Introduction: Incidence of non-Hodgkin lymphoma (NHL) is 200 times higher in HIV-positive patients than otherwise healthy persons. Plasmablastic lymphoma (PBL) accounts for less than 3% of all NHL in HIV patients and it’s associated with very poor prognosis. Immunodeficiency and HIV viremia are major risk factors for developing cancers, and high adherence to highly active antiretroviral therapy (HAART) has a protective role. Case: We report here a case of 21-year-old HIV positive boy, presented to our Emergency Room for persisting dysphagia, vomit and weight-loss. He had a history of mother-to-child HIV transmission and multiple virological failures due to his poor HAART-adherence. On oral examination a mass in mandibular gingiva and one in hard palate were detected in association with oral candidiasis. Blood test revealed T-CD4+ cells 16/µl and HIV viral load 161.000 copies/µl. An esophagogastroduodenoscopy showed a 70 mm diameter mass on gastric small curvature. Total body CT scan documented also bilateral expansive lesions of maxillary sinus. Biopsies of gingival, palatine and gastric lesions were performed and all showed CD138+, CD20-, Epstein-Barr-virus (EBV)-associated PBL. Osteomedullary biopsy was positive for bone marrow involvement. The patient (classified as IV/B Ann-arbor stage) re-started HAART and initially underwent 2 CHOP regimens without benefits, thus DHAP chemotherapy was started, but also this proved ineffective. The patient received radiotherapy combined to corticosteroids and gemcitabine until his death, that occurred during a hospitalization for febrile neutropenia 6 months after PBL diagnosis. Conclusions NHL is the most common HIV related cancer after Kaposi’s sarcoma. AIDS-related NHLs are predominantly aggressive high-grade B-cell lymphomas, such as PBL. This, like in our case, is characterized by weak/absent expression of conventional B-cell markers (CD20,CD45), by strong expression of plasma cell marker(CD138) and by EBV-relationship. Recognition of this entity by pathologists and clinicians is not easy but mandatory in order to start correct and early treatment. This lymphoma shows a tropism to the oral cavity, but involvement of areas other than head and neck (such as gastrointestinal tract) is rarely described. In literature, the apparent rarity of extra-oral sites and of such an advanced stage, as our patient, may be due to a closer follow up of HIV patients (among whom this lymphoma is more common) in addition to a lack of recognition. To the best of our knowledge, this is the first report of this entity in a vertically transmitted HIV-positive young man. This case highlights the increased risk of NHL in young people with vertical HIV infection, because of persistent immunodeficiency and viremia. Indeed low adherence to HAART, typical in this group of patients, might represent an emerging problem and NHL screening should be offered, in association with intervention to improve adherence, such as HAART simplification, if possible.

PLASMABLASTIC LYMPHOMA OF ORAL CAVITY WITH GASTRIC AND BONE-MARROW INVOLVEMENT IN A YOUNG PATIENT WITH MOTHER-TO-CHILD TRANSMITTED HIV

FABBRI, GABRIELE;CONTINI, Carlo
2014

Abstract

Introduction: Incidence of non-Hodgkin lymphoma (NHL) is 200 times higher in HIV-positive patients than otherwise healthy persons. Plasmablastic lymphoma (PBL) accounts for less than 3% of all NHL in HIV patients and it’s associated with very poor prognosis. Immunodeficiency and HIV viremia are major risk factors for developing cancers, and high adherence to highly active antiretroviral therapy (HAART) has a protective role. Case: We report here a case of 21-year-old HIV positive boy, presented to our Emergency Room for persisting dysphagia, vomit and weight-loss. He had a history of mother-to-child HIV transmission and multiple virological failures due to his poor HAART-adherence. On oral examination a mass in mandibular gingiva and one in hard palate were detected in association with oral candidiasis. Blood test revealed T-CD4+ cells 16/µl and HIV viral load 161.000 copies/µl. An esophagogastroduodenoscopy showed a 70 mm diameter mass on gastric small curvature. Total body CT scan documented also bilateral expansive lesions of maxillary sinus. Biopsies of gingival, palatine and gastric lesions were performed and all showed CD138+, CD20-, Epstein-Barr-virus (EBV)-associated PBL. Osteomedullary biopsy was positive for bone marrow involvement. The patient (classified as IV/B Ann-arbor stage) re-started HAART and initially underwent 2 CHOP regimens without benefits, thus DHAP chemotherapy was started, but also this proved ineffective. The patient received radiotherapy combined to corticosteroids and gemcitabine until his death, that occurred during a hospitalization for febrile neutropenia 6 months after PBL diagnosis. Conclusions NHL is the most common HIV related cancer after Kaposi’s sarcoma. AIDS-related NHLs are predominantly aggressive high-grade B-cell lymphomas, such as PBL. This, like in our case, is characterized by weak/absent expression of conventional B-cell markers (CD20,CD45), by strong expression of plasma cell marker(CD138) and by EBV-relationship. Recognition of this entity by pathologists and clinicians is not easy but mandatory in order to start correct and early treatment. This lymphoma shows a tropism to the oral cavity, but involvement of areas other than head and neck (such as gastrointestinal tract) is rarely described. In literature, the apparent rarity of extra-oral sites and of such an advanced stage, as our patient, may be due to a closer follow up of HIV patients (among whom this lymphoma is more common) in addition to a lack of recognition. To the best of our knowledge, this is the first report of this entity in a vertically transmitted HIV-positive young man. This case highlights the increased risk of NHL in young people with vertical HIV infection, because of persistent immunodeficiency and viremia. Indeed low adherence to HAART, typical in this group of patients, might represent an emerging problem and NHL screening should be offered, in association with intervention to improve adherence, such as HAART simplification, if possible.
non-Hodgkin lymphoma; Plasmablastic lymphoma; highly active antiretroviral therapy (HAART); HIV positive; T-CD4+ cells; AIDS-related NHLs
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11392/1965014
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