Successful second allogeneic stem-cell transplantation from the same sibling donor for a patient with recurrent hepatosplenic gamma-delta (γ/δ) T-cell lymphoma: A case report.

RATIONALE: Hepatosplenic T-cell lymphoma (HSTCL) is a rare but aggressive type of peripheral T-cell lymphoma (PTCL). There is an urgent need for effective treatment due to the poor prognosis of HSTCL. Here, for the 1st time we describe the rare successful case of HSTCL who relapsed after a previous allogeneic stem-cell transplantation (allo-SCT), achieved remission with the second allo-SCT from the same donor. PATIENT CONCERNS: A 24-year-old male, presented with a 2-week history of fever, drenching night sweats and nonquantified weight loss. DIAGNOSES: Laboratory studies, flow cytometry of immunophenotyped, and physical examination results strongly suggested hepatosplenic γ/δ T-cell lymphoma, stage IVB. INTERVENTIONS: We proceeded to an allo-SCT with a human leukocyte antigen (HLA) identical sibling donor. The bone marrow examination and fluorescent in situ hybridization were observed for complete donor chimerism of bone marrow cells on day 34. On day 157 after the initial allo-SCT, the bone marrow examination revealed the relapse of the sinusoidal infiltration with lymphoma cells. Considering the disease persistence, we conducted the second allo-SCT from the same HLA-identical sibling donor immediately. OUTCOMES: Bone marrow examination indicated hematologic recovery without residual lymphoma cells. LESSONS: Our encouraging outcome suggests that the latter allo-SCT needs to be considered early for patients with disease recurrence, and it also demonstrates that graft-vs-lymphoma conferred by allo-SCT may play an essential role on HSTCL treatment. Furthermore, detecting related genes at diagnosis may have prognostic implications and guidance value for personal chemotherapy program.

[Cutaneous and systemic T-cell lymphoma treated with haploidentical bone marrow transplantation]. / Lymphome T cutané et systémique traité avec succès par greffe haplo-identique.

BACKGROUND: Herein, we report a case of systemic cutaneous T-cell lymphoma refractory to standard therapy, the course of which resulted in haplo-identical bone marrow grafting. PATIENTS AND METHODS: A 53-year-old woman consulted for facial erythema with infiltration, keratotic lesions on the trunk, and adenopathies measuring around 1cm on the axilla and inguinal folds. A diagnosis was made of Sézary syndrome (SS), a leukaemic form of epidermotropic cutaneous T-cell lymphoma. After three years of treatment with methotrexate, the patient developed transformed SS with visceral involvement. Given the high risk of relapse and the absence of an HLA-compatible donor, haploidentical bone marrow grafting was performed. The patient was still in complete remission two and a half years later. The disease course was nevertheless marked by the emergence one year after grafting of a Blaschko-distributed lichenoid eruption having histological features consistent with chronic graft-versus-host disease (GVHD); treatment with topical betamethasone proved efficacious. DISCUSSION: To our knowledge, this is the first reported case of haploidentical grafting for systemic and transformed cutaneous T-cell lymphoma. This approach could henceforth represent a therapeutic option for patients requiring an allograft in the absence of compatible donors. The Blaschko-distributed lichenoid lesions attributed to chronic GVHD could be the result of reduced immune tolerance to abnormal embryological clones leading to a T-lymphocyte-mediated inflammatory reaction.

Peripheral T-Cell Lymphoma: A Posttransplant Lymphoproliferative Disorder Presenting as a Jejunal Mass in a Renal Transplant Recipient.

Posttransplant lymphoproliferative disorders are a spectrum of lymphoproliferative disorders seen in recipients of solid-organ, bone marrow, and stem cell allografts. They include polyclonal early lesions mimicking infectious mononucleosis and monoclonal proliferations of B and T cells, indistinguishable from lymphomas occurring in immunocompetent individuals. Although most posttransplant lymphoproliferative disorders are B-cell neoplasms, T-cell posttransplant lymphoproliferative disorders are very rare. Among solid-organ transplants, renal allografts have low risk for development of posttransplant lymphoproliferative disorders. We describe the case of an adult male who developed a T-cell posttransplant lympho?roliferative disorder involving the small intestine after renal transplant, which was diagnosed as peripheral T-cell lymphoma, not otherwise specified.

[Lymphoma misinterpreted as osteomyelitis]. / Lymfom fejltolket som osteomyelitis

Patients are admitted based on a tentative diagnosis. If that is incorrect it may have negative consequences for the patient and the system. In this case we present an eight-month elucidation with several diagnostic procedures with a tentative diagnosis of osteomyelitis. This diagnosis was kept despite contradicting diagnostic results. Finally, the patient was diagnosed with peripheral T-cell lymphoma. Many cognitive diagnostic pitfalls are known to delay diagnosis. We recommend having them in mind and seeking quick interdisciplinary help when treatment is not successful.

Bone Marrow Transplantation for Peripheral T-Cell Non-Hodgkins' Lymphoma in First Remission.

Opinion statement: Peripheral T-cell lymphomas (PTCLs) are rare and heterogeneous diseases that carry, with the exception of anaplastic lymphoma kinase-positive (ALK+) anaplastic large cell lymphoma, a poor prognosis when treated with conventional chemotherapy. Historically, PTCL was treated like aggressive B-cell lymphomas, and to date cyclophosphamide, prednisone, vincristine, and doxorubicin (CHOP) remains the most commonly used regimen, despite disappointing results. Given the poor outcomes of PTCL patients, a number of studies have investigated the role of high-dose chemotherapy and autologous stem cell transplantation (HDT/ASCT) in the upfront setting, with different results. However, there are no prospective randomized trials, and the clinical benefit appears to be restricted to patients who achieve an objective response after induction chemotherapy. Nevertheless, with the exception of low-risk ALK+ anaplastic large cell lymphoma, in light of the available data, HDT/ASCT for consolidation should be recommended for patients deemed eligible. The results of phase II trials showed that allogeneic stem cell transplantation can cure some relapsed/refractory patients, and few studies have evaluated this strategy in the frontline setting. With the availability of recently approved new drugs as well as new targeted agents under investigation, a number of ongoing studies are testing novel combinations aiming to improve rate and durability of responses to induction chemotherapy.

Primary natural killer/T cell lymphoma of the cervix: case report and clinicopathological analysis.

OBJECTIVE: To present pathological and molecular characterizations of a rare case that was diagnosed as nasal-type natural killer (NK)/T cell lymphoma primarily arising in the cervix. CASE REPORT: An Asian woman was admitted to hospital with a hysteromyoma, and laparotomy was performed. A large tumor of the uterus was found, which was limited to the cervix. Pathological examination showed NK/T cell lymphoma, which was supported by histological and immunohistochemical studies and was confirmed by evidence of Epstein-Barr virus infection. Less commonly, this case concerned a cytotoxic T cell phenotype, as molecular studies showed evidence of a clonal T cell receptor γ chain gene rearrangement. Microscopically, prominent and extensive necrosis was the distinctive feature of this case, which reminded us of considering it as a tumor. CONCLUSION: Primary NK/T lymphoma of the cervix is rare. Our experience in this case provided variable information on both pathological and molecular studies. This case may be of value in the differential diagnosis of lymphoid lesions and other small cell tumors of the cervix.

Atypical presentation of isolated peripheral T-cell lymphoma in the hand: case report.

Peripheral T-cell lymphoma is a rare malignancy that characteristically demonstrates generalized lymphadenopathy and extranodal involvement. We describe an atypical case of peripheral T-cell lymphoma manifesting as isolated hand swelling without evidence of nodal disease or systemic symptoms. This report emphasizes the highly variable clinical presentation of T-cell lymphomas and the importance of expeditious incisional biopsy in the setting of an undiagnosed hand mass.

Autologous stem cell transplantation as the first-line treatment for peripheral T cell lymphoma: results of a comprehensive meta-analysis.

OBJECTIVE: In view of the poor prognosis of most peripheral T cell lymphoma (PTCL) subtypes treated with conventional chemotherapy such as CHOP/CHOP-like regimens, high-dose chemotherapy followed by autologous stem cell transplantation (HDT/ASCT) seems a reasonable option in eligible patients. Nevertheless, owing to the small size of the study and the heterogeneity of most published series, a consensus on the role of ASCT as the first-line consolidation therapy for high-risk PTCL patients has not been reached so far. STUDY DESIGN: We searched MEDLINE, EMBASE, EBSCO, Web of Science, clinicaltrials.gov and the Cochrane Library. Hazard ratio (HR) and 95% confidence intervals (CIs) were calculated by a fixed/random effect model. RESULTS: Twenty-one studies were eligible. Although no statistical significance was observed in these studies, there was a trend toward survival advantage for the HDT/ASCT group as compared to the historical control group (HR 0.81, 95% CI 0.31-2.13). Statistical differences were confirmed in terms of overall survival (OS) between complete remission (CR) and non-CR patients (HR 3.17, 95% CI 0.92-5.42), patients with good and poor risk according to the International Prognostic Index (IPI; HR 0.36, 95% CI 0.22-0.60, I(2) 49%) and Prognostic Index for PTCL (PIT; HR 0.31, 95% CI 0.17-0.58; HR 0.31, 95% CI 0.18-0.54). CONCLUSION: The clear and convincing proof of the effects of up-front HDT/ASCT still depends on sufficient large PTCL-restricted randomized trials in the future. Patients who failed to attain CR before transplant exhibited a worse prognosis; patients with good risk of IPI or PIT had a substantially better OS after ASCT.

Peripheral T-cell lymphoma: the role of hematopoietic stem cell transplantation.

Peripheral T-cell lymphoma (PTCL) is a rare and heterogeneous group of non-Hodgkin lymphomas (NHLs). Whereas the incidence of the disease appears to increase during last decades and the prognosis remains dramatically poor, so far no standard treatment has been established. High-dose chemotherapy and autologous stem cell transplantation (HDT-ASCT) has been proven effective in relapsed PTCL, while retrospective studies have shown a survival benefit as first-line treatment in some subsets of PTCL patients. However, given disease rarity, there is a paucity of randomized trials in both upfront and relapse setting. Here, we critically evaluated eligible prospective and retrospective studies that address the role of ASCT in treatment of PTCL, with respect to quality of design and performance. Additionally, the role of allogeneic transplantation has been reviewed. The comparison of ASCT with novel agents that emerge or the combination of both, are to be ascertained via prospective randomized trials in this field.

Post cardiac transplantation T-cell lymphoproliferative disorder presenting as a solitary lung nodule.

Post-transplantation lymphoproliferative disorder (PTLD) is an infrequent, but serious complication of solid organ and bone marrow transplantations. The vast majority of the cases are of B-cell origin and usually associated with Epstein-Barr virus (EBV) infection. The non-B (T and NK cell) PTLDs account for up to 14% of the PTLD cases in Western countries. We report a case of a 66-year-old man who received an orthotopic heart transplant for cardiomyopathy 7 years prior to presentation. He was referred to our institution with a hypermetabolic solitary right lower lobe lung nodule with an SUV of 9.2 on PET scan. The combined histomorphological and immunohistochemical pattern was most consistent with monomorphic PTLD, peripheral T-cell lymphoma with angioimmunoblastic features. Molecular studies showed clonal T-cell gamma receptor gene rearrangement. Primary pulmonary involvement of T-cell PTLD is extremely rare. This is the third reported case of T-cell PTLD after cardiac transplantation, primarily involving the lung. Further, studies will be required to determine the appropriate treatment and prognosis of this rare entity.

Current state of art for transplantation paradigms in peripheral T-cell lymphomas.

Peripheral T-cell lymphomas (PTCLs) are a rare and aggressive lymphoproliferative disorder characterized, with few exception, by poor prognosis. A correct diagnosis is still hampered by the heterogeneity and rarity of disease. Historically, PTCLs were treated like aggressive B-cell lymphoma with anthracycline-based combination chemotherapy with disappointing results. Neither dose intensification nor dose escalation of chemotherapy has been successful in prolonging the survival of PTCL patients. Due to this discouraging scenario, new therapeutic strategies have been tested. A therapeutic program including hematopoietic stem-cell transplantation (SCT) may represent an interesting strategy for high-risk patients. Although no randomized trials are available, autologous SCT may offer a chance of cure in chemosensitive disease. Nonmyeloablative allogeneic SCT has shown a curative potential with limited toxicity, and it is actively being investigated. We will review the role of the current therapeutic approach to PTCL focusing on the most recent advances in SCT.

Strategies for relapsed peripheral T-cell lymphoma: the tail that wags the curve.

A 69-year-old woman was referred for further evaluation and management of relapsed angioimmunoblastic T-cell lymphoma. At diagnosis, she received six cycles of dose-adjusted EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) and achieved a complete response (CR). Her first surveillance computed tomography scan 3 months later demonstrated enlarging cervical lymphadenopathy. A lymph node excision confirmed relapsed angioimmunoblastic T-cell lymphoma with atypical lymphocytes expressing CD3, CD4, CD10, PD-1, and EBER, with loss of CD5 (Fig 1). A clonal T-cell receptor beta and gamma rearrangement by polymerase chain reaction was identical to that in her initial diagnostic biopsy. At our initial consultation, options for standard as well as investigational therapies were discussed, and HLA typing was initiated. The patient was enrolled onto an investigational phase II study; however, she developed progressive disease after two cycles. She was then treated with romidepsin 14 mg/m(2) administered intravenously for 3 consecutive weeks with 1 week off. After two cycles, she achieved a partial response, and after four additional cycles, she maintained her response without further improvement. We discussed additional treatment options.

Autologous stem cell transplantation as consolidation therapy for patients with peripheral T cell lymphoma in first remission: long-term outcome and risk factors analysis.

This report is a retrospective analysis of 65 patients with peripheral T cell lymphoma (PTCL), who underwent high-dose therapy and autologous hematopoietic stem cell transplantation (autoHCT) as a consolidation of first response achieved with either induction or salvage chemotherapy. We intended to determine the prognostic factors that influenced outcome after autoHCT and to define the predictive value of the scoring systems most often applied for transplant outcomes. Nineteen patients in either complete or partial remission underwent autoHCT after induction chemotherapy. Forty-six patients received second-line chemotherapy as a consolidation of partial response after induction chemotherapy (n = 34) or as a salvage therapy after primary induction failure (n = 12), and thereafter proceeded to autoHCT. Finally, the 36 patients were in complete remission, and 29 in partial remission at autoHCT. The median follow-up of survivors was 53 months (range 7-157 months). The 5-year overall survival and progression-free survival for all patients were 61.5% (95% CI 47.0-74.2%) and 59.4% (95% CI 46.1-71.5%), respectively. In multivariate analysis, bone marrow involvement at diagnosis and less than partial remission after induction chemotherapy were factors independently predictive for overall survival and progression-free survival. The prognostic index for PTCL could reliably stratify the prognosis of PTCL in this analysis.

DexaBEAM versus ICE salvage regimen prior to autologous transplantation for relapsed or refractory aggressive peripheral T cell lymphoma: a retrospective evaluation of parallel patient cohorts of one center.

High-dose chemotherapy (HDT) followed by autologous stem cell transplantation (ASCT) is considered standard in the treatment of patients with relapsed or refractory aggressive peripheral T cell lymphoma (PTCL). However, the optimal salvage regimen before ASCT has not yet been established. We retrospectively analyzed 31 patients with relapsed or refractory aggressive PTCL after anthracycline-based first-line chemotherapy who received either DexaBEAM (dexamethasone, carmustine, etoposide, cytarabine, and melphalan; n = 16) or ICE (ifosfamide, carboplatin, and etoposide; n = 15) regimen as first salvage chemotherapy followed by HDT/ASCT. The overall response rate (OR) was significantly higher for patients treated with DexaBEAM (69 %; 95 % confidence interval 46.0-91.5 %) as compared to the ICE group (20 %; 95 % confidence interval -0.2-40.2 %; P = 0.01), with higher complete response (CR; 38 %; 95 % confidence interval 13.8-61.2 %; vs. 7 %; 95 % confidence interval -6.0-19.6 %) as well as partial response (PR; 31 vs. 13 %) rate. Changing regimen due to failure of first salvage therapy, 12 patients initially receiving ICE still achieved an OR of 58 % (33 % CR, 25 % PR) with DexaBEAM as second salvage therapy, whereas in three patients receiving ICE after DexaBEAM failure, only one achieved an OR (1 PR). Median progression-free survival was significantly higher in the DexaBEAM group (6.4 vs. 2 months; P = 0.01). Major adverse event in both groups was myelosuppression with higher but tolerable treatment-related toxicity for patients in the DexaBEAM group. For all patients proceeding to HDT/ASCT, a 3-year overall survival was 50 %. Together, considering the limitations of the retrospective design of the evaluation and the small sample size, our data suggest that DexaBEAM salvage chemotherapy is superior to ICE for patients with relapsed or refractory aggressive PTCL for remission induction prior to autologous transplantation, with higher but manageable treatment-related toxicity.

Peripheral T-cell lymphoma presenting as testicular mass; a diagnostic challenge.

BACKGROUND: T-cell lymphomas involve the testis infrequently, which deserve special attention because of the poor prognosis and the need to make an appropriate diagnosis, which could lead to a better therapeutic strategy. CASE PRESENTATION: A 40-year-old man presented with right testicular swelling for past three months. The swelling was painless, hard and rubbery. Testicular ultrasound showed diffuse increase in size of the testicle, with alteration in its echogenicity. The patient underwent orchidectomy, and based on histopathological and immunohistochemical tests, a peripheral T-cell lymphoma, not otherwise specified was diagnosed. CONCLUSION: Testicular peripheral T-cell lymphomas are rare and aggressive cancers, with clinical differentials of seminoma and non-neoplastic conditions.