Humans ; Lymphoma, T-Cell, Cutaneous/complications* ; Neuroglia ; Pruritus/pathology* ; Skin Neoplasms/complications* ; Spinal Cord/pathology* ; Sympathetic Nervous System

Objective: To investigate the clinicopathological features and prognosis of cytotoxic T-cell lymphoma (CTL). Methods: The clinicopathological data of 134 CTL patients in Beijing Friendship Hospital Affiliated to Capital Medical University, the 989 Hospital of PLA Joint Logistics Support force (formerly the 152 Hospital) and the Fourth Hospital of Hebei Medical University from 2008 to 2020 were retrospectively collected. Immunophenotype, Epstein-Barr virus infection status and T cell receptor (TCR) clonality of tumor cells were assessed, and clinicopathological features and prognosis of patients were analyzed. Results: Among the 134 CTL patients, the male to female ratio was 1.7∶1.0, the median age was 49.5 years (range 3-83 years), and 100 cases (74.6%) were under 60 years old. Forty-six point nine percent of the patients (53/113) had B symptoms. Most of the patients presented with systemic superficial lymphadenopathy. According to the Ann Arbor staging system, 36.8% (39/106) of the patients were in stage Ⅰ-Ⅱ, and 63.2% (67/106) in stage Ⅲ-Ⅳ. The rate of extranodal involvement was 51.6% (66/128). Spleen was involved in 24.2% (31/128) of the cases. Morphology showed diffuse growth of abnormal lymphocytes, infiltrating and destroying normal tissue structure. Immunohistochemical staining showed that tumor cells expressed T cell antigens (CD2, CD3, CD5, and CD7), and 72.0% (77/107) of them had decreased or lost expression of one or more antigens. According to the numbers of CD4 and CD8 expression in tumor cells, 70 cases (52.2%) were grouped into CD8⁺>CD4⁺group. The expression rates of TIA-1 and granzyme B were 99.2% (119/120) and 79.8% (95/119), respectively. CD20 abnormal expression rate was 27.6% (37/134) and CD56 was negative in all cases. The median Ki-67 proliferative index was 45.0% (range 5%-80%). In situ hybridization of small RNA encoded by Epstein-Barr virus was negative. Clonal TCR gene rearrangement analysis was performed on 49 cases and was positive in all cases. Ninety-one patients were followed up for a median of 36 months (range, 1 to 240 months), and 40 of the 91 patients (44.0%) died. The twenty-three patients were in complete remission (including 13 cases with localized single extranodal mass). The 3-year and 5-year overall survival rates were 53.5% and 49.4%, respectively. Univariate analysis showed that B symptom, spleen involvement, extranodal involvement, clinical stage, CD8⁺>CD4⁺phenotype, abnormal expression of CD20 and Ki-67 proliferation index (>60%) were associated with overall survival (P<0.05). The multivariate Cox regression analyses showed that spleen involvement and CD8⁺>CD4⁺ phenotype were independent prognostic factors for overall survival in CTL patients. Conclusions: CTL are more commonly found in adult males under 60 years old, often accompanied by B symptom, with a high proportion of extranodal involvement and more CD8 positive phenotypes. Spleen involvement and CD8⁺>CD4⁺phenotype are independent predictors of CTL overall survival. Some patients with localized extranodal CTL may have a good prognosis.
Epstein-Barr Virus Infections/complications* ; Female ; Herpesvirus 4, Human/genetics* ; Humans ; Lymphoma, T-Cell/pathology* ; Male ; Prognosis ; Retrospective Studies

Objective: To investigate the clinicopathological characteristics and prognosis of mature T/NK cell lymphomas with aberrant CD20 or CD79α expression. Methods: A retrospective analysis of 641 cases of mature T/NK cell lymphoma diagnosed from January 2014 to December 2020 was performed, and 14 cases of CD20-positive and one case of CD79α-positive mature T/NK-cell lymphoma were identified. Histological examination, immunohistochemical characterization, in situ hybridization for Epstein-Barr virus encoded early RNA (EBER), and PCR testing for immunoglobulin and T cell receptor (TCR) gene rearrangements were performed. Clinicopathological characteristics of these lymphomas were analyzed. Results: There were 13 males and 2 females, with a median age of 56 years. There were 8 cases of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), 3 cases of extranodal NK/T-cell lymphoma, nasal type (ENKTCL), 2 cases of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) and 2 cases of angioimmunoblastic T-cell lymphoma (AITL). Twelve cases were stage Ⅲ or Ⅳ lymphomas. The prognosis was overall poor. The histology, immunophenotype and TCR gene rearrangement were not significantly different from the corresponding types of lymphoma. Ki-67 proliferation index was over 70% in all cases. The expression of CD20 or CD79α was weak and heterogeneous. All 15 case of Ig gene rearrangement were polyclonal. Conclusions: Mature T/NK cell lymphoma with abnormal expression of CD20 or CD79α is rare, commonly found in advanced stage, and associated with poor prognosis. The expression of CD20 or CD79α in these cases is weaker than the corresponding mature T/NK cell lymphomas, while its proliferation index is higher. Histomorphology, extensive immunoprofiling and molecular detection are required for accurate diagnosis.
Antigens, CD20 ; Epstein-Barr Virus Infections/complications* ; Female ; Herpesvirus 4, Human/genetics* ; Humans ; Killer Cells, Natural/pathology* ; Lymphoma, T-Cell, Peripheral/pathology* ; Male ; Middle Aged ; Receptors, Antigen, T-Cell ; Retrospective Studies

No abstract available.
Child, Preschool ; Female ; Flow Cytometry ; Humans ; Hydroa Vacciniforme/*diagnosis/pathology ; Immunophenotyping ; Lymphocytosis/complications ; Lymphoma/*diagnosis ; Receptors, Antigen, T-Cell, gamma-delta/genetics/*metabolism ; STAT3 Transcription Factor/genetics/metabolism ; Sequence Analysis, DNA ; Skin/metabolism ; T-Lymphocytes/*metabolism

Enteropathy-associated T-cell lymphoma (EATL) is a rare extranodal T-cell lymphoma arising from the intestine. Two types of EATL have been reported. In contrast to the classic EATL type I, EATL type II occurs sporadically, is unrelated to celiac disease, and comprises 10% to 20% of all EATL cases. A total of five cases of EATL type II were diagnosed at our clinic from January 2009 to September 2012. Four of the five patients were diagnosed with the help of endoscopy. Among the four patients, two of the cases involved both the small and large intestines, whereas in the other two patients, EATL was limited to the small intestine. Common endoscopic findings included innumerable fine granularities (also called mosaic mucosal patterns) and diffuse thickening of the mucosa with a semicircular shallow ulceration in the lesions of the small bowel. In contrast, the endoscopic findings of the colon were nonspecific and could not distinguish EATL type II from other diseases. There are only few published reports regarding the representative endoscopic findings of EATL. Here, we present the clinical and endoscopic findings of four cases of EATL type II diagnosed by endoscopy.
Aged ; Celiac Disease/complications ; *Colonoscopy ; Enteropathy-Associated T-Cell Lymphoma/etiology/*pathology ; Female ; Humans ; Intestinal Mucosa/pathology ; Intestine, Large/pathology ; Intestine, Small/pathology ; Male ; Middle Aged

No abstract available.
Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; B-Cell-Specific Activator Protein/metabolism ; Bone Marrow/metabolism/*pathology ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Endoscopy, Digestive System ; Female ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Genetic Loci ; Humans ; Liver/metabolism/pathology ; Lymphocytes/cytology/immunology ; Lymphoma, Large B-Cell, Diffuse/complications/*diagnosis/drug therapy ; Lymphoma, T-Cell, Peripheral/complications/*diagnosis/drug therapy ; Middle Aged ; Prednisone/therapeutic use ; Receptors, Antigen, T-Cell, gamma-delta/genetics ; Tomography, X-Ray Computed ; Vincristine/therapeutic use

No abstract available.
Aged ; Humans ; Leukemia, Plasma Cell/complications/*diagnosis/pathology ; Leukocytosis ; Lymph Nodes/pathology ; Lymphoma, T-Cell/complications/*diagnosis/pathology ; Male ; Paraproteinemias/complications ; Polymerase Chain Reaction ; Receptors, Antigen, T-Cell, gamma-delta/genetics/metabolism ; Tomography, X-Ray Computed

A 68-year-old Chinese man presented with an eight-month history of pyrexia of unknown origin and chronic sinusitis despite multiple courses of antibiotics. He underwent extensive investigations, including workups for infections, chronic granulomatous diseases and malignancy. Nasal biopsies were performed twice under local anaesthesia, but did not show any evidence of malignancy. Eventually, the patient was diagnosed with natural killer (NK)/T-cell lymphoma, nasal variant, based on histopathological findings from harvested deep tissue obtained via functional endoscopic sinus surgery. This study highlights that, for patients presenting with pyrexia of unknown origin and nasal symptoms, NK/T-cell lymphoma must be considered as a differential diagnosis. Generous amounts of tissue should be harvested under general anaesthesia rather than limited tissue under local anaesthesia, in order to facilitate and ensure a definitive diagnosis.
Aged ; Anti-Bacterial Agents ; chemistry ; Biopsy ; China ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; diagnostic imaging ; pathology ; Male ; Nasal Cavity ; pathology ; Nose Neoplasms ; complications ; diagnosis ; radiotherapy ; Prognosis ; Radiography ; Radiotherapy ; Sinusitis ; complications ; diagnosis ; Treatment Outcome

OBJECTIVETo evaluate the clinicopathological features and prognosis of primary hepatic lymphoma (PHL).

METHODSThirty-five patients with PHL who underwent surgical resection and were confirmed by pathology in our hospital from 1982 to 2012 were re-evaluated for clinicopathological data, including their symptoms, radiological features, recurrence interval, histopathological properties and prognosis.

RESULTSOf the 35 patients, 25 were men (71.4%) and 10 were women (28.6%), with an average age of 52.6 years old (range, 17-79 years). Presented symptoms were epigastric phymatosis, abdominal pain and low-grade fever. In the present study, 21 (60.0%) patients were positive for HBsAg, 1(2.9%) patient was positive for anti-HCV, 3 patients were positive for AFP, 12 patients and 2 patients were complicated by cirrhosis and hepatocellular carcinoma, respectively. Pathologically, 35 PHL were classified into 19 DLBCL (54.3%), 13 T cell-lymphoma (37.1%), and 3 MALT lymphoma (8.6%). Patients with DCBCL showed better postoperative survival than patients with T cell-lymphoma (31.7 ± 3.2) months vs. (22.9 ± 2.2) months (P < 0.05).

CONCLUSIONSHepatitis B virus (HBV) infection may contribute to the pathogenesis of Chinese patients with PHL. Surgical resection followed by comprehensive therapy is the first-line option for PHL. The prognosis of patients with PHL is associated with PHL subtypes.

Adolescent ; Adult ; Aged ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma, Hepatocellular ; pathology ; therapy ; virology ; Chemotherapy, Adjuvant ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Hepatitis B ; complications ; Hepatitis B Surface Antigens ; metabolism ; Hepatitis C Antibodies ; metabolism ; Humans ; Leukocyte Common Antigens ; metabolism ; Liver Cirrhosis ; complications ; Liver Neoplasms ; pathology ; therapy ; virology ; Lymphoma ; pathology ; therapy ; virology ; Lymphoma, B-Cell, Marginal Zone ; pathology ; therapy ; virology ; Lymphoma, Large B-Cell, Diffuse ; pathology ; therapy ; virology ; Lymphoma, T-Cell ; pathology ; therapy ; virology ; Male ; Middle Aged ; Prednisone ; therapeutic use ; Retrospective Studies ; Survival Rate ; Vincristine ; therapeutic use ; Young Adult ; alpha-Fetoproteins ; metabolism

Natural killer T-cell lymphoma (NKTL) is a malignant neoplasm which usually involves the nasal cavity or paranasal sinuses, while an orbit origin is extremely rare. Here we report the clinical, radiological and histopathologic features of a patient with NKTL originating from the orbit. We analyzed the clinical and radiologic records in the whole course of the disease. We also reviewed the morphology and immunohistochemistry of the neoplasm biopsy, including the presence of CD56, CD3 and cytotoxic molecules. This case demonstrated that nasal-type NKTL with a poor prognosis can originate from the orbit.
Humans ; Lymphoma, T-Cell ; etiology ; metabolism ; Male ; Middle Aged ; Natural Killer T-Cells ; metabolism ; pathology ; Orbital Neoplasms ; complications ; metabolism