well as genotypic markers of the lymphocytes is necessary. Until now there are not specific standards of treatment for PTCL-NOS. The schemas for aggressive lymphoma are the front line approaches and HDT-ASCT

Very rare solitary primary peripheral nerve onset cytotoxic molecule-positive peripheral T-cell lymphoma (PTCL)

Here we present the first report of solitary primary peripheral nerve onset cytotoxic molecule (CM)-positive peripheral T-cell lymphoma (PTCL) diagnosed after nerve biopsy. An 84-year-old female with rheumatoid arthritis (RA) complained of asymmetric severe tenderness in her upper limbs. The biopsy pathology revealed a direct invasion of CM-positive PTCL. When RA patients complain of numbness, tenderness, or weakness, lymphomatic peripheral nerve invasion should be considered

Romidepsin for the treatment of relapsed/refractory peripheral T cell lymphoma: prolonged stable disease provides clinical benefits for patients in the pivotal trial.

BACKGROUND: Achievement of durable responses in patients with relapsed/refractory peripheral T cell lymphoma (PTCL) is challenging with current therapies, and there are few data regarding the potential benefits of continuing treatment in patients with the best response of stable disease (SD). Histone deacetylase inhibitors are a novel class of drugs with activity in T cell malignancies. Romidepsin was approved by the US Food and Drug Administration for the treatment of relapsed/refractory PTCL based on a pivotal trial demonstrating an objective response rate of 25 % (33/130), including 15 % with confirmed/unconfirmed complete response and a median duration of response of 28 months. Our objective was to further study the clinical benefits of romidepsin in patients that had the best response of SD. METHODS: Patients with PTCL relapsed/refractory to ≥1 prior therapy were treated with the approved dose of 14 mg/m(2) romidepsin on days 1, 8, and 15 of six 28-day cycles; patients with SD or response after cycle 6 were allowed to continue on study until progression. By protocol amendment, patients treated for ≥12 cycles could receive maintenance dosing twice per cycle; after cycle 24, dosing could be further reduced to once per cycle in those who had received maintenance dosing for ≥6 months. RESULTS: Of the 32 patients (25 %) with the best response of SD, 22 had SD for ≥90 days (SD90; cycle 4 response assessment). The longest SD was \u3e3 years in a patient who received maintenance dosing of 14 mg/m(2) on days 1 and 15 beginning in cycle 13. Patients with the best response of SD90 or partial response achieved similar overall and progression-free survival. Prolonged dosing of romidepsin was well tolerated. CONCLUSIONS: We concluded that patients who achieve SD may consider continuing treatment because the clinical benefits of romidepsin may extend beyond objective responses. TRIAL REGISTRATION: NCT00426764

Peripheral T-Cell Lymphoma: Review and Updates of Current Management Strategies

The classification of T-cell and natural-killer- (NK-) cell lymphomas has been updated in the 4th edition of the World Health Organization (WHO) classification of tumors of the haematopoietic and lymphoid tissue published in 2008. Based on recent epidemiological studies, NK-cell lymphomas occur almost exclusively in Asia and South America, although T-cell lymphomas appear to occur in the East as commonly as in the West. Due to the low prevalence of this disease, diagnosis and optimal treatment of patients have not been studied prospectively in large randomized trials. Nevertheless, there has been development in the understanding of T-cell lymphomas and how they should be managed; FDG-PET emerges as an increasingly important tool in diagnosis, gene-expression signatures may aid with prognostication in the future, and novel therapies are currently being studied to improve outcomes in T-cell lymphomas. More work, however, needs to be done, and international collaboration will be pertinent to deriving meaningful results from future clinical studies

Rapid Remission in Peripheral T-Cell Lymphoma of the Nasal Type by the Bortezomib plus CHOP Therapy

Peripheral T-cell lymphoma (PTCL) is rare and difficult to treat for its high relapse rate. The authors report a case of PTCL of the skin, regarding which clinical and pathological features, treatment, and prognosis were discussed. A 66-year-old woman was admitted with complaints of enlarging erythematous noduloplaques on the right anterior tibial skin for one year and similar lesions on the left for 6 months. Surgical resection of right leg lesion and biopsy of enlarged inguinal lymph nodes histologically indicated a PTCL of the nasal type. The patient was treated by CHOP plus bortezomib, reached complete remission just after two courses of chemotherapy and then received another two as consolidation. The patient remained in remission for 11 months until local relapse. As for cutaneous lesions, detailed lymph node examination and prompt tissue biopsy are judicious choices prior to any medical management. The chemotherapy consisting of bortezomib and CHOP is safe and efficient in PTCL of the skin

High-Dose Therapy and Autologous Hematopoietic Cell Transplantation in Peripheral T Cell Lymphoma (PTCL): Analysis of Prognostic Factors

Patients with peripheral T cell lymphoma (PTCL) have a poor prognosis with current treatment approaches. We examined the outcomes of high-dose therapy (HDT) and autologous hematopoietic cell transplant (AHCT) on the treatment of PTCL and the impact of patient/disease features on long-term outcome. Sixty-seven patients with PTCL–not otherwise specified (n = 30), anaplastic large cell lymphoma (n = 30), and angioimmunoblastic T cell lymphoma (n = 7) underwent HDT/AHCT at the City of Hope. The median age was 48 years (range: 5-78). Twelve were transplanted in first complete remission (1CR)/partial remission (PR) and 55 with relapsed or induction failure disease (RL/IF). With a median follow-up for surviving patients of 65.8 months (range: 24.5-216.0) the 5-year overall survival (OS) and progression-free survival (PFS) were 54% and 40%, respectively. The 5-year PFS was 75% for 1CR/PR compared to 32% for RL/IF patients (P = .01). When the Prognostic Index for PTCL unspecified (PIT) was applied at the time of transplant, patients in the PIT 3-4 group had 5-year PFS of only 8%. These results show that HDT/AHCT can improve long-term disease control in relapsed/refractory PTCL and that HDT/AHCT should ideally be applied either during 1CR/PR, or as part of upfront treatment. More effective and novel therapies are needed for patients with high-risk disease (PIT 3-4 factors) and allogeneic HCT should be explored in these patients

Peripheral T-cell lymphoma of the eyelid

Makoto Ishikawa, Hiroshi Watabe, Masahiro Hayakawa, Takeshi YoshitomiDepartment of Ophthalmology, Akita University Faculty of Medicine, Akita, JapanPurpose: To report a case of a 25-year-old woman with previously treated peripheral T-cell lymphoma (PTCL) presenting with a recurrent lower eyelid lesion.Patients and method: Case report.Results: A 25-year-old young woman with previously treated PTCL noted an induration in the skin of her left lower eyelid. Upon diagnosis of a chalazion, antibiotic eye drops and ointments as well as steroid eye drops were administered. However, the condition worsened rapidly and swelling of the lower eyelid became remarkable. An excisional biopsy revealed that the palpebral lesion was diagnosed as recurrence of PTCL. Electron irradiation was applied as a radical treatment, and the prognosis was satisfactory without recurrence at 10 months after the last irradiation.Conclusions: Although lymphoma isolated to the ocular adnexa is rare, it should always be included in the differential diagnosis of any patient presenting with progressive swelling of the eyelid or ocular region.Keywords: peripheral T-cell lymphoma, chalazion, eyeli