Primary intestinal T-cell lymphoma is a rare disease entity, which is approximately 10% to 25% of intestinal lymphomas, and most of the lymphomas occur in the small intestine. We report here a case of a 56-year-old woman who has been suffering from chronic diarrhea and weight loss for 6 months. Abdominal CT scan and small bowel series showed diffuse wall thickening of the small bowel. Gastroscopic examination showed diffuse erythematous lesions on the esophagus and small gastric ulcerations on the antrum of the stomach, and colonoscopic examination also showed multiple punched-out ulcerations and erosions on the entire colon, including the sigmoid colon to the terminal ileum. Diffuse infiltration of CD 3 positive lymphoma cells was found on biopsy. The patient was diagnosed as primary intestinal T-cell lymphoma with diffuse involvement of the entire gastrointestinal tracts from the esophagus to the rectum. Although the patient received systemic combination chemotherapy and achieved partial response initially, the lymphoma relapsed repeatedly.
Case Report ; Esophageal Neoplasms/therapy* ; Esophageal Neoplasms/pathology ; Esophageal Neoplasms/diagnosis ; Female ; Gastrointestinal Neoplasms/therapy* ; Gastrointestinal Neoplasms/pathology ; Gastrointestinal Neoplasms/diagnosis ; Human ; Lymphoma, T-Cell/therapy* ; Lymphoma, T-Cell/pathology ; Lymphoma, T-Cell/diagnosis ; Middle Age

Adolescent ; Female ; Humans ; Lymphoma, Extranodal NK-T-Cell ; pathology ; therapy ; Neoplasm Staging ; Nose ; pathology

A seven months old male infant visited Severance Children's Hospital for evaluation of anterior mediastinal mass. With chest computed tomography (CT) image and biopsy, precursor T-cell lymphoblastic lymphoma was suspected but the ultrasonography guided biopsy specimen was insufficient to confirm the disease. Because there was a life-threatening risk to perform open biopsy to the small infant, we started chemotherapy empirically. The mass decreased, however, the lesion increased again and did not respond to the drugs. Finally we decided to resect the anterior mass with sternostomy and the pathology report finally resulted in thymoma.
Biopsy ; Drug Therapy ; Humans ; Infant ; Male ; Pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Thorax ; Thymoma ; Ultrasonography

OBJECTIVETo investigate the clinical and pathological features and prognostic factors of primary intestinal non-Hodgkin's lymphoma (NHL).

METHODSThirty-Two cases of intestinal NHL were studied clinically and pathologically. All cases were reclassified according to the WHO classification of lymphoma in 2001.

RESULTSTwenty-one cases (65.6%) were diagnosed as B-cell lymphomas, including 15 cases of diffuse large B-cell lymphoma, 2 cases of mantle cell lymphoma, 1 case of follicular lymphoma, 2 cases of MALT lymphoma and 1 case of Burkitt's lymphoma. Ten cases (31.2%) were diagnosed as T cell lymphomas, including 2 enteropathy-associated T cell lymphomas (EATCL) and 8 cases non-EATCL. One case (3.1%) was diagnosed as histiocytic lymphoma. Nine cases were at stage I-II and 23 cases were at stage III-IV. Follow-up information was available in all cases ranging from 4 to 168 months (mean 25.6 months). Fifteen cases died of the disease. The mortality rates were 33% for B cell lymphoma and 80% for T cell neoplasms. Based on Cox multivariate analysis, the clinical stage and histological types of the disease were important prognostic factors (P < 0.05). T cell lymphoma had a relatively poor prognosis.

CONCLUSIONSDiffuse large B cell lymphoma is the most common subtype of primary intestinal lymphomas. Colon is the most common site involved, followed by the small intestine. Early stage of the disease and low-grade lymphoma appear to have a better prognosis. T cell lymphoma has a very poor prognosis.

Adolescent ; Adult ; Aged ; Child ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Intestinal Neoplasms ; pathology ; therapy ; Lymphoma, B-Cell ; pathology ; therapy ; Lymphoma, Non-Hodgkin ; pathology ; therapy ; Lymphoma, T-Cell ; pathology ; therapy ; Male ; Middle Aged ; Neoplasm Staging ; Prognosis ; Survival Rate

Extranodal NK/T-cell lymphoma, nasal type (ENKT), is a distinct clinicopathologic disease, common among East Asia and Latin America population. Clinically, it frequently occurs in middle-aged men. It predominantly occurs in the nasal or paranasal areas and less frequently in the skin. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. The pathogenesis of ENKT remains uncertain. It is thought that Epstein-Barr virus (EBV) may play a role in the development of this entity. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. It has unique characteristics including the expression of cytoplasmic CD3, CD56 and cytotoxic molecules such as TIA-1, and is positive for EBV in situ hybridization. The overall prognosis of this disease is poor because of frequent relapse or resistance to treatment. Although several studies have explored the treatment of ENKL in recent years, the optimal therapy has still not been found. Due to the highly aggressive features of tumors, every endeavor has been made to investigate factors associated with poor outcome. In this review, the recent advances on cause and pathogenesis, clinical manifestations and staging, pathologic characteristics, diagnosis and differential diagnosis, therapy and prognosis of ENKL are summarized.
Diagnosis, Differential ; Humans ; Lymphoma, T-Cell ; diagnosis ; etiology ; pathology ; therapy ; Neoplasm Staging ; Nose Neoplasms ; diagnosis ; etiology ; pathology ; therapy

CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Hematologic Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Leukemia, Myeloid ; pathology ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Skin Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery

OBJECTIVETo investigate the clinical and pathological characteristics of patients with primary Waldeyer's ring lymphomas (PWRL), and to analyze its therapeutic efficacy and prognostic factors.

METHODSA total of 112 patients with PWRL confirmed by pathological and immunohistochemical methods between January 2009 and January 2014 were studied. Clinical data were collected and analyzed retrospectively.

RESULTSPWRL accounted for 3.9% of lymphoma over the same period. Median age of patients with PWRL was 51.5 years old. The affected areas were tonsil, nasopharynx, tongue base and oropharynx, which accounted for 63.4% (71/112), 22.3% (25/112), 5.3% (6/112) and 4.5% (5/112) respectively. The most common pathological types of these four areas were diffused large B-cell lymphoma (DLBCL) and extranodal NK/T cell lymphoma (NKTCL) which accounted for 58% and 15.2%. The overall response rate (CR/CRu = 51.4%; PR = 30.8%) in all patients was 82.2%, the estimated 5-year overall survival (OS) rate were 71.6%. The 5-year OS rate were 94.7% in the group used Rituximab. Meanwhile, chemotherapy combined with radiotherapy could improve the outcome of T-cell PWRL patients and the 5-year OS rate were 88.9%. Age, disease stages, pathological types, IPI scores, LDH level, β2-MG level and the efficacy of initial therapy were prognostic factors with statistical significance. Cox multivariate analysis showed that age of more than 60 years, LDH level, pathological types and the efficacy of the initial therapy were independently associated with OS.

CONCLUSIONPWRL has a relatively good prognosis. The pathological types affect the prognosis directly and guide treatment. Combined modality therapy should be chosen for patients with PWRL. Patients with T-cell PWRL should accept chemotherapy combined with radiotherapy, while rituximab may be better for B-cell PWRL. The efficacy of initial therapy is crucial for the outcome of patients. Age and LDH level are also important prognostic factors.

Combined Modality Therapy ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; pathology ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; pathology ; Middle Aged ; Multivariate Analysis ; Prognosis ; Retrospective Studies ; Rituximab ; Survival Rate

Herein, we report a case of nasal natural killer T-cell lymphoma (NKTL) with intraocular involvement. A 57-year-old woman was referred due to a three-day history of photophobia and diplopia in the left eye. One-month previously, she was diagnosed with nasal NKTL of the right nasal cavity. Ophthalmic examination revealed conjunctival injection and ptosis. The left pupil was fully dilated and non-reactive to light. Ocular motion was restricted on left-upper gaze. Five days later, anterior uveitis developed and persisted despite topical steroid treatment. An orbital magnetic resonance imaging was without specific findings, however, ophthalmoplegia, vitreous opacity, and an iris mass were observed. A diagnostic anterior chamber aspiration was performed. Aqueous humor aspiration revealed 35% morphologically atypical lymphocytes. After an intravitreal triamcinolone injection, radiotherapy and chemotherapy were administered; this resolved the uveitis and iris mass. When refractory uveitis or orbital pseudotumor occurs in patients with nasal NKTL, ocular and orbital involvement of the NKTL should be considered.
Diagnosis, Differential ; Eye Neoplasms/diagnosis/*secondary/therapy ; Fatal Outcome ; Female ; Humans ; Lymphoma, T-Cell/*pathology ; Magnetic Resonance Imaging ; Middle Aged ; Natural Killer T-Cells/*pathology ; Nose Neoplasms/*pathology

Chromosome Aberrations ; Combined Modality Therapy ; Humans ; Killer Cells, Natural ; pathology ; Lymphoma, T-Cell ; etiology ; genetics ; pathology ; therapy ; Nose Neoplasms ; etiology ; genetics ; pathology ; therapy ; Prognosis

OBJECTIVETo study the clinical features, diagnosis and therapy of hydroa vacciniforme-like cutaneous T cell lymphoma.

METHODSThe clinical presentations and the findings of laboratory examinations and skin biopsy of affected tissue in a child with hydroa vacciniforme-like cutaneous T cell lymphoma were retrospectively reviewed.

RESULTSThe child manifested as rash, fever and lymph node intumesce. Rash was pantomorphia, including edematous erythema, vesicles, crusts, necrosis and depressed scar, and it was mild in winter and severe in summer, mainly involving in the face and extremities. Epstein-Barre virus (EBV)-IgM was positive. Histopathological findings revealed focal lymphocyte invasion in subcutaneous panniculus adiposus, mainly surrounding the blood vessels. Immunohistochemistry showed CD3 (+), CD43 (+), CD20 (-), pax-5 (-), TIA (+), CD5 (+), CD8 (+), Granmye (+) and CD4 (-). The clinical symptoms were improved after glucocorticoid treatment in this child.

CONCLUSIONSHydroa vacciniforme-like cutaneous T cell lymphoma has special clinical manifestations. This disorder may be definitely diagnosed by skin biopsy of affected tissue and immunohistochemistry assay. Glucocorticoid treatment is effective. EBV infection may be related to the development of this disorder.

Child, Preschool ; Female ; Humans ; Hydroa Vacciniforme ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; immunology ; pathology ; Skin ; pathology ; Skin Neoplasms ; drug therapy ; immunology ; pathology