Peripheral T-cell lymphoma of the oral cavity: A case report.

BACKGROUND: Peripheral T-Cell Lymphoma Not Otherwise Specified (PTCL-NOS) is a rare type of non-Hodgkin T-cell lymphoma which frequently seen in immunocompromised individuals. It is estimated that only 2% of lymphomas are located on the buccal mucosa. In this case report, we present a 34-year-old male with a PTCL diagnosis. CASE: A 34-year-old immune-competent male presented with a buccal progressive ulcerated lesion. Histopathologic and immunohistochemical findings were compatible with PTCL-NOS and classified as stage IIEA according to the Ann Arbor staging. The patient underwent chemotherapy followed by radiotherapy. He remained disease-free after 12 months of follow-up. CONCLUSION: Although lymphoma is uncommon in the oral cavity, physicians especially dentists in ordinary dental checkups should consider persistent progressive lesions as an important differential diagnosis of lymphoma.

Conditional survival and hazards of death for peripheral T-cell lymphomas.

Typically, peripheral T-cell lymphoma (PTCLs) prognosis is estimated using overall survival before treatment. However, these estimates cannot show how prognosis evolves with the changing hazard rate over time. Patients (n = 650) with newly diagnosed PTCLs were enrolled retrospectively. After a median follow-up of 5.4 years, angioimmunoblastic T-cell lymphoma, peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) and NK/T cell lymphoma had initially lower 3-year conditional overall survival (COS3; i.e., the 3-year conditional overall survival was defined as the probability of surviving an additional 3 years) and higher hazards of death (26-44.3%). However, after 2 years, the COS3 increased and the death risk decreased over time, whereas anaplastic lymphoma kinase-positive anaplastic large-cell lymphoma constantly had a lower risk over time (0-19.5%). For patients with complete remission after initial treatment, prognosis varied by histological subtypes, with PTCL, NOS having a negative impact. Our data suggested that the risk stratification using the International Prognostic Index might not accurately predict the COS3 for survivors of PTCLs. The COS3 provided time-dependent prognostic information for PTCLs, representing a possible surrogate prognosis indicator for long-term survivors after systemic chemotherapy.

New perspectives in the therapeutic approach of peripheral T-cell lymphoma.

PURPOSE OF REVIEW: Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of mature T-cell and natural killer (NK)-cell neoplasms in the WHO 2016 classification. Patient prognosis is poor when treated with CHOP, and there is an unmet need for new drugs. Several agents have been developed for PTCL, and their use is the subject of this review. RECENT FINDINGS: Phase 2 studies demonstrated the activity of new drugs in Relapsed/refractory PTCL. Only four compounds were approved by the food and drug administration: romidepsin and belinostat, which are epigenetic modifiers, the antifolate agent pralatrexate, the immuno-conjugate brentuximab vedotin. New combinations have been tested, but the results were disappointing. Given the latest progress in biology, targeted agents are evaluated in different subtypes of PTCL. Relapsed anaplastic large-cell lymphoma exhibits improved prognosis with the approved anti-CD30 drug conjugate brentuximab vedotin. Localized nasal NK/T is treated with radiotherapy and nonanthracycline chemotherapy with L-asparaginase. Recently, immune checkpoint inhibitors demonstrated activity in NK/T lymphoma and can be used in elderly patients. SUMMARY: Treatment remains a challenge for PTCL, and several targeted drugs provide new approaches. Progress will be made incrementally in the different subtypes. One of the critical situations facing new drugs is the ability to run robust clinical trials in rare diseases.

Clinical and prognostic differences between ALK-negative anaplastic large cell lymphoma and peripheral T cell lymphoma, not otherwise specified: a single institution experience.

Clinical differences between anaplastic lymphoma kinase (ALK)-negative anaplastic large-cell lymphoma (ALK(-) ALCL) and peripheral T cell lymphoma, not otherwise specified (PTCL-NOS), remain unclear. The aim of this study was to compare the clinical and prognostic features of these two lymphoma types. We retrospectively analyzed 167 patients with ALK(-) ALCL (n = 48) and PTCL-NOS (n = 119). Compared with ALK(-) ALCL patients, PTCL-NOS patients exhibited distinct differences in clinical features with a propensity for more advanced stages, frequent extranodal involvement, and a poor performance status, leading to a higher risk group according to the International Prognostic Index or Prognostic Index for PTCL-NOS. Patients with ALK(-) ALCL were associated with a higher complete response rate (47.9 vs. 31.0 %; P = 0.041) after initial chemotherapy than patients with PTCL-NOS. The prognosis was significantly different between two subtypes, with a 5-year overall survival (OS) rate of 57.9 % for ALK(-) ALCL and 23.9 % for PTCL-NOS (P = 0.002). The subgroup analysis showed significant differences in OS and progression-free survival between the two subtypes in early-stage diseases, but not in advanced-stage diseases. We conclude that patients with ALK(-) ALCL showed favorable clinical features, higher chemosensitivity, and a superior outcome than those with PTCL-NOS.

Survival in patients with limited-stage peripheral T-cell lymphomas.

The natural history of limited-stage peripheral T-cell lymphoma (PTCL) remains poorly defined. Therefore, we examined outcomes in patients with the most common PTCL subtypes (PTCL, not otherwise specified [PTCL, NOS], angioimmunoblastic T-cell lymphoma [AITL], anaplastic large cell lymphoma [ALCL]) and limited-stage disease. In this retrospective, multicenter study, 75 patients with limited-stage disease were identified. The median event-free survival (EFS) and overall survival (OS) observed were 2.1 and 6.5 years, respectively. In a landmark analysis excluding patients with primary refractory disease, no significant benefit was observed for patients undergoing consolidative radiation therapy. With the exception of patients undergoing salvage hematopoietic stem cell transplant, survival following disease relapse or progression was poor, thus highlighting the need for improved therapeutic strategies.

Therapeutic effects and influencing factors in sixty-eight cases of peripheral T-cell lymphoma unspecified.

OBJECTIVE: To analyze the clinical characteristics, therapeutic short-term efficacy, long-term survival and influencing factors in 68 cases of peripheral T-cell lymphoma unspecified. METHODS: Sixty-eight cases of peripheral T-cell lymphoma unspecified were retrospectively studied. The effect of different treatments on survival of patients was analyzed by the Kaplan-Meier method. Using single factor analysis and the Cox proportional hazards regression model, the effect of the various clinical factors on the survival of patients was evaluated. RESULTS: The complete remission rate of 68 cases of peripheral T-cell lymphoma unspecified according to treatment was 28%. The 5-year overall survival rate was 25.63%. Chemotherapy alone and chemotherapy combined with radiotherapy gave overall survival rates of 19.4% and 37.1%, respectively. Chemotherapy combined with radiotherapy gave a long-term survival rate significantly superior to that of chemotherapy alone (P <0.05). CONCLUSIONS: Patients with peripheral T-cell lymphoma unspecified treated by the CHOP chemotherapy regimen had a high response rate but a low long-term survival rate. A complete remission with initial treatment and chemotherapy combined with radiotherapy can improve patient survival. The performance score before treatment, therapeutic effect, and bone marrow involvement are independent factors that affect survival.

Impressive activity of lenalidomide monotherapy in refractory angioimmunoblastic T-cell lymphoma: report of a case with long-term follow-up.

Angioimmunoblastic T-cell lymphoma (AITL) is characterized by an aggressive clinical course and unfavourable prognosis. Refractory AITL patients have very few treatment options. Lenalidomide has previously been reported to have clinical efficacy in this setting; however, long-term reports are limited. A 59-year-old man was referred to the hospital with fatigue, skin rash, weight loss and generalized lymphadenopathy and was diagnosed with AITL; clinical stage was IV B with bone marrow involvement. The patient had an unsatisfactory response despite three lines of conventional chemotherapy and radiotherapy. The patient received lenalidomide monotherapy (25 mg once daily) on days 1 to 21 of every 28-day cycle for six cycles, followed by maintenance therapy with six cycles of lenalidomide 15 mg once daily on days 1 to 21 of every 28-day cycle. A computed tomography scan was assessed before lenalidomide treatment, after the third cycle, at disease restaging 2 months after completion of the induction phase, every 3 months during the maintenance phase and every 6 months during the follow-up period. At the last evaluation, after a follow-up of 30 months, the patient maintained a clinical and radiological complete response. The treatment was well tolerated with manageable toxicity. Lenalidomide treatment demonstrated for the first time in the literature impressive and long-term clinical efficacy in a heavily pretreated chemorefractory AITL patient.

The aggressive peripheral T-cell lymphomas: 2012 update on diagnosis, risk stratification, and management.

BACKGROUND: T-cell lymphomas make up approximately 10-15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia. DIAGNOSIS: The diagnosis of aggressive peripheral T-cell lymphoma (PTCL) is usually made using the WHO classification. The ability of hematopathologists to reproducibly diagnose aggressive PTCL is lower than for aggressive B-cell lymphomas, with a range of 72-97% for the aggressive PTCLs. RISK STRATIFICATION: Patients with aggressive PTCL are staged using the Ann Arbor Classification. Although somewhat controversial, positron emission tomography (PET) scans appear to be useful as they are in aggressive B-cell lymphomas. The most commonly used prognostic index is the International Prognostic Index. The specific subtype of aggressive PTCL is an important risk factor, with the best survival seen in anaplastic large-cell lymphoma-particularly young patients with the anaplastic lymphoma kinase positive subtype. RISK ADAPTED THERAPY: Anaplastic large-cell lymphoma is the only subgroup to have a good response to a cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like regimen. Angioimmunoblastic T-cell lymphoma has a prolonged disease-free survival in only ∼20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL-not otherwise specified (NOS) is not one disease. Anthracycline containing regimens have disappointing results and a new approach is needed. NK/T-cell lymphoma localized to the nose and nasal sinuses seems to be best treated with radiotherapy containing regimens. Enteropathy associated PTCL and hepatosplenic PTCL are rare disorders with a generally poor response to therapy, although selected patients with enteropathy associated PTCL seem to benefit from intensive therapy.

[Clinical features and prognostic factors of angioimmunoblastic T cell lymphoma].

OBJECTIVE: To retrospectively analyze the clinical features and prognostic factors of patients with angioimmunoblastic T-cell lymphoma (AITL). METHODS: The clinicopathological and follow-up data of 18 AITL patients undergoing integrated treatment from Feb. 1998 to April 2009 in our department were retrospectively analyzed. All of the patients received CHOP-like regimens as initial chemotherapy, including 4 once treated with radiotherapy and 1 with high dose therapy followed by autologous stem cell transplantation (HDT-ASCT) as upfront consolidation therapy. B-cell, T-cell and NK-cell subgroup proportions in the peripheral blood were tested by flow cytometry in 6 patients. RESULTS: The median age of the 18 patients was 55 years, male and female ratio was 2.6:1. Seventy-two percent of the patients were in an advanced stage. 72% of them had B symptoms, 69% hypergammaglobulinemia, 60% elevated LDH and 47% anemia. Forty-four percent achieved CR after initial treatment with CHOP-like regimens. With the median follow-up of 26 months, the overall 2-year survival and disease free survival (DFS) rates were 62.2% and 44.4%, respectively. In the univariate analysis, only age > 30 years and primary refractory disease adversely affected overall survival (OS); age > 30 years, advanced stage, B symptoms and splenomegaly adversely affected DFS. Four patients suffered from severe pneumonia during treatment, 2 of them died of respiratory failure. Flow cytometry of peripheral blood lymphocytes showed that 5 of the 6 tested cases had decreasing proportion of CD3(+)CD4(+) T cells, B cells and NK cells but elevated CD3(+)CD8(+) T cells. Two heavily treated patients achieved partial and complete response by thalidomide therapy, with a progression free survival (PFS) of 2 and 6+ months, respectively. CONCLUSION: AITL patients do not response well to CHOP-like regimens chemotherapy. Age < 30 years and sensitive to initial chemotherapy are associated with prolonged OS. Effectiveness of thalidomide in the treatment of AITL deserves further investigation. Peripheral blood lymphocytes test indicates that AITL patients suffered from both natural and acquired immune defects.

Primary cutaneous peripheral T-cell lymphoma, unspecified with an indolent clinical course: a distinct peripheral T-cell lymphoma?

Primary cutaneous peripheral T-cell lymphomas (PTL), unspecified, are rare lymphomas, with a poor prognosis. They grow and disseminate rapidly, leading to widespread disease. We report a case of PTL, unspecified occurring on the nose. Despite its aggressive histology, this tumour behaved indolently. It is remarkably similar, clinically and histologically, to four recently described cases that occurred on the ear.

Enhancing existing approaches to peripheral T-cell lymphoma.

The National Comprehensive Cancer Network (NCCN) practice guidelines for peripheral T-cell lymphoma (PTCL) accentuate the lack of standard treatment options for this disease. Outcomes with conventional therapies, many of which are borrowed from B-cell lymphoma, are poor. Strategies to enhance existing approaches include creating a new platform for first-line therapy and adding novel agents, such as denileukin diftitox, to existing chemotherapy platforms. Furthermore, to improve outcomes, patients must reach transplant through effective first-line therapies. Additionally, treatment should be individualized based on histopathologic subtype, as all PTCL patients will not respond to the same treatment regimen.

Clinical features and prognostic factors of patients with "peripheral T cell lymphoma, unspecified".

The purpose of this retrospective study was to investigate clinical features and treatment outcomes in patients with peripheral T cell lymphoma-unspecified (PTCL-U). Eighty-four patients who were diagnosed with PTCL-U between February 1995 and December 2005 were included in the study. Around 70% of the patients presented with stage III-IV disease; 24% were categorized as high or high-intermediate risk according to the International Prognostic Index (IPI) scoring system, and 45% were classified as groups 3 or 4 using the Prognostic Index for PTCL-U (PIT). Extranodal involvement was found in 51 of 85 patients (60%). Most of the initial chemotherapy regimens were anthracycline-based (75%). The overall response rates for patients treated with initial chemotherapy and salvage chemotherapy were 63.1% (52.6% of complete response (CR), 10.5% of partial response (PR)) and 35% (9% of CR, 26%of PR), respectively. The median progression-free survival and overall survival of all patients were 17.1 months (95% CI, 0.0-40.5) and 35.5 months (95% CI, 1.2-69.8), respectively. Poor performance status, the presence of B symptoms, IPI scores >or=3 and PIT class >or=2 were predictive prognostic factors for survival.

[Clinical characteristics and survival of peripheral T-cell lymphoma-not otherwise specified: a report of 57 cases].

OBJECTIVE: To analyze the clinical characteristics and survival data of 57 patients with peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS). METHODS: The medical records of 57 patients with PTCL-NOS classified according to the revised REAL-WHO criteria, treated from Jan 1993 to Dec 2007 at the First and the Third affiliated Hospitals of Medical School of Xi'an Jiaotong University, were retrospectively evaluated by K-M univariate and COX multivariate analysis. RESULTS: 39 of the patients (68.4%) were males and 18 (31.6%) were females, aged 44 (3 - 88). Nine of the 57 patients (15.8%) were treated with chemo-radiotherapy, 43 (75.4%) with chemotherapy, 3 (5.3%) with radiotherapy, and 2 with supported treatment alone (2.5%). The overall response rate was 87.3%, with a complete remission (CR) rate of 60.0% in 55 evaluable cases. The 1-, 3-, and 5-year overall survival (OS) rates were 67.0%, 48.0% and 24.3%, respectively, with a median follow-up of 30.4 months (ranged 1-100 months). The median survival time (MST) was 36.0 months. Multivariate analysis showed that the prognostic index for T cell lymphoma (PIT) score was an independent prognostic factor for PTCL-NOS (P < 0.05), but bone marrow involvement, performance status, extranodal involvement, stage, B symptom were not independent prognostic factors. CONCLUSION: Although conventional chemotherapy yields a high response rate for PTCL-NOS, the long-term survival is still low and further investigation for optional treatment is needed.

[Long-term survival after nasal NK/T cell lymphoma]. / Nazális NK/T-sejtes lymphoma hosszú túlélése.

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein-Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.

Successful treatment of a child with late-onset T-cell post-transplant lymphoproliferative disorder/lymphoma.

We report a novel regimen for refractory post-transplant T-cell lymphoma (PTL). Our patient presented with non-Epstein-Barr virus (EBV) related, T-cell post-transplant lymphoproliferative disease (PTLD) 3.5 years after liver transplantation. Initially diagnosed as polyclonal PTLD, the disease progressed to a monoclonal, T-cell PTL that was refractory to several chemotherapy regimens but responded to a regimen consisting of fludarabine, cyclophosphamide, cytarabine, and alemtuzumab. Consolidation therapy included high-dose chemotherapy, autologous hematopoietic stem cell rescue, and radiation therapy. She remains in remission 2.5 years later. T-cell PTL is a rare disease with a poor prognosis; this regimen provides a novel, potentially curative approach for its treatment.

[Primary non-Hodgkin's lymphoma of the nasal cavity at early stage: long-term treatment outcomes and prognostic analyses of 108 cases].

BACKGROUND & OBJECTIVE: Primary non-Hodgkin's lymphoma (NHL) of the nasal cavity has unique clinicopathologic features, and optimal treatment regimen remains unclear. This study was to summarize the clinical features, treatment outcomes, and prognostic factors of primary NHL of the nasal cavity at early stage. METHODS: Records of 108 patients with primary NHL of the nasal cavity, consecutively treated at Cancer Center, Sun Yat-sen University from Jun. 1990 to Sep. 2004, were reviewed. All diagnoses were confirmed with pathology and immunochemistry. Seven cases were of B-cell phenotype. Survival prognostic factors were analyzed by Kaplan-Meier method and Cox regression model with SPSS12.0 software. RESULTS: Median follow-up time for survived patients was 41 months. The overall complete remission (CR) rate after primary treatment was 67.6%, and CR rates were 80.2% for the patients received radiochemotherapy and 29.6% for the patients received chemotherapy alone. There were evidences indicating systemic relapse in 33 (30.6%) patients. With regard to the control of local, regional, and systemic failure, radiochemotherapy was better than chemotherapy alone. The 5-year overall survival rate was 50.0% for all patients. Both univariate analysis and multivariate analysis showed that pre-treatment history of more than 3 months, primary lesion limited in the nasal cavity, CR after primary treatment, and radiochemotherapy were favorable prognostic factors. CONCLUSIONS: Nasal cavity is frequently involved by peripheral T- and NK-cell lymphomas. Pre-treatment history of disease, extent of primary lesion involvement, and response to the primary treatment may be independent prognostic factors.

[Prolonged remission after immunotherapy of a previously refractory peripheral T-cell non-Hodgkin lymphoma]. / Anhaltende Remission nach Immuntherapie bei zuvor refraktärem peripherem T-Non-Hodgkin-Lymphom.

HISTORY AND ADMISSION FINDINGS: A 54-year-old woman in good condition was admitted because of a rapidly growing tumor in the right groin with skin infiltration. There were no B-symptoms and no increased susceptibility to infections. INVESTIGATIONS: Histology revealed a peripheral T cell non-Hodgkin lymphoma (NHL). Laboratory tests showed borderline elevation of lactate dehydrogenase. Staging computed tomography (CT) revealed widespread moderately enlarged lymph nodes and the main lesion in right groin measuring 5 x 6 cm which was classified as stage IIIAE. TREATMENT AND COURSE: According to the phase-II-trial DSHNHL-2003 - 1 (German study group for high grade NHL) the patient was scheduled for cycles of CHOEP at 14-day intervals plus granulocyte colony stimulating factor. During the first cycle she developed a worsening wound infection and an infection of her port catheter. Because response to treatment was only minimal a biopsy was performed which showed persisting malignant lymphoma. As a result she was not given the 4-weekly addition of alemtuzumab, as provides in the study protocol. Instead she received alemtuzumab subcutaneously for eight weeks at a dose of 3 x 30 mg/week plus prophylactic antibiotics. A good partial response was obtained, followed by radiotherapy with 36 Gy to the main lesion in the right groin. The follow up CT-scans one year after treatment showed a stable remission. CONCLUSION: In T-NHL with primary non-response it is especially difficult to induce a stable remission. Despite a course which was complicated by infections and a failing response to increased chemotherapy a remission was achieved in this patient with a monotherapy of alemtuzumab for eight weeks without complications, followed by radiotherapy.

[Clinical features of 89 patients with primary non-Hodgkin's lymphoma of the tonsil].

BACKGROUND & OBJECTIVE: Head and neck lymphoma develops predominantly in the tonsil. This study was to investigate the clinical features of primary non-Hodgkin's lymphoma (NHL) of the tonsil, and to explore possible ways to improve the prognosis and quality of life of the patients after treatment. METHODS: Clinical data of 89 naive patients with NHL of the tonsil, treated from May 1990 to Jan. 2003, were retrospectively reviewed. All patients were confirmed pathologically and classified according to revised European-American Lymphoid Neoplasms and World Health Organization Classification, and staged according to the Ann Arbor classification. Stage I-II patients received radiochemotherapy-predominant treatment, whereas stage III-IV patients received chemotherapy-predominant treatment. RESULTS: Of the 89 cases, 60 (67%) were diffuse large B-cell subtype, 11 (12%) were peripheral T-cell subtype, 5 (6%) were indolent lymphoma, 1 was anaplastic large T-cell lymphoma, and 1 was T lymphoblastic lymphoma; 81 (91%) were stage I-II disease. Of the 89 patients, 58 (72%) received radiochemotherapy, 19 (21%) received radiotherapy alone, 3 received chemotherapy alone, and 1 received radiochemotherapy combined with rituximab. The 5-year overall survival rate was 80%, that of stage I-II patients was 84%. Cox regression multivariate analysis showed that the survival rate was correlated to the value of international prognostic index (IPI), and whether the patient had primary refractory or relapsed disease, but was not correlated to sex, age, pathologic subtype, B symptoms, and bulky disease. CONCLUSIONS: Most patients with NHL of the tonsil are at early stages, with good prognosis. Diffuse large B-cell lymphoma is the most common pathologic subtype. Primary refractory, relapse, and IPI>1 are independent prognostic factors.

Orbital peripheral T-cell lymphoma in a child.

A 6-year-old boy with known peripheral T-cell lymphoma had progressive left periorbital swelling and CT findings consistent with abscess or necrosis. Biopsy revealed peripheral T-cell lymphoma and associated necrosis involving the lacrimal gland and surrounding orbital tissue. Immunohistochemical and T-cell receptor gene rearrangement studies confirmed the diagnosis. The patient responded to local radiation and systemic chemotherapy. This is the first pathologically confirmed case of orbital peripheral T-cell lymphoma with subpanniculitic features.