Although eosinophilic fasciitis (EF) may precede hematologic malignancy or Hodgkin's disease, association with peripheral T-cell lymphoma (PTCL) is extremely rare. Only four cases of EF preceding or concomitant PTCL have been reported in the world literature. We experienced the first Korean case of EF complicated by the later relapse of peripheral T-cell lymphoma. A 63-year-old Korean male has been followed at our outpatient clinic periodically after treatment for stage IV PTCL. He had been in complete remission for seven and a half years when he developed edema of both lower extremities followed by sclerodermatous skin change in both hands with peripheral eosinophilia. Biopsy from the left hand showed fibrous thickening of the fascia with lymphoplasmacytic and eosinophilic infiltrate, consistent with EF. Twenty-five months later, a newly developed lymph node from the left neck showed recurrence of PTCL. EF may occur as a paraneoplastic syndrome associated with the relapse of PTCL. Therefore, in a patient with EF, the possibility of coexisting and/or future occurrence of hematologic neoplasm should be considered.
Case Report ; Eosinophilia/pathology ; Eosinophilia/complications* ; Fasciitis/pathology ; Fasciitis/complications* ; Human ; Lymphoma, T-Cell, Peripheral/pathology ; Lymphoma, T-Cell, Peripheral/complications* ; Male ; Middle Age ; Recurrence

Epstein-Barr Virus Infections/complications* ; Herpesvirus 4, Human ; Hodgkin Disease/etiology* ; Humans ; Lymphoma, T-Cell, Peripheral

Hypereosinophilic syndrome (HES) is a clinical disorder characterized by persistent eosinophilia and systemic involvement, in which a specific causative factor for the eosinophilia cannot be verified during a certain period of time. There have been only a few reported cases of this syndrome associated with malignant lymphoma. We report a case of peripheral T-cell lymphoma-unspecified with hypereosinophilic syndrome. The patient was a 42-year-old woman with an uncontrolled fever and a sore throat. Eosinophilia was observed on the peripheral blood smear. We confirmed the diagnosis by bone marrow and liver biopsies: A bone marrow aspiration demonstrated markedly increased eosinophils (24.8%), and a liver biopsy demonstrated infiltration by scattered eosinophils and atypical lymphoid cells, which were confirmed to be T-cell lymphoma cells. This case was a distinctive presentation of peripheral T-cell lymphoma with hypereosinophilic syndrome, probably due to a paraneoplastic condition.
Recurrence ; Pleural Effusion/*etiology ; Lymphoma, T-Cell, Peripheral/complications/*diagnosis ; Liver Neoplasms/complications/*diagnosis ; Hypereosinophilic Syndrome/*etiology ; Humans ; Female ; Fatal Outcome ; Adult

To explore the clinical and pathological characteristics of hepatosplenic gammadelta T-cell lymphoma and its relationship with Epstein-Barr virus infection, the clinical features of a 9-year-old girl with hepatosplenic gammadelta T-cell lymphoma were investigated, the smears of bone marrow was stained with Wright' s stain, biopsies of bone marrow and liver specimen were embedded in plastic and sliced about 4 microm in thickness and routinely stained with HE staining, the immunohistochemical staining was used to mark the tumor cells, and EBER probes were used to detect Epstein-Barr virus RNA. The results showed that the girl presented with prolonged fever, anemia, thrombocytopenia, hepatosplenomegaly, chronic active Epstein-Barr virus infection, and elevated levels of serum ferritin and lactate dehydrogenase. Bone marrow aspirate revealed the infiltration of atypical lymphocytes in the bone marrow stroma. The liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids, which was positive for the T-cell associated marker CD3 and activated cytotoxicity-associated marker granzyme B. In-situ hybridization analysis with EBER probes revealed that the above-mentioned characteristics were negative in neoplastic cells. It is concluded that hepatosplenic gammadelta T-cell lymphoma is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Hepatic and/or splenic and/or bone marrow biopsy with combined phenotype is beneficial to diagnosis. Epstein-Barr virus infection is late event involving an already transformed gammadelta T-cell clone.
Child ; Epstein-Barr Virus Infections ; complications ; Female ; Humans ; Liver Neoplasms ; diagnosis ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; diagnosis ; pathology ; virology ; Receptors, Antigen, T-Cell, gamma-delta ; analysis ; Splenic Neoplasms ; diagnosis ; pathology ; virology

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

OBJECTIVETo explore the clinical efficacy for treatment of patients with peripheral T cell lymphoma (PTCL) by HyperCVAD and CHOPCHOP-like protocols.

METHODSA total of 97 patients with peripheral T cell lymphoma were divided into observation group (50 cases) and control group (47 cases). The patients in observation group were treated by HyperCVAD, and the patients in the control group were treated by CHOP/CHOP-like protocol. The clinical efficacy, accumulate survival rate and side effect of the 2 groups were compared.

RESULTSThe remission rate in the observation group were higher than that in control group (P < 0.05); The PFS rate (1 year) in observation group was higher than that in control group (P < 0.05); the PFS rate (2 years, 3 years) was not significantly different (P > 0.05). The OS rate (1 year, 2 years, 3 years) did not show difference (P > 0.05); the number of patients with neutropenia in observation group was higher than that in control group (P < 0.05); the levels of CD4(+) CD45RA(+), CD4(+) CD45RO(+) in observation group were lower than those in control group (P < 0.05); the levels of CD4(+) and CD4(+)/CD8(+) in observation group was lower than those in control group (P < 0.05); the level of CD8(+) in observation group was higher than that in control group (P < 0.05); the incidence of pneumonia, cardiotoxicity, severe anemia, and thrombopenia were not significantly different (P > 0.05).

CONCLUSIONHypeCVAD protocol shows good clinical effects on the patients with peripheral T cell lymphoma, displaying a high remission rate and PFS rate (1 year), but it has a high incidence of neutropenia, thus it needs more attention in clinic.

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Dexamethasone ; therapeutic use ; Doxorubicin ; therapeutic use ; Humans ; Lymphoma, T-Cell, Peripheral ; drug therapy ; Neutropenia ; complications ; Prednisone ; therapeutic use ; Remission Induction ; Survival Rate ; Treatment Outcome ; Vincristine ; therapeutic use

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