Familial Melanoma Associated with Li-Fraumeni Syndrome and Atypical Mole Syndrome: Total-body Digital Photography, Dermoscopy and Confocal Microscopy.

Li-Fraumeni syndrome (LFS) is a rare autosomal dominant disorder caused by a mutation in the p53 gene. Melanoma is considered to be a rare, controversial component of LFS. The aim of this study is to describe the utility of systematic screening for melanoma in patients with LFS and atypical mole syndrome. Two 28-year-old identical twin sisters with LFS and atypical moles were monitored by physical examination, total-body digital photography and dermoscopy be-tween 2006 and 2014. A total of 117, predominantly dark-brown, reticular naevi were identified on case 1 and 105 on case 2. Excisions were performed during the evaluation period of 1 in-situ melanoma and 3 basal cell carcinomas in case 1, and 1 in-situ melanoma and 1 early invasive melanoma in case 2. The remaining melanocytic lesions in both patients were stable during follow-up. The 3 melanomas were new atypical lesions detected with total-body photography and dermoscopy. In conclusion, monitoring LFS patients with total-body photography and dermoscopy may be useful to detect early melanoma.

Secondary diffuse large B-cell lymphoma after chemotherapy for acute myeloid leukemia: looking for the unexpected diagnosis.

Development of Epstein-Barr virus (EBV) positive lymphoproliferative disorders in patients with immunosuppression has become more frequently reported. A patient with acute myeloid leukemia was treated to remission, when on follow-up 9 months after his initial diagnosis, he was noted to have a generalized rash and lymphadenopathy. Evaluation of skin and bone marrow biopsies was suggestive of a relapsed leukemia, and treatment was initiated. Fever evaluation revealed a high load of EBV in his blood. A lymph node biopsy and retrospective examination of his skin and bone marrow revealed an EBV-positive diffuse large B-cell lymphoma with no recurrence of acute myeloid leukemia. His chemotherapy-induced immunosuppression likely predisposed him to develop this EBV-positive diffuse large B-cell lymphoma. This case highlights the need to consider a broader differential and immunohistochemical profiling of these neoplasms to avoid misdiagnosing complex oncology patients.

Intravenous immune globulin therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis complicated by hemolysis leading to pigment nephropathy and hemodialysis.

BACKGROUND: Intravenous immune globulin (IVIG) is generally thought to be of relatively low risk for adverse events and some experts consider this to be the best treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis. OBJECTIVE: We evaluated the underlying cause of anemia and renal failure in 2 consecutive patients being treated with IVIG for Stevens-Johnson syndrome/toxic epidermal necrolysis. METHODS: This is a retrospective chart review. RESULTS: We present 2 patients with Stevens-Johnson syndrome/toxic epidermal necrolysis and severe hemolysis requiring blood transfusion who subsequently developed pigment nephropathy necessitating hemodialysis after treatment with IVIG. Both patients had antibodies to their ABO blood type detected in the eluate from their red blood cell membrane. LIMITATIONS: This is a retrospective review with only 2 cases. CONCLUSIONS: We propose that IVIG-associated hemolysis is an adverse reaction that may not be as rare as once thought, presenting as a mild decrease in hemoglobin and hematocrit. Antibodies to blood type A and B are given as part of pooled immune globulin and are considered to be the cause of hemolysis. More severe anemia requiring transfusion is less common, and the breakdown products produced by hemolysis can lead to pigment nephropathy and renal failure. We present methods by which this severe complication can be anticipated and managed more effectively.

Aiming to improve care of older adults: an innovative faculty development workshop.

INTRODUCTION/AIMS: Internists care for older adults and teach geriatrics to trainees, but they often feel ill-prepared for these tasks. The aims of our 1-day Continuing Medical Education workshop were to improve the knowledge and self-perceived competence of general internists in their care of older adults and to increase their geriatrics teaching for learners. SETTING: Two internal medicine training programs encompassing University, Veterans Affairs, and a community-based hospital in Portland, OR, USA. PROGRAM DESCRIPTION: Course faculty identified gaps in assessment of cognition, function, and decisional capacity; managing care transitions; and treatment of behavioral symptoms. To address these gaps, our workshop provided geriatric content discussions followed by small group role plays to apply newly learned content. Forty teaching faculty participated. PROGRAM EVALUATION: Participants completed 13-item multiple-choice pre- and post-workshop geriatric knowledge tests, pre- and post-workshop surveys of self-perceived competence to care for older adults, and completed an open-ended 'commitment to change' prompt after the intervention. Knowledge scores improved following the intervention (61% to 72%, p < .0001), as did self-perceived competence (11 of 14 items significant). Seventy-one percent of participants reported success in meeting their commitment to change goals. DISCUSSION: A 1-day intervention improved teaching faculty knowledge and self-perceived competence to care for older patients and led to self-perceived changes in teaching behaviors.

Uncovering frustrations. A qualitative needs assessment of academic general internists as geriatric care providers and teachers.

BACKGROUND: General internists commonly provide medical care for older adults and geriatric education to trainees, but lack the necessary knowledge and skills to fulfill these tasks. OBJECTIVE: Assess the geriatric training needs of academic general internists in 3 hospital systems in Portland, OR. DESIGN: Ten focus groups and 1 semi-structured interview. Interview transcripts were analyzed using thematic analysis, a well-recognized qualitative technique. PARTICIPANTS: A convenience sample of 22 academic general internists and 8 geriatricians from 3 different teaching hospitals. MEASUREMENTS: We elicited stories of frustration and success in caring for elderly patients and in teaching about their care. We asked geriatricians to recount their experiences as consultants to general internists and to comment on the training of Internists in geriatrics. RESULTS: In addition to deficits in their medical knowledge and skills, our Internists reported frustration with the process of delivering care to older adults. In particular, they felt ill prepared to guide care transitions for patients, use multidisciplinary teams effectively, and were frustrated with health care system issues. Additionally, general internists' approach to medical care, which largely relies on the medical model, is different from that of geriatricians, which focuses more on social and functional issues. CONCLUSIONS: Although our findings may not be broadly representative, improving our general internists' abilities to care for the elderly and to teach learners how to do the same should address deficits in medical knowledge and skills, barriers to the processes of delivering care, and philosophical approaches to care. Prioritizing and quantifying these needs and measuring the effectiveness of curricula to address them are areas for future research.