A Rare Case of Severe Manifestation of COVID-19 Infection Presenting as Immune-Related Thrombotic Thrombocytopenic Purpura With Multiorgan Involvement Treated With Plasmapheresis, Steroids, Rituximab, and Caplacizumab.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) caused by decreased activity of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Platelet-rich thrombi in small vessels lead to fragmentation of RBCs causing microangiopathic hemolytic anemia (MAHA). Therapeutic plasma exchange is life-saving and is the mainstay of the treatment of TTP. Higher dose IV steroids along with rituximab are used as an adjunct to plasma exchange. Our case report describes a 26-year-old healthy male who presented with new onset seizures and encephalopathy. Blood work demonstrated anemia, severe thrombocytopenia, elevated lactate dehydrogenase, decreased haptoglobin, and elevated creatinine, and peripheral blood smear showed marked schistocytosis indicating MAHA. Plasma exchange and high-dose steroids were started on a presumptive diagnosis of TTP. ADAMTS13 activity was undetectable and ADAMTS13 inhibitor levels were elevated. Rituximab and caplacizumab were then added. Symptoms of encephalopathy improved by day five and platelet counts started improving by day nine. After several days of plasma exchange, he showed a "clinical response" with several weeks of active treatment. The association between coronavirus disease 2019 (COVID-19) infection and the severity of TTP with multiorgan failure is not well understood yet. Although we describe a successful multimodal approach to the management of TTP, which we believe is secondary to COVID-19 infection, further research is warranted to analyze and understand the pathophysiology by which COVID-19 infection causes TTP. It would help in establishing standardized therapy in the future.

Can Discord Domain-Containing Receptor 2 Mutation Act as a Disease Modifier for PRKAR1A Associated Melanotic Schwannoma?

Melanotic Schwannomas are rare neural sheath tumors with distinctive findings of both Schwann cells and melanocytic cells. Recognition of this entity has prompted the importance of distinction from similar tumor types such as melanomas. Early diagnosis facilitates removal of the mass with less risk of local invasion and metastasis. Although previously known as mostly benign lesions, malignant conversion and recurrence are recognized. This paper presents a patient with melanotic schwannoma, describes the distinctive features that will separate it from melanoma, and addresses the possibility of further guided therapy through next-generation sequencing.

Biomarkers associated with breast cancer are associated with obesity.

BACKGROUND: Obesity is linked to the development of postmenopausal breast cancer, and some studies indicate obesity predicts a worse prognosis for premenopausal women who develop the disease. It was our hypothesis that proteins associated with breast cancer would be associated with body mass index (BMI). METHODS: We searched our database of women enrolled in breast health translational research trials for information on BMI and markers predictive of breast cancer (basic fibroblast growth factor (bFGF), prostate-specific antigen (PSA), human kallikrein (hK)2, and urinary plasminogen activator (uPA). Information on BMI and one or more nipple aspirate fluid (NAF) or serum biomarkers was available from 382 women. RESULTS: In this data set, NAF and serum levels of PSA (nPSA and sPSA), and NAF levels hK2, bFGF and uPA were each associated with pre- and/or postmenopausal breast cancer. sPSA was inversely associated with BMI in both pre- (r=-.56, p=.001) and postmenopausal women (r=-.62, p=.0035) without breast cancer. This association was lost when controlling for plasma volume. In women without breast cancer, NAF bFGF (p=.07, premenopausal subjects) and NAF hK2 (p=.09, postmenopausal subjects) were borderline associated with BMI. In women with breast cancer, nPSA was inversely (r=-.53, p=.049) associated with BMI in premenopausal women and directly associated with BMI in postmenopausal women (r=.37, p=.017). nPSA trended higher in hormone sensitive cancers, especially those that expressed progesterone receptor (p=.059). CONCLUSIONS: sPSA was inversely associated with BMI in all pre- and postmenopausal women and specifically in pre- and postmenopausal women without breast cancer. NAF PSA was associated with BMI in pre- and postmenopausal women with breast cancer. Evaluating the change in PSA with changes in weight may provide clues regarding a subject's breast cancer risk.

Differences in treatment and in outcomes between idiopathic and secondary forms of organizing pneumonia.

Organizing pneumonia is a major reparative response of the lung tissue to an acute injury and is a pathological hallmark of an entity called bronchiolitis obliterans organizing pneumonia (BOOP). It can be idiopathic and called cryptogenic organizing pneumonia (COP) or be secondary to various conditions such as infections, drugs, connective tissue disorders, and radiation. Fifty-seven patients with pathologically confirmed BOOP were identified and were classified as having either COP or secondary BOOP on the basis of whether there was an identifiable cause. The two groups were compared for demographic, clinical, laboratory, radiological and treatment variables. Duration of treatment with corticosteroids was longer for patients with COP.

Improving death certificate completion: a trial of two training interventions.

The death certificate is an important medical document that impacts mortality statistics and health care policy. Resident physician accuracy in completing death certificates is poor. We assessed the impact of two educational interventions on the quality of death certificate completion by resident physicians. Two-hundred and nineteen internal medicine residents were asked to complete a cause of death statement using a sample case of in-hospital death. Participants were randomized into one of two educational interventions: either an interactive workshop (group I) or provided with printed instruction material (group II). A total of 200 residents completed the study, with 100 in each group. At baseline, competency in death certificate completion was poor. Only 19% of residents achieved an optimal test score. Sixty percent erroneously identified a cardiac cause of death. The death certificate score improved significantly in both group I (14+/-6 vs 24+/-5, p<0.001) and group II (14+/-5 vs 19+/-5, p<0.001) postintervention from baseline. Group I had a higher degree of improvement than group II (24+/-5 vs 19+/-5, p<0.001). Resident physicians' skills in death certificate completion can be improved with an educational intervention. An interactive workshop is a more effective intervention than a printed handout.

Death certificate completion: how well are physicians trained and are cardiovascular causes overstated?

PURPOSE: To determine the ability of residents in completing death certificates correctly. METHODS: A total of 4800 residents were asked to complete a survey and cause-of-death statement using a sample case of in-hospital death due to urosepsis, of whom 590 residents (12%) responded. A scoring scale designed based on instructions from the National Association of Medical Examiners and the American College of Pathologists was used to measure responses quantitatively. RESULTS: Overall performance was poor, with only 23% (n = 137) of responses in the optimal scoring range. The average score was influenced significantly by level of residency training, as well as previous experience, prior formal training, and awareness of the guidelines regarding death certificate completion. Optimal scores correlated with level of residency training and prior formal training in death certificate completion, suggesting the benefits of experience and instruction. Forty-five percent (n = 267) of respondents incorrectly identified a cardiovascular event as the primary cause of death. CONCLUSION: The residents in this study demonstrated suboptimal performance in death certificate completion. Cardiovascular events were often incorrectly identified as the primary cause of death. Formal training can improve performance and should be emphasized in medical schools and residencies.

Fatal pulmonary artery embolism in a sickle cell patient: case report and literature review.

This article reports a rare case of fatal saddle embolism to the pulmonary artery presenting as an Acute Chest Syndrome (ACS) in a Sickle Cell patient. We present a review of the etiology, pathophysiology, clinical manifestation and management of ACS. Clinicians should be aware of the fact that a sickle cell crisis admission may represent a life-threatening process. Such awareness will help physicians to act promptly and execute proper therapeutic interventions. It is important for clinicians to be expectant of impending clinical deterioration and likewise be aware that ACS can develop in patients hospitalized for other medical or surgical conditions.