Angiocentric T cell lymphoma is a variant of peripheral T cell lymphoma characterized by an angiocentric and angioinvasive infiltrates, invasion of vascular walls, and occulsion of lumen by atypical lymphoid cells with varying degrees of cytologic atypia. We recently experienced a woman with angiocentric T cell lymphorma, who presented with multiple erythematous nodules of skin, subcutaneous mass on right face and peripheral polyneuropathy. At the skin biopy specimen, we could find angiocentric and angioinvasive infiltrate by atypical lymphoid cells which showed a positive immunohistochemical reaction for CD45RO that is one of the T cell markers and a negative reaction for B cell marker. She was treated with two cycles of combination chemotherapy composed of CVP (cyclophosphamide, vincristine, prednisolone), that resulted in complete remission.
Drug Therapy, Combination ; Female ; Humans ; Lymphocytes ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Polyneuropathies* ; Skin ; Vincristine

Angiocentric T-cell lymphoma is a subtype of peripheral T-cell lymphoma and histologically characterized by angiocentric and angioinvasive infiltration of lymphoid cells with varying degrees of cytological atypia, leading to angiodestruction. This disease represents the malignant end in the histological spectrum of angiocentric immunproliferative lesions. This malignancy is commonly a disease with considerable morbidity and is often fatal. However, primary cutaneous angiocentric T-cell lymphoma has a tendency to give a favorable prognosis. Epstein-Barr virus can be identified in involved tissue by immunostaining or by in situ hybridization and it is considered significant in the prognosis. We report a case of cutaneous angiocentric T-cell lymphoma, where the Epstein-Barr virus was identified within neoplastic cells. CVP(cyclophosphamide, vincristine, prednisone) chemotherapy was instigated and an initial complete remission was achieved.
Drug Therapy* ; Herpesvirus 4, Human ; In Situ Hybridization ; Lymphocytes ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Prognosis ; T-Lymphocytes* ; Vincristine

A case of peripheral T-cell lymphoma, large cell type(NIH) is reported. The literature review yielded a rare case report of a malignant lymphoma especially in the even more rare epidural site. The patient's symptoms were relieved by decompressive laminectomy and subtotal removal of mass and combined scheduled chemotherapy with radiotherapy. The pathological examination revealed malignant lymphoma, diffuse, large cell type(NIH) by H & E and immunologic marker analysis.
Biomarkers ; Drug Therapy ; Epidural Space* ; Laminectomy ; Lymphoma ; Lymphoma, T-Cell, Peripheral* ; Radiotherapy

A 72-year-old male presented with respiratory discomfort. A simple chest X-ray and abdominal computed tomography showed pleural effusion and multiple lymph node enlargement. The pleural effusion was determined by thoracentesis to be chylothorax. An inguinal lymph node biopsy showed peripheral T-cell lymphoma. Following three cycles of cyclophospamide, hydroxyl doxorubicin, vincristine, prednisolone (CHOP) chemotherapy, a partial response was observed. Chylothorax is an extremely rare complication of T-cell lymphoma. We present a case of peripheral T-cell lymphoma presenting with chylothorax. We suggest that clinicians should consider chylothorax when examining patients with lymphoma who present with atypical pleural effusion.
Aged ; Biopsy ; Chylothorax* ; Doxorubicin ; Drug Therapy ; Humans ; Lymph Nodes ; Lymphoma ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral* ; Male ; Pleural Effusion ; Prednisolone ; Thoracentesis ; Thorax ; Vincristine

Accurate diagnosis of autoimmune pancreatitis (AIP) is important to clinicians since it is difficult to differentiate AIP from pancreatic malignancies. Furthermore, unlike pancreatic malignancies, AIP has dramatic response to steroids. A 61-years-old man presented with acute pancreatitis. Imaging studies showed two separate pancreatic masses, irregular narrowing of main pancreatic duct, and a renal mass that highly suggested AIP. Endoscopic ultrasound-guided core needle biopsy of the pancreatic masses and ultrasound-guided biopsy of the renal mass revealed peripheral T-cell lymphoma. The patient is currently undergoing chemotherapy. We present a case of pancreatic lymphoma masquerading as AIP with literature review.
Biopsy ; Biopsy, Large-Core Needle ; Diagnosis ; Drug Therapy ; Humans ; Lymphoma* ; Lymphoma, T-Cell, Peripheral ; Pancreatic Ducts ; Pancreatic Neoplasms ; Pancreatitis* ; Steroids

Aminopyridines ; Antineoplastic Combined Chemotherapy Protocols ; Benzamides ; Humans ; Leukemia, Myeloid, Acute/drug therapy* ; Lymphoma, T-Cell, Peripheral/drug therapy*

PURPOSE: To report a case of complete remission of primary orbital peripheral T-cell lymphoma with panniculitis-like features after chemotherapy. CASE SUMMARY: A 57-year-old healthy female presented with periorbital swelling and symptoms of diplopia. The patient was first treated with high-dose systemic corticosteroids, however, symptoms persisted. Therefore, anterior orbitotomy with excisional biopsy was performed for diagnostic purposes. On microscopic examination, the excised mass showed localized dense lymphocyte infiltrates, and cytologic atypia was observed under a high-power field. On immunehistochemical examination, tumor cells were positive for CD3 and CD8 but negative for CD4, CD20 and CD56. Based on histopathological results, primary orbital peripheral T-cell lymphoma with panniculitis-like features was diagnosed. Additionally, molecular pathological testing was positive for Epstein-Barr virus. Subsequently, the patients underwent chemotherapy and complete remission was obtained. CONCLUSIONS: Peripheral T-cell lymphoma often manifests as systemic symptoms, including lymph node enlargement and B symptom. The primary form of the disease in an orbit is very rare, and has a poor prognosis with a high mortality rate because the disease quickly progresses. Herein, the authors report a rare case of a healthy patient without any past medical history who achieved complete remission of a fast-growing primary orbital T-cell lymphoma with no preceding systemic symptoms.
Adrenal Cortex Hormones ; Biopsy ; Diplopia ; Drug Therapy ; Female ; Herpesvirus 4, Human ; Humans ; Lymph Nodes ; Lymphocytes ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral* ; Middle Aged ; Mortality ; Orbit* ; Prognosis

Angiocentric T-cell lymphomas have been described as a distinctive clinicopathologic entity in the spectrum of peripheral T-cell lymphomas, with a prominent invasion of blood vessels by lymphomatous cells. In these conditions, the presence of Epstein-Barr virus (EBV) genomes has been demonstrated, suggesting that EBV might play a major role in their cause. Herein, we report a case of cutaneous angiocentric T cell lymphoma associated with the EBV. The patient was diagnosed with nasal angiocentric T cell lymphoma 5 months ago, and treated with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) combination chemotherapy. After three cycles of CHOP, skin lesions developed. A skin biopsy specimen showed an angiocentric and angioinvasive infiltrate containing some atypical lymphocytes. EBV encoded RNA (EBER) was demonstrated in lesional skin by in situ hybridization.
Biopsy ; Blood Vessels ; Cyclophosphamide ; Doxorubicin ; Drug Therapy, Combination ; Genome ; Herpesvirus 4, Human* ; Humans ; In Situ Hybridization ; Lymphocytes ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Prednisolone ; RNA ; Skin ; Vincristine

A 43-year-old male presented with a painless left testicular mass. The pathologic diagnosis of the radical orchiectomy specimen was peripheral T-cell lymphoma, unspecified (PTCL-u). According to the Ann Arbor staging system, his initial stage was III because of the right nasopharyngeal involvement. After first-line chemotherapy with four courses of the CHOP regimen and this was followed by involved-field radiotherapy, he achieved complete remission. Two months later, disease recurred to the left ciliary body of the left eye without evidence of involvement at other sites. Although the patient received intensive chemotherapy with autologous hema-topoietic stem cell transplantation, he ultimately died of leptomeningeal seeding. Because both the central nervous system (CNS) and the orbit are sanctuary sites for chemotherapy, orbital infiltration of lymphoma should prompt physicians to evaluate involvement of the CNS and to consider performing prophylactic intrathecal chemotherapy as a treatment option.
Adult ; Central Nervous System ; Ciliary Body ; Diagnosis ; Drug Therapy ; Eye Neoplasms ; Humans ; Lymphoma ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Male ; Orbit* ; Orchiectomy ; Radiotherapy ; Recurrence* ; Stem Cell Transplantation ; T-Lymphocytes* ; Testis

BACKGROUND: Peripheral T-cell lymphoma (PTCL) is a rare form of non-Hodgkin's lymphoma (NHL), and it is usually associated with poor outcome. Here, we report our experience in treating this disease over 19 years, with the aim of helping to establish better treatment methods.METHODS: We retrospectively investigated 18 non-anaplastic PTCL cases that were diagnosed at a medical center in Seoul between October 1995 and October 2014. The clinical characteristics, treatments, and outcomes were reviewed.RESULTS: According to the World Health Organization (WHO) classifications for PTCL, 11 patients had PTCL, not otherwise specified (PTCL-NOS), 6 patients had extranodal natural killer/T-cell lymphoma, nasal type (ENKL), and 1 patient had subcutaneous panniculitis-like T-cell lymphoma. Patients were treated with various chemotherapeutic regimens. Of these 18 patients, 5 (27.7%) relapsed and 7 (38.9%) died from disease progression. Two relapsed patients received high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HDCT-ASCT). The 5-year event-free and overall survival rates were 43.2% and 66.7% in all cases, 45.5% and 54.5% in PTCL-NOS, and, 25.0% and 83.3% in ENKL, respectively.CONCLUSION: PTCL-NOS showed a suboptimal outcome. Among 6 ENKL patients, 3 relapsed, but 2 of 3 relapsed patients were salvaged. For better prognosis, HDCT-ASCT in relapsing and refractory PTCL and chemo-radiotherapy in ENKL could be considered as a salvage treatment. Larger studies are needed to confirm the outcome. Furthermore, an effort should be made to develop more efficient initial therapies through collaborative research.
Adolescent ; Child ; Classification ; Disease Progression ; Drug Therapy ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Prognosis ; Retrospective Studies ; Seoul ; Survival Rate ; T-Lymphocytes ; World Health Organization