Hairy Cell Leukemia: Hepatic Affinity Status Post Splenectomy.

Hairy cell leukemia (HCL) is a rare neoplasm of the B-cell lineage that is characterized by an indolent course and infiltration of the spleen, the bone marrow, and the reticuloendothelial system. Splenectomy is considered an effective treatment for peripheral cytopenia in patients with HCL. Hepatic involvement of hairy cells with infiltration of the sinusoidal endothelial cells is rarely reported in the literature and is not well understood. We present the case of an 88-year-old male with a history of traumatic splenectomy who was found to have a relapse of classic hairy cell leukemia within the hepatic portal system.

Thyrotoxic Crisis in the Absence of Risk Factors: A Case Report.

Thyrotoxic crisis is a severe, life-threatening form of thyrotoxicosis characterized by elevated circulating thyroid hormone that can lead to profound complications. Early diagnostic interventions include a thorough physical examination, laboratory assessments of thyroid hormone levels, and the utilization of quantifying assessment tools to grade the severity of the condition. A targeted therapeutic regimen involving a combination of thioamides, beta-blocking agents, and iodide therapies is administered to combat each stage of the physiological process involved in a thyroid storm. The prompt recognition of clinical manifestations and systemic complications of thyrotoxic crisis is of paramount significance to prevent therapeutic delay and reduce patient mortality. Here, we report an atypical case of a new-onset thyrotoxic crisis in a patient without apparent underlying predisposing factors.

Acute Pancreatitis: A Rare Cause of Complement-Mediated Thrombotic Microangiopathy.

Disruption of the complement regulatory system can provoke thrombotic microangiopathy (TMA), leading to clinical manifestations of generalized fatigue from hemolytic anemia, purpura caused by thrombocytopenia, and acute kidney injury from end-organ ischemia. This particular classification of TMA is known as complement-mediated thrombotic microangiopathy (CM-TMA). In CM-TMA, an inciting event such as infection, surgery, vaccination, or pregnancy triggers an inflammatory response resulting in the expression of inherited mutations or the development of autoantibodies against complement regulatory proteins, which leads to microangiopathic hemolytic anemia, thrombocytopenia, and direct damage to renal endothelial cells. The diverse etiologies of CM-TMA make diagnostic and therapeutic decisions a challenging endeavor. We encountered a young male patient who presented with significant lethargy and confusion. The initial diagnosis was consistent with systemic inflammatory response syndrome secondary to acute pancreatitis; however, the hospital course was complicated by subsequent acute renal failure, hemolytic anemia, and thrombocytopenia, likely indicating CM-TMA. The patient was successfully treated with plasma exchange therapy and eculizumab. We suspect that our patient likely developed CM-TMA from an episode of acute pancreatitis. Prompt diagnosis and early intervention are essential to improving morbidity and mortality. This is underscored by the development of monoclonal antibody therapy that directly targets the pathogenic complement proteins, which have been shown to improve renal disease outcomes.

Transfusion-Related Acute Lung Injury in an Alcoholic Hepatic Cirrhosis Patient: A Case Report.

Transfusion-related acute lung injury (TRALI) is one of the leading causes of transfusion-related mortality in the United States. It is a rare, potentially fatal complication of blood product transfusion, often seen in one in 5000 transfusion cases. On average, studies show a reported estimated fatality rate of 5-24% with a mortality rate of 12%. In the US, TRALI has been responsible for 30% of transfusion-related deaths. In this report, we discuss a case of a 51-year female with a past medical history of alcohol dependence and depression who presented complaining of dizziness and lightheadedness for 1 week. Subsequent diagnostic assessment and therapeutic interventions included various imaging studies, serial hematological evaluations, and eventual administration of blood transfusions, intravenous corticosteroids, supplemental oxygenation, and diuresis for clinical management. The occurrence of TRALI is often underreported due to a lack of timely recognition resulting in delayed treatment. Overall, we were able to not only diagnose TRALI in this patient but also effectively comprehend the significance of guiding appropriate management strategies due tohuman leukocyte antigen (HLA) TRALI-mediating antibodies to potentially reduce the overall incidence of such transfusion reactions.

Intrahepatic cholestasis in sickle cell disease: A review of diagnostic criteria, treatments, and case reports.

Objective To delineate the etiology, symptomatology, and treatment of sickle cell intrahepatic cholestasis (SCIC). Sickle cell disease (SCD) is the most frequently inherited hematologic disease, and SCIC is one rare and often fatal complication and comorbid disease. The literature contains only a small number of case reports involving SCIC and hence limited guidance can be obtained. Methods We reviewed the scientific literature to evaluate the science of SCIC to determine if there were consistencies in presentation, evaluation, treatment, and clinical outcomes. Results We reviewed 6 case reports and a limited number of clinical papers on SCIC. We reported consistencies in clinical presentation and treatment outcomes among cases as well as serological and hematological finding. Conclusions While there is some consistency in the symptom presentation of individuals with SCIC, reliable evaluation and clinical procedures were not demonstrated in what we reviewed. Further research is needed to delineate the attributes of this complicated disease that occurs within SCD.

Cannabis-Associated Pneumothorax: A Case Report.

The use of cannabis for therapeutic and recreational purposes has been on the rise in recent years. This has increased the prevalence of cannabis use disorder across various demographic subgroups. A recent medical literature review describes a few cases demonstrating the association of spontaneous pneumothorax and bullous lung disease in cannabis users without concomitant tobacco use. We herein present a case report of a young male with chronic cannabis use who presented with right-sided spontaneous pneumothorax and bilateral apical blebs.

Pilot study of bempegaldesleukin in combination with nivolumab in patients with metastatic sarcoma.

PD-1 blockade (nivolumab) efficacy remains modest for metastatic sarcoma. In this paper, we present an open-label, non-randomized, non-comparative pilot study of bempegaldesleukin, a CD122-preferential interleukin-2 pathway agonist, with nivolumab in refractory sarcoma at Memorial Sloan Kettering/MD Anderson Cancer Centers (NCT03282344). We report on the primary outcome of objective response rate (ORR) and secondary endpoints of toxicity, clinical benefit, progression-free survival, overall survival, and durations of response/treatment. In 84 patients in 9 histotype cohorts, all patients experienced ≥1 adverse event and treatment-related adverse event; 1 death was possibly treatment-related. ORR was highest in angiosarcoma (3/8) and undifferentiated pleomorphic sarcoma (2/10), meeting predefined endpoints. Results of our exploratory investigation of predictive biomarkers show: CD8 + T cell infiltrates and PD-1 expression correlate with improved ORR; upregulation of immune-related pathways correlate with improved efficacy; Hedgehog pathway expression correlate with resistance. Exploration of this combination in selected sarcomas, and of Hedgehog signaling as a predictive biomarker, warrants further study in larger cohorts.

Association of uridine diphosphate-glucuronosyltransferase 2B gene variants with serum glucuronide levels and prostate cancer risk.

AIMS: Uridine diphosphate-glucuronosyltransferase 2B (UGT2B) enzymes conjugate testosterone metabolites to enable their excretion in humans. The functional significance of the UGT2B genetic variants has never been described in humans. We evaluated UGT2B variants in relation to plasma androstane-3α,17ß-diol-glucuronide (AAG) levels and the prostate cancer risk. RESULTS: AAG levels were measured in sera from 150 controls and compared to the polymorphisms of UGT2B17, UGT2B15, and UGT2B7. Genomic DNA from controls (301) and cases (148) was genotyped for the polymorphisms, and odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated using unconditional logistic regression analyses. Having two copies of UGT2B17 was associated with higher AAG levels in controls among Whites (p=0.02), but not Blacks (p=0.82). Logistic regression models adjusting for age and race revealed that homozygosity for the G allele of the UGT2B15(D85Y) polymorphism was directly associated with the prostate cancer risk (OR=2.70, 95% CI=1.28, 5.55). CONCLUSIONS: While the small sample size limits inference, our findings suggest that an association between the UGT2B17 copy number variant (CNV) and serum AAG levels in Whites, but unexpectedly not in Blacks. This novel observation suggests that genetic determinants of AAG levels in Blacks are unrelated to the UGT2B17 CNV. This study replicates the results that show an association of UGT215(D85Y) with an increased prostate cancer risk.

Theater as a tool to educate African Americans about breast cancer.

BACKGROUND: The current study examines the effectiveness of theater in educating African American women about breast cancer. METHODS: Four hundred and forty-eight participants viewed a play entitled Stealing Clouds. Employing a retrospective/pre-post test design and paired sample t tests, researchers assessed the effectiveness of theater in increasing participants' knowledge about breast cancer and likelihood of participating in efforts to address and reduce breast cancer disparities. RESULTS: Results indicated that the play succeeded in increasing knowledge and awareness about breast cancer and intentions to improve health related behaviors. CONCLUSIONS: Findings suggest that theater may be an effective tool in educating African Americans about breast cancer.

Psychosocial factors contribute to resting blood pressure in African Americans.

OBJECTIVES: African Americans as a group have higher blood pressure than individuals of northern European ancestry (non-Hispanic Whites). We investigate whether psychosocial factors explain the resting blood pressure of healthy, community-dwelling African Americans in our study. PARTICIPANTS: A convenience sample of self-reported normotensive African Americans aged 18-65 years who live in the North Carolina Triangle region. DESIGN: The study protocol consisted of three resting blood pressure sessions with assessment of the following psychosocial factors: anger expression, interpersonal support, anxiety, depression, hostility, active coping, and perceived racism. Additional clinical assessments were height, weight, waist girth, fasting glucose, insulin, triglycerides, and cholesterol. RESULTS: Resting systolic blood pressure was positively associated with male sex (P<.001) and positively correlated with age (P<.0001), waist girth (P<.0001), body mass index (P=.023), and a Cook Medley Hostility subscale identified as aggressive responding (P=.031). Mean arterial pressure was positively correlated with age (P<.0001), waist girth (P=.0041), Spielberger Anger Expression subscale anger control (P=.023), and aggressive responding (P=.020). CONCLUSIONS: Anger and hostility are significantly associated with resting blood pressure and may modulate behavioral and traditional (biologic) risk factors that determine cardiovascular physiology.

Drama-based education to motivate participation in substance abuse prevention.

BACKGROUND: The substance abuse prevention goal of the theatre production "TUNNELS" was to provide community education on substance abuse to an audience in Durham, NC and surrounding communities. The education effort intended to increase awareness and understanding of the risk and protective factors associated with alcohol and other drug use, and to promote pro-active behaviors in substance abuse prevention within the adult community. It was hypothesized that community-based education via drama would change attitudes toward alcohol and substance abuse, and increase participation in family and community activities aimed at substance abuse prevention. METHODS: A focus group comprised of educators, substance abuse researchers and local substance abuse counselors developed "life stories" of users of alcohol and other drugs and a local playwright incorporated these and other experiences into a series of six vignettes. The production was publicized throughout the Durham area, and 700 adults attending the play signed a consent form and completed the pre-play survey. The participant pool was restricted to those adults who completed both the time-1 and time-2 surveys and resided within Durham and surrounding communities. Paired comparisons of mean responses were analyzed using a paired sample two-tailed t-test. A telephone survey three months after the play assessed attitudes toward substance abuse as a disease, and whether the respondents had increased their participation in prevention activities including discussions of the play with others. RESULTS: Viewing the play increased the knowledge base of participants regarding substance abuse as a disease, even though the audience demonstrated an appreciation of risk and protective factors prior to attending the performance. In the pre-play survey, participants indicated a strong opinion that parental involvement in teen life was important, and therefore this was not increased as a result of viewing the play. It was found that the drama increased intent to participate in substance abuse prevention activities at home and in the community. Follow-up surveys performed three months after the performance indicated that participants had discussed the play with others and had increased their participation in substance abuse prevention activities, particularly regarding donations of money. CONCLUSION: Drama incorporates a component of emotional response to the informational content, and the combination of emotion and information works together to promote individual intentions to become more involved in family and community prevention activities. This study demonstrates the efficacy of drama as a mechanism to educate and motivate. Support for this mechanism is warranted at the level of state, local community, school district, and faith-based and community organizations.