Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Series and Review.

ABSTRACT: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy that frequently has cutaneous manifestations. The diagnosis can be a challenge because of its heterogenous clinical presentation, ranging from a brown or violaceous solitary nodule or patch to mixed, disseminated lesions. Furthermore, BPDCN tumor cells express immunohistochemical markers in common with acute myeloid leukemia, which can lead to misdiagnosis. Timely diagnosis requires awareness of its cutaneous manifestations and unique histopathology and immunophenotype. We present a case series of patients diagnosed with BPDCN and review the cutaneous and histopathologic characteristics of this uncommon entity.

Relapsing Multiple Myeloma Presenting as Cardiac Tamponade and Obstructive Uropathy.

Cardiac tamponade is a rare manifestation of relapsing extramedullary multiple myeloma and portends poor prognosis. No cases of cardiac tamponade with co-occurring renal obstruction from plasmacytoma have been reported in the literature, making this case a unique presentation of relapsing multiple myeloma. The presence of known malignancy should not limit our differential diagnosis when evaluating patients with signs of cardiac tamponade.

What lies beneath: Cutaneous involvement of mantle cell lymphoma underlying an insect-bite-like reaction.

Mantle cell lymphoma (MCL) is an uncommon subtype of mature B-cell non-Hodgkin lymphoma characterized by specific morphologic, immunophenotypic, and genetic characteristics, namely the t(11;14)(q13;q32) chromosomal translocation with resultant cyclin D1 overexpression. MCL has a generally aggressive course and is often widely disseminated at the time of diagnosis. Skin involvement is exceedingly rare and is seldom the first manifestation of MCL. We present a case of MCL in an 84-year-old man with cutaneous involvement as the first manifestation, discovered incidentally after biopsy of a persistent nodule believed to be an insect bite. This case not only serves to raise awareness of the possibility of MCL presenting in the skin but also to point out that MCL can have lesions with both an insect-bite-like reaction and a deeper dermal MCL infiltrate.

Correlation of ELISA method with three other automated serological tests for the detection of anti-SARS-CoV-2 antibodies.

Public health emergency of SARS-CoV-2 has facilitated diagnostic testing as a related medical countermeasure against COVID-19 outbreak. Numerous serologic antibody tests have become available through an expedited federal emergency use only process. This paper highlights the analytical characteristic of an ELISA based assay by AnshLabs and three random access immunoassay (RAIA) by DiaSorin, Roche, and Abbott that have been approved for emergency use authorization (EUA), at a tertiary academic center in a low disease-prevalence area. The AnshLabs gave higher estimates of sero-prevalence, over the three RAIA methods. For positive results, AnshLabs had 93.3% and 100% agreement with DiaSorin or Abbott and Roche respectively. For negative results, AnshLabs had 74.3% and 78.3% agreement with DiaSorin and Roche or Abbott respectively. All discrepant samples that were positive by AnshLabs and negative by RAIA tested positive by all-in-one step SARS-CoV-2 Total (COV2T) assay performed on the automated Siemens Advia Centaur XPT analyzer. None of these methods, however, are useful in early diagnosis of SARS-CoV-2.

An unusual case of cutaneous Waldenström macroglobulinemia with the MYD88 L265P mutation.

Waldenström macroglobulinemia is a lymphoplasmacytic lymphoma with bone marrow involvement and a monoclonal IgM gammopathy. Infiltration of the skin by neoplastic cells is very rare, and it can be difficult to distinguish from marginal zone lymphoma. The MYD88 L265P mutation is strongly associated with Waldenström macroglobulinemia, and it may be helpful in differentiating the two disorders, although the presence of this mutation is not specific, and other factors must be considered when making the final diagnosis. We present a diagnostically challenging case of cutaneous Waldenström macroglobulinemia in which the MYD88 L265P mutation was identified in the skin but not in the bone marrow, due to a low tumor burden.

A rare case of CD1a-negative Langerhans cell histiocytosis of the central nervous system in a child.

Langerhans cell histiocytosis is a dendritic cell disorder with a wide spectrum of severity and presentations. Histopathology typically demonstrates a proliferation of Langerhans cells and a lymphohistiocytic inflammatory infiltrate with eosinophils. The diagnosis is supported by immunohistochemistry with the cell markers S100, CD1a, CD68, and Langerin [Blood, 126, 2015, 26 and N Engl J Med, 331, 1994, 154].

Perianal Comedones: A Rare Incidental Finding.

Comedones occur when an overproliferation of keratinocytes blocks sebum secretion in a pilosebaceous duct. Comedones have multiple possible etiologies and contributing factors. While comedones are common to acne, they are also seen in occupational exposures and are associated with certain syndromes. We describe a particularly rare case of comedones at the perianus that is not associated with any known exposure or disease and is a rare incidental finding.

Evidence-based behavioral interventions to promote diabetes management in children, adolescents, and families.

As members of multidisciplinary diabetes care teams, psychologists are well-suited to support self-management among youth with Type 1 diabetes (T1D) and Type 2 diabetes (T2D) and their families. Psychological and behavioral interventions can promote adherence to the complex and demanding diabetes care regimen, with the goals of promoting high quality of life, achieving optimal glycemic control, and ultimately preventing disease-related complications. This article reviews well-researched contemporary behavioral interventions to promote optimal diabetes family- and self-management and health outcomes in youth with T1D, in the context of key behavioral theories. The article summarizes the evidence base for established diabetes skills training programs, family interventions, and multisystemic interventions, and introduces emerging evidence for technology and mobile health interventions and health care delivery system interventions. Next steps in behavioral T1D intervention research include tailoring interventions to meet individuals' and families' unique needs and strengths, and systematically evaluating cost-effectiveness to advocate for dissemination of well-developed interventions. Although in its infancy, this article reviews observational and intervention research for youth with T2D and their families and discusses lessons for future research with this population. Interventions for youth with T2D will need to incorporate family members, consider cultural and family issues related to health behaviors, and take into account competing priorities for resources. As psychologists and behavioral scientists, we must advocate for the integration of behavioral health into routine pediatric diabetes care in order to effectively promote meaningful change in the behavioral and medical well-being of youth and families living with T1D and T2D. (PsycINFO Database Record

New approaches to providing individualized diabetes care in the 21st century.

Building from a foundation of rapid innovation, the 21(st) century is poised to offer considerable new approaches to providing modern diabetes care. The focus of this paper is the evolving role of diabetes care providers collaboratively working with patients and families toward the goals of achieving optimal clinical and psychosocial outcomes for individuals living with diabetes. Advances in monitoring, treatment and technology have been complemented by trends toward patient-centered care with expertise from multiple health care disciplines. The evolving clinical care delivery system extends far beyond adjustment of insulin regimens. Effective integration of patient-centered strategies, such as shared-decision making, motivational interviewing techniques, shared medical appointments, and multidisciplinary team collaboration, into a dynamic model of diabetes care delivery holds promise in reaching glycemic targets and improving patients' quality of life.

Motivational interviewing to promote adherence behaviors in pediatric type 1 diabetes.

Suboptimal regimen adherence among youth with type 1 diabetes (T1D) is a common challenge for patients, families, and providers. Motivational interviewing (MI) is a brief communication style designed to elicit intrinsic motivation and strengthen commitment to behavior change goals. As pediatric MI research expands, a critical review of its evidence base and applicability to promote adherence behaviors for youth with T1D is needed. This review introduces the core tenets of MI and clinical applications in T1D, synthesizes the existing MI research in T1D, and discusses the next steps in MI research. Overall, mixed results for MI interventions in T1D reflect variations in research study design and clinical implementation. Targeting adherence rather than glycemic outcomes typically demonstrates greater results, highlighting the promise of MI to facilitate meaningful and enduring improvements in youths' T1D adherence behaviors.

Neurocognitive outcomes in pediatric diabetes: a developmental perspective.

The impact of diabetes on the developing brain is well-accepted. Effects on neurocognitive functioning are moderate but have larger functional implications, especially when considered through a developmental lens. Pathophysiological factors such as severe hypoglycemia and chronic hyperglycemia can alter developmental trajectories in early childhood and perhaps at later periods. In this paper, we selectively review neurocognitive outcomes in pediatric diabetes (largely type 1), integrating recent research from developmental neuroscience and neuroimaging. We examine the effects of diabetes at different stages and place findings within a neurodevelopmental diathesis/stress framework. Early-onset diabetes is associated with specific effects on memory and more global cognitive late-effects, but less is known about cognitive outcomes of diabetes in later childhood and in adolescence, a time of increased neurobehavioral vulnerability that has received relatively limited empirical attention. Studies are also needed to better elucidate risk and protective factors that may moderate neurodevelopmental outcomes in youth with diabetes.

Maternal depressive symptoms and disease care status in youth with type 1 diabetes.

OBJECTIVE: The current study assessed relations among maternal depressive symptoms, poorer youth diabetes adherence, and glycemic control. Specifically, hypothesized mediating links of lowered expectations of parental involvement, less parental monitoring, and more conflict were examined. METHOD: Participants included 225 mothers and their young adolescents, aged 11-14 years (M = 12.73 years, SD = 1.2) diagnosed with T1D. Maternal depressive symptoms and outcome expectancies for maternal involvement were evaluated with self-report questionnaires. Multisource, parent/youth, and multimethod assessment of adherence, parental monitoring, and conflict were evaluated during a baseline assessment from a larger randomized clinical trial. RESULTS: The first hypothesized structural equation model demonstrated a good fit and indicated that more maternal depressive symptoms were directly associated with less parental monitoring and more conflict, which in turn each were associated with poorer adherence and glycemic control. Although higher involvement expectancies were associated with more monitoring and less conflict, they were not associated with other model variables. A second alternative model also fit the data well; poorer youth adherence was associated with more conflict that in turn related to maternal depressive symptoms. CONCLUSIONS: Two models were tested by which maternal depressive symptoms and poorer youth adherence were interrelated via less monitoring and more conflict. Follow-up longitudinal evaluation can best characterize the full extent of these relations.

Sociodemographic effects on biological, disease care, and diabetes knowledge factors in youth with type 1 diabetes.

Sociodemographic risk factors place youth with type 1 diabetes at higher risk for immediate and long-term health complications, yet research has still to disentangle the confounding effects of ethnicity, socioeconomic status (SES), and parental marital status. Group-oriented and variable-oriented analyses were conducted to investigate sociodemographic differences in biological, disease care, and diabetes knowledge factors in youth with type 1 diabetes. The sample included 349 youth, age 9-17 years (79.9% Caucasian, 71.3% lived with two biological parents, M SES = 46.24). Group t-tests confirmed commonly reported ethnic differences in HbA1c and disease care behaviors. However, variable-oriented analyses controlling for confounding sociodemographic influences showed most disease care effects attributed to ethnicity were better explained by SES. Results may inform development of diabetes literacy programs that integrate culturally sensitive lifestyle and language components for families of youth at risk of poor metabolic control.