RESUMEN
Composite lymphoma (CL) involving B-cell lymphoma and T-cell lymphoma is extremely rare. Herein, we report three such cases using immunohistochemistry, flow cytometry, and the next-generation sequencing (NGS) to identify the pathological and molecular characteristics of CL. In the first case, the patient was admitted to hospital for generalized pruritic maculopapular rash over the whole body. An excisional biopsy of the skin lesions showed T-cell lymphoma. At the same time, the staging bone marrow (BM) biopsy revealed a diffuse large B-cell lymphoma (DLBCL). After R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapies, the patient produced a good response with substantial dissipation of the rashes and relief of skin. The other two patients were admitted to hospital due to lymphadenopathy and were diagnosed with DLBCL and follicular lymphoma (FL) after core needle biopsy of lymph nodes, BM biopsy, BM aspiration, and flow cytometry. Following R-CHOP and R-COP (rituximab, cyclophosphamide, vincristine, and prednisone) therapies, they achieved complete remission unconfirmed (CRu) and complete remission (CR). However, one or two years later, they suffered a relapse of lymphadenopathy. The shocking fact was that re-biopsy of lymphadenopathy revealed peripheral T-cell lymphoma (PTCL) and angioimmunoblastic T-cell lymphoma (AITL). NGS findings identified DNA methyltransferase 3a (DNMT3a), isocitrate dehydrogenase 2 (IDH2), Ras homolog gene family, member A (RHOA), splicing factor 3B subunit 1 (SF3B1), and tumor protein p53 (TP53) mutations. After immunochemotherapy, these patients achieved CRu and CR again. Nevertheless, they suffered a second relapse of T-cell lymphoma. Finally, they died due to progression of disease. We found that the occurrence of CL is associated with Epstein-Barr virus infection and DNMT3a, IDH2, and TP53 mutations, and the prognosis of the disease is closely related to the T-cell lymphoma components.
Asunto(s)
Humanos , Rituximab/uso terapéutico , Vincristina/uso terapéutico , Prednisona/uso terapéutico , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Herpesvirus Humano 4 , Recurrencia Local de Neoplasia , Linfoma de Células T/tratamiento farmacológico , Ciclofosfamida/uso terapéutico , Linfoma de Células B Grandes Difuso/patología , Doxorrubicina/uso terapéutico , Linfadenopatía/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéuticoRESUMEN
Objective:To investigate the clinicopathological features, treatment and prognosis of patients with angioimmunoblastic T-cell lymphoma (AILT) complicated with Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL).Methods:The clinical data of 2 cases of AILT with EBV-positive DLBCL in the Fifth Medical Center of PLA General Hospital were retrospectively analyzed, and the literature was reviewed.Results:One case of complex lymphoma (CL) developed from low fever with systemic superficial lymphadenopathy, the right axillary tumor biopsy showed that AILT complicated with EBV-positive DLBCL. After 8 cycles of chemotherapy, the patient reached unconfirmed complete remission, then received maintenance treatment with chidamide and survived until the deadline. Another case of discordant lymphoma began with subcutaneous nodules and then developed progressive enlargement of superficial lymph nodes. The pathological examination of subcutaneous nodule diagnosed as DLBCL, and the pathological examination of right inguinal lymph node diagnosed as AITL. After 7 cycles of chemotherapy, the patient died of hemophagocytic syndrome.Conclusions:AILT with EBV-positive DLBCL is rare. The main clinical symptom is AITL and the immunophenotypic characteristic indicates the presence of T cells and B cells. At the same time, the prognosis is poor, and the treatment strategy mainly depends on the lymphoma type with unfavorable prognosis.
RESUMEN
A 67 year old woman with a 10 year history of rheumatoid arthritis (RA) treated with methotrexate and prednisone, presented with a 2 year history of worsening multiple cutaneous plaques of variable appearance. Two distinct skin lesions were biopsied to reveal a composite cutaneous lymphoma, possibly caused by long term methotrexate therapy. An [18F] fluoro-2-deoxy-D-glucose (¹⁸F-FDG) positron emission tomography/computed tomography (PET/CT) was performed to stage the malignancy, and was later repeated to evaluate response to chemotherapy, which guided subsequent management. We present the PET/CT imaging findings of this very rare iatrogenic (methotrexate induced) immunodeficiency-associated lymphoproliferative disorder.
Asunto(s)
Femenino , Humanos , Artritis Reumatoide , Linfoma Compuesto , Quimioterapia , Electrones , Linfoma , Trastornos Linfoproliferativos , Metotrexato , Tomografía Computarizada por Tomografía de Emisión de Positrones , Prednisona , PielRESUMEN
Within the past few years, an increasing number of reports of Hodgkin's disease following the diagnosis of, and frequently coexisting with, mycosis fungoides have appeared. Previously, Hodgkin's disease found in the lymph nodes of the patient diagnosed as mycosis fungoides was considered as a transformed form of the mycosis fungoides. But, now it has been proven that Hodgkin's disease and mycosis fungoides are histologically and immunohistochemically distinct disease entities. We report a well-documented case of a man who developed Hodgkin's disease and mycosis fungoides simultaneously as a composite lymphoma. Our case emphasizes the importance of considering the diagnosis of another lymphoma in patients with mycosis fungoides who have lymphadenopathy. The cutaneous mycosis fungoides and the Hodgkin's disease should be treated as an independent disease.