e16555 Background: Langerhans cell histiocytosis (LCH) of the female genital tract is an extremely rare disease. We report a 14 year follow up of a patient with LCH successfully treated with lenalidomide in the relapsed setting. Methods: N/A. Results: A 43 year old Ethiopian woman initially presented to another institution with a nodular lesion on her left vulva with biopsy consistent with LCH. Metastatic workup at the time did not reveal evidence of distant disease. The patient was initially treated with radiotherapy to the vulva and 2 years later she was diagnosed with recurrent disease in the vulva and underwent a wide local excision. Six months afterwards she developed a lesion on her right labium majus consistent with disease recurrence. She was treated with radiotherapy again and underwent a wide radical vulvar excision. She recurred only 3 months later and was started on salvage therapy with thalidomide, 3 years after the initial diagnosis. Within 2 months of starting thalidomide therapy, the patient experienced resolution of vulvar lesions and symptoms. She remained symptom free for 8 years while on thalidomide but then presented to our institution with a new central vulvar lesion after being off therapy for 4 months. Vulvar biopsy confirmed the presence of LCH. She was subsequently restarted on thalidomide and achieved symptom control for an additional 18 months before developing worsening pain and neuropathy associated with therapy. Imaging revealed no evidence of metastatic disease. We then started lenalidomide therapy at dose of 10 mg daily for 21 days in a 28 day cycle and subsequently increased the dose to 25 mg daily continuously. She achieved marked improvement in vulvar symptoms within a month of initiation of lenalidomide. She has tolerated therapy well with no neuropathy or cytopenias. Conclusions: Primary vulvar LCH is a rare disease with no standard therapies. Thalidomide has been described in the literature as an effective treatment option. We describe the use of lenalidomide as an alternative and well tolerated therapy in the relapsed setting. Further investigation is required to determine whether lenalidomide is a viable first line therapy for this rare disease.
e18110 Background: Patients with hematological cancers are at a high risk for increasingly resistant and severe infections. The Infectious Diseases Society of America has defined commonly resistant bacteria as ESKAPE (Enterococcus, Staphylococcus aureus, Klebsiella pneumoniae, Acinetobacter, Pseudomonas aeruginosa, Enterobacter/Enterobacteriaceae). We performed a retrospective review of the rate of ESKAPE infections, resistance profile, and outcomes in patients with various hematological malignancies at the Houston Methodist Hospital from 2006 to 2015. Methods: The patient data was obtained from METEOR (Methodist Environment for Translational Enhancement and Outcomes Research), a clinical data warehouse that contains records dating back to January 1, 2006 with over 3 million patients and over 10 million unique patient encounters. We queried for leukemia (AML, CML, ALL, CLL), amyloidosis and myelodysplastic syndrome (MDS) along with hospitalizations due to bacterial infections. Baseline demographics and overall outcomes were also obtained. Results: Out of 6017 patients with Hematological Malignancies, 632 patients were found; 322 had MDS, 225 had AML, 136 had CLL, 49 had ALL, 60 had CML, 97 had amyloidosis and 13 had an unspecified hematological cancer. Of 1091 infectious events, 60.5% were ESKAPE infections. The bacteria most frequently isolated were Enterococcus (24.7%), MRSA (19.8%) and Pseudomonas (18.0%). Patients with MDS (41.5%), AML (20.6%) and CLL (14.4%) were mostly affected. A prevalent resistance to levofloxacin was detected in gram positives and gram negatives (29-54%). Pseudomonas, E. coli, Proteus and K. pneumoniae showed a significant resistance to broad spectrum antibiotics including aztreonam (23-34%), cefepime (7-23%) and imipenem (22%). Proteus had the highest mortality rate (45.2%), followed by Enterococcus (44.2%) and Pseudomonas. (36.7%). Conclusions: Hematological cancers with risk for neutropenia such as MDS and AML were the most affected by ESKAPE. A significant resistance to levofloxacin, a prophylactic antibiotic, was seen. Gram negative pathogens had an increased resistance to broad spectrum antibiotics and higher mortality rates. New strategies for reducing ESKAPE in MDS and AML are required.
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