Primary cutaneous follicle center lymphoma with aberrant CD8 expression.

We report an unusual case of primary cutaneous follicle center lymphoma (PCFCL) with aberrant expression of the T-cell marker CD8. The patient is a 48-year-old male with no significant past medical history who presented with red indurated plaques on the abdomen. A punch biopsy showed abnormal lymphoid follicles in the dermis with reduced mantle zones and decreased tingible body macrophages. The epidermis was uninvolved. The follicles expressed CD20, PAX-5, and bcl-6 by immunohistochemistry. CD8, however, was strongly positive, highlighting neoplastic cells, which were negative for any additional T-cell markers. TIA and granzyme B were also negative. The patient underwent further staging workup, without evidence of nodal involvement. His course has been indolent thus far. In summary, we present a case of PCFCL with aberrant expression of the T-cell marker CD8, a finding not previously reported in the literature and a potential diagnostic pitfall.

Two Unusual Cases of Discoid Lupus Erythematosus Associated With Xanthomatized Macrophages.

We present 2 patients with chronic discoid lupus erythematosus (LE) associated with xanthomatized macrophages on light microscopic findings. Skin biopsies revealed hyperkeratotic and atrophic epidermis, vacuolar degeneration of the dermal-epidermal junction, thickened basement membrane, follicular plugging, and perivascular and perifollicular lymphohistiocytic infiltrate. Notably, large collections of lipid-laden histiocytes were observed within the subjacent dermis. The patients denied history of intralesional steroid treatment. The patients did not demonstrate any clinical or laboratory signs of hyperlipidemia, cholestasis, and diabetes mellitus and insipidus. Accumulation of lipid-laden foam cells in cutaneous LE is a rare phenomenon that has been reported in discoid LE and lupus panniculitis, each only once in the literature. It has also been described within lesions of various other dermatoses in patients without lipid, hepatic, or endocrine abnormalities. Its mechanism remains unclear, but it has been hypothesized that intracellular lipids released from degenerating cells contribute to lipidization of mononuclear scavengers. Xanthomatous infiltration in cutaneous LE is an unusual feature, and its presence may not necessarily signify an underlying metabolic disorder.

Gait and dementia.

Cognitive decline and neurodegenerative disease have been implicated in gait dysfunction via disturbance of top-down control mechanisms. Gait velocity decreases, variability increases, and ability to multitask while walking is impaired as cognition declines. Changes in gait can be used to predict incident mild cognitive impairment states as well as dementia. Slow gait velocity together with a cognitive complaint, the Motoric Cognitive Risk syndrome, can serve as a clinical biomarker for high risk of neurologic decline. While patients with Alzheimer's disease typically have quantitative gait impairment, those with other forms of dementia often manifest more overt, qualitative changes to walking. A variety of interventions may be useful to improve gait, including physical and cognitive rehabilitation, treatment of specific underlying causes of gait problems, and treatment of the dementia itself. Understanding the relationship between gait and dementia can elucidate pathology and improve patient care.

Cognition and gait in older people.

Cognitive difficulties and gait abnormalities both increase with age. We review normal and pathologic changes in both gait and cognition in older adults. Gait performance in older individuals is linked to specific cognitive changes, in particular in executive function. Structural and functional assays highlight the shared anatomic control of cognitive and gait function, mostly in the prefrontal cortices. Cognitive impairment can be used to predict incident gait difficulties. Changes in gait, especially decreased gait velocity, may be a harbinger of impending cognitive decline. The combination of slow gait and cognitive complaints (the Motoric Cognitive Risk syndrome) is a powerful new clinical tool to identify those at high risk of developing dementia and therefore may be used to target interventions. Evidence is limited, but cognitive training and targeted physical activity may be useful to mitigate or prevent gait and cognitive decline with age.

Effects of metabolic syndrome on language functions in aging.

This study explored effects of the metabolic syndrome (MetS) on language in aging. MetS is a constellation of five vascular and metabolic risk factors associated with the development of chronic diseases and increased risk of mortality, as well as brain and cognitive impairments. We tested 281 English-speaking older adults aged 55-84, free of stroke and dementia. Presence of MetS was based on the harmonized criteria (Alberti et al., 2009). Language performance was assessed by measures of accuracy and reaction time on two tasks of lexical retrieval and two tasks of sentence processing. Regression analyses, adjusted for age, education, gender, diabetes, hypertension, and heart disease, demonstrated that participants with MetS had significantly lower accuracy on measures of lexical retrieval (action naming) and sentence processing (embedded sentences, both subject and object relative clauses). Reaction time was slightly faster on the test of embedded sentences among those with MetS. MetS adversely affects the language performance of older adults, impairing accuracy of both lexical retrieval and sentence processing. This finding reinforces and extends results of earlier research documenting the negative influence of potentially treatable medical conditions (diabetes, hypertension) on language performance in aging. The unanticipated finding that persons with MetS were faster in processing embedded sentences may represent an impairment of timing functions among older individuals with MetS.

Characteristics of emergency department patients who receive a palliative care consultation.

BACKGROUND: A large gap exists between the practice of emergency medicine and palliative care. Although hospice and palliative medicine has recently been recognized as a subspecialty of emergency medicine, few palliative care teams routinely interact with emergency providers, and primary palliative care skills among emergency providers are lacking. OBJECTIVE: To identify the proportion and characteristics of patients who receive a palliative care consultation and arrive via the emergency department (ED). METHODS: A descriptive study of adult ED patients from an urban, academic tertiary care hospital who received a palliative care consultation in January 2005 or January 2009. RESULTS: In January 2005, 100 of the 161 consults (62%) arrived via the ED versus 63 of 124 consults (51%) in January 2009 (p=0.06). Mean days from admission to consultation in January 2005 were six days (standard deviation 11), versus nine days (SD 26) in January 2009 (p=0.35). Three of the 100 consultations (3%) in January 2005 were initiated in the ED, versus 4 of the 64 (6%) in January 2009. CONCLUSIONS: At an urban academic medical center with a well-developed palliative care service, the majority of palliative care consultations were for patients who arrive via the ED. Despite this, only a small minority of consultations originated from emergency providers and consultation was on average initiated days into a patient's hospital stay.

GAB2 amplifications refine molecular classification of melanoma.

PURPOSE: Gain-of-function mutations in BRAF, NRAS, or KIT are associated with distinct melanoma subtypes with KIT mutations and/or copy number changes frequently observed among melanomas arising from sun-protected sites, such as acral skin (palms, soles, and nail bed) and mucous membranes. GAB2 has recently been implicated in melanoma pathogenesis, and increased copy numbers are found in a subset of melanomas. We sought to determine the association of increased copy numbers of GAB2 among melanoma subtypes in the context of genetic alterations in BRAF, NRAS, and KIT. EXPERIMENTAL DESIGN: A total of 85 melanomas arising from sun-protected (n = 23) and sun-exposed sites (n = 62) were analyzed for copy number changes using array-based comparative genomic hybridization and for gain-of-function mutations in BRAF, NRAS, and KIT. RESULTS: GAB2 amplifications were found in 9% of the cases and were associated with melanomas arising from acral and mucosal sites (P = 0.005). Increased copy numbers of the KIT locus were observed in 6% of the cases. The overall mutation frequencies for BRAF and NRAS were 43.5% and 14%, respectively, and were mutually exclusive. Among the acral and mucosal melanomas studied, the genetic alteration frequency was 26% for GAB2, 13% for KIT, 30% for BRAF, and 4% for NRAS. Importantly, the majority of GAB2 amplifications occurred independent from genetic events in BRAF, NRAS, and KIT. CONCLUSIONS: GAB2 amplification is critical for melanomas arising from sun-protected sites. Genetic alterations in GAB2 will help refine the molecular classification of melanomas.

Effects of health status on word finding in aging.

OBJECTIVES: To evaluate effects of health status on word-finding difficulty in aging, adjusting for the known contributors of education, sex, and ethnicity. DESIGN: Cross-sectional. SETTING: Community. PARTICIPANTS: Two hundred eighty-four adults aged 55 to 85 (48.6% female) participating in an ongoing longitudinal study of language in aging. MEASUREMENTS: Medical, neurological, and laboratory evaluations to determine health status and presence or absence of hypertension and diabetes mellitus. Lexical retrieval evaluated with the Boston Naming Test (BNT) and Action Naming Test. RESULTS: Unadjusted regression models showed that presence of diabetes mellitus was not related to naming. Presence of hypertension was associated with significantly lower accuracy on both tasks (P<.02). Adjustment for demographics attenuated the effect of hypertension (P<.08). For the BNT, a variable combining presence, treatment, and control of hypertension was marginally significant (P<.10), with subjects with uncontrolled hypertension being least accurate (91.4%). Previously observed findings regarding the effects of age, education, sex, and ethnicity were confirmed. CONCLUSION: In this sample of older adults, hypertension contributed to the word-finding difficulty of normal aging, but diabetes mellitus did not.

Are all melanomas the same? Spitzoid melanoma is a distinct subtype of melanoma.

BACKGROUND: Although the majority of melanomas demonstrate high rates of mutations in B-RAF or N-RAS that result in constitutive activation of the mitogen-activated protein kinase-signaling pathway, emerging data suggest molecular differences among melanoma subtypes. In this study, the authors evaluated the contribution of B-RAF and N-RAS mutations to the pathogenesis of Spitzoid melanomas. METHODS: In total, 33 Spitzoid melanomas were analyzed for clinical and pathologic characteristics as well as for hot-spot mutations in the B-RAF and N-RAS genes. In the majority of patients (28 of 33 melanomas), the tumors were confined to the skin with no evidence of metastasis (average follow-up, 32.5 mos). There were five metastasizing melanomas (5 of 33 tumors) with regional or systemic spread. RESULTS: Of 33 Spitzoid melanomas, only 1 showed the V600E mutation in the B-RAF gene (1 of 33 tumors; 3%). It was noteworthy that none of the metastatic Spitzoid melanomas (0 of 5 tumors; 0%), of which 2 resulted in fatal outcomes, demonstrated mutations in B-RAF or N-RAS. CONCLUSIONS: In contrast to the majority of cutaneous melanomas, activating hot-spot mutations in B-RAF or N-RAS were not involved in the pathogenesis of Spitzoid melanoma. These data suggested that Spitzoid melanoma is a distinct form of melanoma with unknown genes and/or signaling pathways involved in its development.

Contextual knowledge reduces demands on working memory during reading.

An experiment is reported in which young, middle-aged, and older adults read and recalled ambiguous texts either with or without the topic title that supplied contextual knowledge. Within each of the age groups, the participants were divided into those with high or low working memory (WM) spans, with available WM capacity further manipulated by the presence or absence of an auditory target detection task concurrent with the reading task. Differences in reading efficiency (reading time per proposition recalled) between low WM span and high WM span groups were greater among readers who had access to contextual knowledge relative to those who did not, suggesting that contextual knowledge reduces demands on WM capacity. This position was further supported by the finding that increased age and attentional demands, two factors associated with reduced WM capacity, exaggerated the benefits of contextual knowledge on reading efficiency. The relative strengths of additional potential predictors of reading efficiency (e.g., interest, effort, and memory beliefs), along with knowledge, WM span, and age, are reported. Findings showed that contextual knowledge was the strongest predictor of reading efficiency even after controlling for the effects of all of the other predictors.

Strongyloides appendicitis: unusual etiology in two siblings with chronic abdominal pain.

Appendicitis is one of the most common causes of acute surgical disease in children and young adults. Parasites, however, are one of the uncommon etiologies. An 8-year-old girl and her 7-year-old sister presented with more than 2 months of chronic abdominal pain that became worse over a 1-week period before presentation. The 2 sisters presented 1 month apart. Both had similar symptomatology and physical examination findings. At operation, the surgical findings included an inflamed appendix with a cross section of the parasite Strongyloides. Strongyloides appendicitis has occurred almost exclusively in areas endemic to the parasite. Its environment is more common outside the United States but occasionally is seen in the Southeast region and in institutionalized individuals. The presentation of acute exacerbation of chronic abdominal pain coupled with the pathologic finding of Strongyloides in an acutely inflamed appendix, should alert the clinician of other possible cases. This increased index of suspicion will allow more prompt diagnosis and help avoid the morbidity of delayed operation.

Recurrent eosinophilic mastitis.

Systemic diseases rarely present with manifestations in the breast. Disorders such as sarcoidosis and pyoderma gangrenosum have been described to produce signs and symptoms in the breast. Peripheral eosinophilia is a systemic disease associated with a finite group of conditions including asthma, allergic or atopic disease, collagen vascular disease, and parasitic infection. In addition it has been described in association with several malignancies. A 50-year-old woman with a history of asthma and significant eosinophilia presented with an enlarging breast mass. Complete excision of the mass revealed eosinophilic mastitis. Two years later the patient again presented with a large mass in the same breast. Repeat excision demonstrated the same pathology. The clinical significance of this lesion is important because it presents as an enlarging, painless, dominant mass. Excision is necessary for management and to conclusively rule out malignancy. Its recurrence despite excision to negative margins, however, may indicate that control of the eosinophilia--and possibly the underlying disorder--is important to prevent further recurrence.