Erdheim-Chester disease: expanding the spectrum of cutaneous manifestations.

BACKGROUND: Erdheim-Chester disease (ECD) is a rare condition and there is limited information available regarding its cutaneous manifestations. OBJECTIVES: To describe the clinical and histopathological features of cutaneous involvement in ECD. METHODS: This study is a single-centre retrospective analysis of patients 18 years old and older with biopsy-proven diagnosis of ECD between 1 January 1990 and 1 April 2017. Patients from this cohort were screened for cutaneous manifestations, and BRAF c.1799T>A (p.V600E) mutational analysis was conducted in novel skin manifestations. Primary outcomes included cutaneous manifestations (morphology and topography of lesions) and BRAF mutation status in novel cutaneous findings. RESULTS: Of 71 patients with ECD, 15 patients (21%; median age 52 years) presented with cutaneous manifestations. The most common finding was the presence of xanthelasma-like lesions (n = 8). Two patients had nonfacial cutaneous xanthomas. Seven patients presented with nonxanthomatous cutaneous involvement, with the most common finding being subcutaneous nodules (n = 5). A single patient presented with granuloma annulare-like lesions. Another patient with mixed ECD and Langerhans cell histiocytosis presented with lightly scaling, pink-red macules. In three patients, the appearance of skin lesions was the first manifestation of the disease. Most patients presented with bone/extremity pain, weight loss and other constitutional symptoms at the time of diagnosis. The BRAF V600E mutation was not found in patients with panniculitis-like and granuloma annulare-like lesions. CONCLUSIONS: The most common presentation in ECD is the presence of periorbital xanthelasma-like lesions. Other presentations include nonfacial cutaneous xanthomas, panniculitis-like lesions and granuloma annulare-like lesions. Associated symptoms at presentation include bone/extremity pain and weight loss. What's already known about this topic? Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis characterized by lipid-laden macrophage infiltration of tissue and subsequent fibrosis. Cutaneous involvement is found in approximately 25% of patients, with the majority presenting with periorbital xanthelasma-like lesions. What does this study add? We report two novel cutaneous findings: panniculitis-like lesions and granuloma annulare-like lesions. Associated bone/extremity pain and weight loss should raise suspicion for Erdheim-Chester disease.

Presentation, complications, and impact of concurrent malaria infection on anticancer therapy.

BACKGROUND: There is very limited data on the effects of malaria on on-going anticancer therapy. MATERIALS AND METHODS: We performed a retrospective analysis of adult solid tumor patients who contracted malaria while on active anticancer therapy. We noted their demographic profile, clinical course and the effects of malaria infection on their on-going anticancer therapy. Analysis was done with simple percentages. RESULTS: We analyzed 33 malarial episodes in 30 patients over 3 months. Plasmodium vivax was the most common type of infection (75%). Presenting symptoms included the typical triad of fever with chills and rigors. Malaria caused multiple complications, necessitating hospitalization in half of the patients and intensive care unit care in 1 of 8 patients. Common complications included thrombocytopenia (73%), anemia (67%), hyponatremia (66%), hepatic dysfunction (27%), and hypotension (12%). There were no deaths as a result of malaria. Malaria caused treatment delays with an average of 2.42 days per event. Plasmodium vivax caused more complications and therapy delays, average: 3.7 days per event, while non-vivax malaria caused an average of 0.5 days delay per event. There was a high level of resistance to chloroquine. CONCLUSION: Malaria is a significant problem in adult solid tumor patients, leading to multiple complications and therapy delays.

Estrogen, progesterone and HER2 receptor expression in breast tumors of patients, and their usage of HER2-targeted therapy, in a tertiary care centre in India.

BACKGROUND: This study was undertaken to document the pattern of expression of estrogen (ER), progesterone (PR) and human epidermal growth factor receptor-2 (HER2) and the usage of HER2-targeted therapy in a large tertiary care hospital in India in the year 2008. MATERIALS AND METHODS: The histopathology reports of all breast cancer patients registered in the hospital in 2008 were extracted from the electronic medical record system. All the cases were immunohistochemically evaluated for estrogen and progesterone receptor status (ER and PR), and c-erbB-2 protein (HER2) expression using standard immunoperoxidase method. The use of HER2-targeted therapies was evaluated by extracting relevant information from the database of the hospital pharmacy and case charts of patients enrolled in ongoing approved trials. RESULTS: A total of 2001 new patients of invasive breast cancers with available pathology reports were registered in the hospital in the year 2008. ER and/or PR expression was positive in tumors of 1025 (51.2%) patients. HER2 3+ expression by immunohistochemistry (IHC) was found in 335 (16.7%) and HER2 2+ in 163 (8.1%). The triple negative phenotype was found in 596 (29.8%) patients. An estimated 441 patients were eligible to receive HER2-targeted therapy based on their HER2 status. Of these 38 (8.6%) patients received some form of HER2-targeted therapy; 20 patients (4.5%) as part of ongoing clinical trials and 18 (4.1%) as part of routine care. CONCLUSIONS: The overwhelming majority of patients eligible for HER2-targeted therapy in our institution are unable to receive it because of financial constraints and limited access to health insurance. There is a higher fraction of patients with the triple negative phenotype compared to the Western population.

Dietary Lycopene: Its Properties and Anticarcinogenic Effects.

Lycopene is the principal pigment of the carotenoids naturally found in tomatoes and is important not only because of the color it imparts but also because of the recognized health benefits associated with its presence. Red tomatoes typically contain about 95% of their lycopene as the all-trans isomer, the most stable form. In tangerine tomatoes, on the other hand, the lycopene is present as tetra-cis-lycopene, a geometric isomer of all-trans lycopene. Lycopene is a major component found in blood serum. This carotenoid has been extensively studied for its antioxidant and cancer-preventing properties. Prevention of heart disease has been shown to be another antioxidant role played by lycopene because it reduces the accumulation of platelets that eventually lead to blood clots, heart attacks, and strokes. In contrast to many other food phytonutrients whose effects have only been studied in animals, lycopene from tomatoes has been repeatedly studied in humans and found to be protective against several cancers, which now include colorectal, prostate, breast, lung, and pancreatic cancers. This review outlines the background information dealing with lycopene and presents the most comprehensive and current understanding of its potential functional role in human health.

Photosensitization of liposomal membranes by hematoporphyrin derivative.

The putative tumor-localizing and -photosensitizing fraction of hematoporphyrin derivative, the fastest migrating fraction of hematoporphyrin derivative separated by polyacrylamide gel filtration (HPD-A), photosensitized lipid peroxidation and membrane lysis in egg phosphatidylcholine liposomes. The rate of membrane damage was approximately 4-fold faster in oxygen compared to anoxia, with evidence for the involvement of singlet oxygen. The diffusion of HPD-A into small liposomes led to a shift of the Soret band from 363 nm in buffer to 398 nm accompanied by 4-fold enhancement of the fluorescence. The presence of human serum albumin retarded the diffusion of HPD-A into small liposomes, which is attributed to partial complexing of the HPD-A. A different effect of serum albumin was the protection of large liposomes from photosensitized lysis by incorporated HPD-A. This protection is attributed to scavenging of singlet oxygen, as evidenced by oxidation of tryptophan in the protein.