Clinical Characteristics, Treatment and Evolution of Splenic and Nodal Marginal Zone Lymphomas-Retrospective and Multicentric Analysis of Portuguese Centers.

INTRODUCTION: Treatment of Splenic (SMZL) and Nodal (NMZL) Marginal Zone Lymphoma is not consensual. Histologic transformation (HT) to aggressive lymphoma is a poorly understood event, with an unfavorable outcome. OBJECTIVES: Describe the clinical characteristics, treatment, outcomes and incidence of HT. METHODS: Characteristics of patients with SMZL and NMZL consecutively diagnosed in 8 Portuguese centers were retrospectively reviewed. Endpoints were overall survival (OS), time to first systemic treatment (TTFST), frequency of HT and time to transformation (TTT). RESULTS: This study included 122 SMZL and 68 NMZL, most of them received systemic treatment: 55.4% and 76.5%, respectively. Splenectomy was performed in 58.7% of patients with SMZL. Different treatment protocols were used. OS or TTFST did not differ significantly according to treatments. Given the small sample size, no conclusion can be made concerning the role of Rituximab in the treatment of NMZL and SMZL based in these results. HT was documented in 18 patients, mainly in SMZL, with a cumulative incidence at 5 years of 4.2%. We confirmed that age is a prognostic factor. CONCLUSION: Randomized prospective trials are needed to standardize treatment in MZL. Patients with HT did appear to have shorter OS in comparison with those who did not experience HT (OS 5 years of 68.4% vs. 80.4%), but the number of HT was too small to reach statistical significance.

Cutaneous diffuse large B-cell lymphoma.

Cutaneous diffuse large B-cell lymphoma accounts for ~6% of all cutaneous lymphomas. It is associated with poor prognosis, and solitary lesions are relatively rare. It often requires an aggressive approach with multi-agent chemotherapy and radiotherapy. It is important to recognize these cases in order to offer rapid and appropriate management.

Mucor infection: difficult diagnosis.

Mucormycosis is a rare, highly aggressive and usually fatal infection, which affects immunocompromised patients. This case report describes a patient with acute promyelocytic leukemia who received antifungal therapy for a suspected pulmonary Aspergillus infection. Material from a lobectomy suggested that on histologic grounds the diagnosis had to be changed to mucormycosis. High suspicion of a Mucor infection favors early detection and timely appropriate antifungal therapy, which is crucial for the prognosis of these patients.