Necrotizing fasciitis of the vulva due to carbapenem-resistant Enterobacteriaceae as a complication of acute myeloid leukemia treatment: a case report.

BACKGROUND: Carbapenem-resistant Enterobacteriaceae strains have been reported in healthcare facilities with a rising incidence and are a major concern owing to infections that are often severe and can be potentially fatal, with limited therapeutic options. Klebsiella pneumonia represents the most frequently isolated microorganism. CASE PRESENTATION: We report the case of a Caucasian 52-year old Caucasian woman with acute myeloid leukemia was admitted to the inpatient hematology unit at a university referral hospital in Portugal. This hospital has endemic colonization of Carbapenem-resistant Enterobacteriaceae and contention measures are being implemented to reduce spreading of these multidrug resistant bacteria. After receiving first line chemotherapy according to the intermediate-dose cytarabine regimen, in context of deep medullary aplasia, the patient developed a localized infection of the vulva, which progressed to a necrotizing fasciitis. This is a rare, life-threatening, and fulminant infection. Carbapenem-resistant Klebsiella was isolated in both vulvar exudate and blood cultures. The patient underwent multiple schemes of antimicrobials, but progressed with multiorgan compromise and was admitted to the intensive care unit for a short period for stabilization. Surgical debridement was performed twice with clinical improvement and, after 6 weeks, a skin graft was executed with good response. Reevaluation of the hematologic disease showed a complete response to first cycle of induction therapy. Despite success in resolving this complex infection, decisions regarding antibiotic treatment represented a tremendous challenge for the whole team. The importance of multidisciplinary collaboration was key for the patient's recovery and survival, and therefore, needs to be acknowledged. CONCLUSIONS: This clinical case raises awareness on a clinical entity that can be life threatening and, therefore, requires a high level of suspicion to assure an early integrated approach to avoid complications. Endemic spreading of carbapenem-resistant Enterobacteriaceae is becoming a reality, and health policies need to be urgently undertaken at the national level to decrease morbidity and mortality because of health facilities-related infections.

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.