Remission of an HHV8-related extracavitary primary effusion lymphoma in an HIV-positive patient during antiretroviral treatment containing dolutegravir.

BACKGROUND: Human herpes virus 8 (HHV8) is the causative agent of Kaposi's sarcoma and has been associated with an increasing number of hematologic diseases such as primary effusion lymphoma (PEL) (both classic and extracavitary form), multicentric Castleman disease and the germinotropic lymphoproliferative disorder. PEL is a rare B cell non-Hodgkin lymphoma that primarily affects immunocompromised patients; aggressive chemotherapy and antiretroviral therapy (ART) with protease inhibitors have been used, with poor results. We present a case of extracavitary PEL in an HIV-infected patient, regressed after ART initiation. CASE PRESENTATION: A 42-year-old male was admitted to the emergency room because of several months of malaise, fever and progressive deterioration of the general conditions. On physical examination soft non-painful subcutaneous masses were palpable at retronuchal, retroauricolar and thoracic regions. HIV serology resulted positive: HIV plasma viremia was 782,270 copies/mL, CD4 103 cells/mL. The excision of one of the masses, metabolically active at a positron emission tomography (PET-CT) scan, revealed an HHV8-related extracavitary PEL. HHV8 plasma viremia was 44,826 copies/mL. ART with tenofovir alafenamide/emtricitabine/dolutegravir was started together with ganciclovir for cytomegalovirus chorioretinitis. The progressive disappearance of the masses was seen after 6 weeks of ART, and a PET-CT scan resulted completely negative at 3 months. After 19 months of ART the patient was in remission of PEL, HIV viremia was undetectable (< 20 copies/mL), CD4 count was 766 cells/mL and HHV8 viremia was undetectable. CONCLUSIONS: In this clinical case, the complete regression of PEL has been achieved after the immune recovery, as a consequence of ART introduction, without chemotherapy. It cannot be excluded that ganciclovir, used for the treatment of CMV chorioretinitis, may have contributed to the control of HHV8 replication. Whether to try or not a conservative approach in HIV-infected PEL patients must be carefully evaluated, considering the patient's characteristics and the prognostic factors.

Efficacy and Safety of Cyclophosphamide Low-Dose Pre-Phase Chemotherapy in Diffuse Large B Cell Lymphoma with Gastrointestinal Involvement.

Background: Gastric Diffuse large B-cell lymphoma (DLBCL) is the most common extranodal site of lymphoma's involvement (30%-40% of all extranodal lymphomas and 55%-65% of all gastrointestinal lymphomas). However, gastric localizations are also sometimes found in systemic DLBCL. Gastric complications such as bleeding, perforation, and stenosis under chemotherapy are well documented. Methods: We retrospectively analyzed 15 patients with newly diagnosed DLBCL with gastrointestinal involvement. Endoscopies were performed in these patients before and after treatment. Treatment consisted of cyclophosphamide low-dose pre-phase chemotherapy before conventional-dose chemotherapy. Results: Endoscopy at staging detected ulcers in 12 patients (80%). After low-dose pre-phase chemotherapy, GI ulcers healed in 91.6% of cases (1 ulcer detected). After the whole treatment (Low-dose pre-phase + chemotherapy) 9 patients (60%) achieved complete response, 4 patients (26.6%) partial response, 2 (13,3%) patients presented disease progression. The most frequent adverse event was neutropenia (73.3%); the most frequent non-hematological adverse event was transaminases elevation (20%). Conclusion: Cyclophosphamide low-dose pre-phase chemotherapy resulted in a safe and effective way to prevent adverse events in systemic DLBCL with gastrointestinal involvement.

Aggressive Primary Cutaneous Anaplastic T-Cell Lymphoma Successfully Treated with Autologous Stem Cell Transplant and Brentuximab Vedotin Consolidation: Case Report and Review of the Literature.

Primary cutaneous CD30+ lymphoproliferative disorders include primary cutaneous anaplastic large cell lymphoma (pcALCL) and lymphomatoid papulosis. The prognosis of the disease is usually excellent but, in a minority of cases, it presents with extracutaneous involvement and aggressive behavior. The case we present-relapsed after surgical excision, immunosuppressive therapy, and conventional chemotherapy-is the first one treated with Autologous Stem Cell transplant followed by Brentuximab Vedotin consolidation, a scheme already used for high risk Hodgkin Lymphoma.

Italian Real-Life Experience on the Use of Mogamulizumab in Patients with Cutaneous T-Cell Lymphomas.

Mycosis fungoides and Sèzary syndrome are the most studied subtypes common cutaneous T-cell lymphomas. The current treatment objective is to improve the clinical manifestations of the disease in the affected areas, to relieve symptoms and to halt disease progression. Patients with early-stage mycosis fungoides are usually managed with skin-directed therapies, whereas patients with resistant or advanced-stage mycosis fungoides or Sèzary syndrome often require systemic drugs. Over the last decade, new drugs have been developed, increasing the breadth of treatment options for cutaneous T-cell lymphomas patients. Mogamulizumab is a first-in-class defucosylated humanized IgG1 κ monoclonal antibody, which exerts its anti-tumour action by selectively binding to C-C chemokine receptor 4 and increasing antibody-dependent cellular cytotoxicity activity against malignant T-cells. Several clinical trials showed that mogamulizumab is able to effectively control the cutaneous T-cell lymphomas in each site (skin, blood, lymph nodes and viscera), improving patients' symptoms, function and overall quality of life with a manageable safety profile. In this report, we discuss 12 cases of patients with mycosis fungoides or Sèzary syndrome successfully treated with mogamulizumab in real-life clinical practice in Italy.

A case of metachronous peripheral T-Cell non-Hodgkin lymphoma following chemotherapy for Hodgkin disease successfully treated with brentuximab vedotin.

Occasionally, non-Hodgkin lymphomas (NHL) occur simultaneously or subsequently to Hodgkin disease. We report on a case of a woman with Hodgkin lymphoma treated with ABVD, who developed 4 years later T-cell NHL with both nodal and extranodal involvement. Brentuximab vedotin could be an effective choice in treating metachronous T-cell NHL.

Primary Cutaneous Anaplastic Large Cell Lymphoma of the Oral Cavity Successfully Treated with Brentuximab Vedotin.

Primary cutaneous anaplastic large cell lymphoma is a CD-30 positive lymphoproliferative disorder with good prognosis, usually treated with radiation therapy and surgery. Head, neck, and extremities are the most frequently involved sites. In this paper, we describe an unusual case of oral localization, recurring after skin-involving radiotherapy, successfully treated with sixteen cycles of brentuximab vedotin. This could be a more effective approach with a less detrimental toll for treating these rare disorders.

The neutrophil/lymphocyte ratio ≥3.5 is a prognostic marker in diffuse large B-cell lymphoma: a retrospective analysis from the database of the Italian regional network 'Rete Ematologica del Lazio per i Linfomi' (RELLI).

In solid tumors and lymphomas, the neutrophil/lymphocyte (N/L) ratio at diagnosis has been shown to be a prognostic factor. The aim of our study was to validate the originally reported N/L ratio cut-point of 3.5 in patients with diffuse large B-cell lymphoma (DLBCL) registered in an Italian real-life database. The prognostic role of the N/L ratio at diagnosis on event-free survival (EFS) and overall survival (OS) was assessed in 505 patients with DLBCL. Patients with an N/L ratio <3.5 (n = 249) had a 4-year EFS probability of 76% and OS probability of 86%, significantly higher than the 4 year EFS rate of 48% and OS rate of 64% in patients with N/L ratio ≥3.5 (n = 256, both p<.0001). The N/L ratio was an independent prognostic factor in the multivariate analysis including the IPI score, and could separate patients with a low/intermediate risk IPI (IPI <3).

Recurrent Sweet's syndrome in a patient with multiple myeloma.

We report on a case of Sweet's syndrome associated with multiple myeloma, as harbinger for disease relapse.