Plasmablastic Lymphoma Causing Adult Intussusception After Cardiac Transplantation.

Intussusception in adults is a rare occurrence at approximately 5% and malignancy as the cause comprises half that number. The most common malignancies found are primary adenocarcinoma, metastatic carcinoma, lymphoma, and gastrointestinal stromal tumors. Lymphoma is the second most common. The management of adult intussusception is generally surgical, which is due to the higher likelihood of malignancy being the underlying cause. The patient's history helps to direct management and the most likely underlying diagnosis. This is especially important in patients who are immunosuppressed and with a history of lymphoproliferative disease. Early management and proper surgical intervention allow for the best survival rate. Here we present a case of adult intussusception caused by a rare and aggressive type of non-Hodgkin lymphoma.

Genomic characterization of HIV-associated plasmablastic lymphoma identifies pervasive mutations in the JAK-STAT pathway.

Plasmablastic lymphoma (PBL) is an aggressive B-cell non-Hodgkin lymphoma associated with immunodeficiency in the context of Human Immunodeficiency Virus (HIV) infection or iatrogenic immunosuppression. While a rare disease in general, the incidence is dramatically increased in regions of the world with high HIV prevalence. The molecular pathogenesis of this disease is poorly characterized. Here, we defined the genomic features of PBL in a cohort of 110 patients from South Africa (15 by whole exome sequencing and 95 by deep targeted sequencing). We identified recurrent mutations in genes of the JAK-STAT signaling pathway, including STAT3 (42%), JAK1 (14%) and SOCS1 (10%), leading to its constitutive activation. Moreover, 24% of cases harbored gain-of-function mutations in RAS family members (NRAS and KRAS). Comparative analysis with other B-cell malignancies uncovered PBL-specific somatic mutations and transcriptional programs. We also found recurrent copy number gains encompassing the CD44 gene (37%), which encodes for a cell surface receptor involved in lymphocyte activation and homing, and was found expressed at high levels in all tested cases, independent of genetic alterations. These findings have implications for the understanding of the pathogenesis of this disease and the development of personalized medicine approaches.

Acute kidney injury and bilateral symmetrical enlargement of the kidneys as first presentation of B-cell lymphoblastic lymphoma.

Lymphoblastic lymphoma is an uncommon subtype of lymphoid neoplasm in adults. Acute kidney injury at initial presentation due to lymphoblastic lymphoma infiltration of the kidneys has rarely been described. We report a 19-year-old woman who presented with acute kidney injury due to massive lymphomatous infiltration of the kidneys. The diagnosis of B-cell lymphoblastic lymphoma was established by immunohistochemical study of the biopsied kidney. The patient had an excellent response to the VDCLP protocol (vincristine, daunomycin, cyclophosphamide, asparaginase, and dexamethasone) with sustained remission. We recommend that lymphomatous infiltration be considered in patients presenting with unexplained acute kidney injury and enlarged kidneys.

Plasmablastic lymphoma of the oral cavity, a B cell-derived lymphoma associated with HIV infection: a case series.

OBJECTIVES: Plasmablastic lymphoma (PBL) of the oral cavity is a rare form of non-Hodgkin lymphoma that is most frequently met in human immunodeficiency (HIV) positive patients. Only a few cases have been reported worldwide since 1997. This clinical entity may escape detection due to its unusual immunophenotype and rare occurrence. Our aim is to present two cases with this rare condition that were diagnosed and treated in our department. MATERIALS AND METHODS: We describe two cases of PBLs in HIV-infected patients, who presented with an expanding painless oral lesion and summarize the literature in order to elucidate the nature of this malignancy. RESULTS: The first patient received chemotherapy with additional radiotherapy that led to complete remission of the disease, while the second experienced a relapse 6 months after treatment with chemotherapy, that caused his death after refusal of further treatment. CONCLUSION: Because of the consistent epidemiological association of PBL with immunosuppression, any patient diagnosed with PBL should be tested for HIV. The clinical picture of PBL, including its affinity with HIV-infection, male sex, and its predilection for the oral cavity, may contribute to the differential diagnosis. Any oral mass occurring in an immunosuppressed patient should be referred for biopsy, since the early diagnosis of these tumors leads to better prognosis of the patients.

[Coexistence of plasmablastic lymphoma, Kaposi sarcoma and Castleman disease in a patient with HIV infection]. / Coexistencia de linfoma plasmablástico, sarcoma de Kaposi y enfermedad de Castleman en un paciente con infección por virus de inmunodeficiencia humana.

Plasmablastic lymphoma (PL) is an uncommon B-cell lymphoma that is strongly associated with human immunodeficiency virus (HIV) infection, and displays distinctive affinity for extranodal presentation in the oral cavity. We report the case of a PL involving the stomach in a 36 year-old man HIV+ patient, associated with Kaposi sarcoma (KS) in sections adjacent to lymphoma. He had a positive history of Castleman disease and KS in a lymphoid node biopsy.

Coexistencia de linfoma plasmablástico, sarcoma de Kaposi y enfermedad de Castleman en un paciente con infección por virus de inmunodeficiencia humana / Coexistence of plasmablastic lymphoma, Kaposi sarcoma and Castleman disease in a patient with HIV infection

El linfoma plasmablástico (LP) es un linfoma de células B poco común que está fuertemente asociado con la infección por el virus de inmunodeficiencia humana (VIH), y muestra una afinidad característica de presentación extra-ganglionar en la cavidad oral. Informamos el caso de un LP afectando el estómago en un paciente masculino de 36 años de edad con infección por VIH, asociado con sarcoma de Kaposi (SK) en áreas adyacentes al linfoma. Tenía el antecedente de enfermedad de Castleman y SK en una biopsia de ganglio linfático.

Plasmablastic lymphoma (PL) is an uncommon B-cell lymphoma that is strongly associated with human immunodeficiency virus (HIV) infection, and displays distinctive affinity for extranodal presentation in the oral cavity. We report the case of a PL involving the stomach in a 36 year-old man HIV+ patient, associated with Kaposi sarcoma (KS) in sections adjacent to lymphoma. He had a positive history of Castleman disease and KS in a lymphoid node biopsy.

Oral and extraoral plasmablastic lymphoma: similarities and differences in clinicopathologic characteristics.

Plasmablastic lymphoma (PBL), initially characterized as an aggressive lymphoma arising in the jaw and oral mucosa in HIV-infected patients, was recently reported to occur with extraoral manifestations, heterogeneous histologic findings, and variable association with immunodeficiency states. We reviewed clinical, morphologic, and immunophenotypic features of 13 cases of PBL to determine whether these different subtypes represent distinct morphologic and clinical entities. Two distinct subtypes of PBL were identified and classified as oral and extraoral PBL. The oral PBLs were strongly associated with HIV infection and commonly demonstrated plasmablastic morphologic features without plasmacytic differentiation. Extraoral PBLs tended to occur in patients with underlying non-HIV-related immunosuppression and universally demonstrated plasmacytic differentiation. The patients with oral PBL demonstrated better overall survival compared with patients with extraoral PBL (P = .02). Our findings suggest that PBL with oral and extraoral manifestation represent 2 distinct clinicopathologic entities.

HIV-associated plasmablastic lymphoma: lessons learned from 112 published cases.

Plasmablastic lymphoma (PBL) is a distinct subtype of non-Hodgkin B-cell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus (HIV)-infected individuals. Data regarding patient age and gender, HIV status, initiation of and response to highly active antiretroviral therapy (HAART), tumor extent, pathology, treatment, and outcome were extracted from 112 cases of PBL identified in the literature. The median age at presentation was 38 years with a male predominance of 7:1, and the median CD4+ count was 178 cells/mm(3). PBL presented on average 5 years after diagnosis of HIV. Common primary sites of presentation included the oral cavity, gastrointestinal tract, and lymph nodes. Most cases presented with either stage I or stage IV disease. There was a variable expression of B-cell markers in tumor cells, but plasma cell markers were expressed in all cases. EBV was detected in 74%. Chemotherapy was used to treat 55% patients and was combined with radiotherapy in 21% cases. Complete response was obtained in 66% of treated cases; the majority of these responses were seen after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). The refractory/relapsed disease rate was 54%. Death occurred in 53% of patients, with a median overall survival of 15 months. Sex, CD4+ count, viral load, clinical stage, EBV status, primary site of involvement, and use of CHOP failed to show an association with survival. PBL is an aggressive B-cell lymphoma that presents in both oral and extra-oral sites of chronically HIV-infected immunosuppressed young men.

AIDS-related plasmablastic lymphoma of the oral cavity associated with an IGH/MYC translocation--treatment with autologous stem-cell transplantation in a patient with severe haemophilia-A.

Plasmablastic lymphoma is an AIDS related lymphoma that continues to have a poor prognosis despite significant advances in the management of HIV and lymphoproliferative diseases. In part this has been due to limited insights into the biology of this disease and the molecular mechanisms of oncogenesis. To date molecular abnormalities have not been described in plasmablastic lymphoma, and its aggressive clinical behaviour has been difficult to understand. We describe the first reported cytogenetic abnormality in plasmablastic lymphoma, an IgH/MYC translocation. It is also the first description of autologous stem cell transplantation in a patient with severe haemophilia A.

Linfoma no Hodgkin oral relacionado al sida (LNHORS): Actualización y presentación de un caso clínico / Aids related oral no Hodgkin lymphoma (ARONHL): up today and report of a case

Introducción: Los linfomas No- Hodgkin presentan características propias cuando se asocian a la infección por el Virus de la Inmunodeficiencia Humana. La cavidad bucal es una de las localizaciones a tener en cuenta en pacientes con Sida (LNHORS) Si bien se registran casos en los primeros estadios de la infección, el diagnóstico previo diferencial estomatológico y su seguimiento no se realizan en nuestro medio con la frecuencia que esta enfermedad requiere. En los pacientes VIH positivos, las características generales están relacionadas con la edad de los pacientes, el estado de base inmunológico de la infección, la adherencia a los tratamientos, una historia de más de 35 años de drogadicción intravenosa y el tipo de linfoma. El presente trabajo tiene dos objetivos: 1) Presentar una actualización del tema ya que en los últimos años se han investigado los diferentes tipos y subtipos histológicos, su estratificación y tratamientos en forma intensa y 2) presentar un caso clínico de Linfoma no Hodgkin con localización en la cavidad bucal de evolución no frecuente. Desarrollo: Una paciente de 34 años de edad con una masa tumoral en el hueso maxilar mucosa gingival y paladar duro y blando. La lesión estaba ulcerada en la primera consulta y hacía protrusión a través de la cavidad bucal. Resultados: se realizaron estudios sistémicos, biopsias de las lesiones orales, estudio histológico y marcación inmunohistoquímica,, búsqueda por diagnóstico por imágenes de otras manifestaciones y localizaciones de linfomas. Fueron establecidos tratamientos, seguimiento y evolución. Conclusiones: Un diagnóstico eficiente y temprano de los pacientes con LNHORS por un equipo de salud, puede incrementar las sobrevidas, en un marco, donde pueda ser posible la reconstitución de la función inmunológica y puedan ser aplicados los nuevos regímenes de infusión contínua con quimioterápicos (AU)

Introduction: Non-Hodgkin Lymphoma presents their own characteristics when it is associated with the human immunodeficiency virus syndrome (HIV). Oral cavity is one localization in Aids patients (LNHOES) and its manifestations are registered on the first state of HIV infection. But oral differential diagnoses is not so frequent as this disease needs, in our environment. On HIV patients general characteristics are related to the age of patients, immunological status, treatment compliance, intravenous drug addictions for more of 35 years and lymphoma type. The present work has two objectives: 1) To present this subject in order to actualize epidemiological news, histological types and subtypes classification, illness stratification and advances on therapy., because it has been, in recent years, intensive researches about it. 2) To report a clinic HIV+ case with NHL, with an infrequent evolution. A female patient of 34 years old, with a tumor mass on maxilla bone, gingival mucosa and hart and soft palate. Lesion was ulcerated at the first consultation and hat protrude trough the mouth. Results considered different systemic studies, biopsies of the oral lesions, histological study and immuno histochemical marcation, and searching of other lymphoma´s manifestations and locations by scan´s studies. Treatments, follow up and evolution were assessed Conclusions: An efficient and early diagnostic of ARONHL by a health team work, can increase longer survival for this patients, in an scene, where it can be possible the reconstitution of immunological function and where it could be applied Continous Infusion Chemotherapy new regimens (AU)