Fatal pulmonary embolism and pulmonary hemorrhage in lupus anticoagulant hypoprothrombinemia syndrome: a case report and review of literature.

: Lupus anticoagulant hypoprothrombinemia syndrome (LAHS) is a rare disorder characterized by development of lupus anticoagulant and antiprothrombin antibodies. The most common clinical manifestation is bleeding. Clinical management can be challenging due to the subtle balance between the bleeding and thrombotic tendencies. We report a novel case of LAHS in which the patient experienced the sequence of hemorrhage-thrombosis-hemorrhage before eventually dying of fatal pulmonary embolism and pulmonary hemorrhage. Specifically, she presented with multiple gastrointestinal bleeding episodes, followed by multifocal subdural hematomas, pulmonary embolism after normalization of prothrombin activity levels with immunosuppression, and finally with fatal pulmonary hemorrhage after enoxaparin treatment for pulmonary embolism. This case illustrates the importance of recognizing early minor bleeding episodes, and detecting specific antiprothrombin antibodies, in the diagnosis of LAHS. Furthermore, it highlights the complex challenge of normalizing prothrombin activity levels while at the same time preventing medical complications.

Diagnostic concordance rates in the subtyping of basal cell carcinoma by different dermatopathologists.

BACKGROUND: There are numerous subtypes of basal cell carcinoma (BCC). Defining the histopathologic subtype is an essential element in patient management, but there is little known data regarding interobserver precision in subtyping BCC. METHODS: We studied interobserver variance between six board-certified dermatopathologists who subtyped 100 BCCs in a blinded fashion. We used kappa statistic to calculate the concordance in suggested subtype by different dermatopathologists. Provided diagnoses were then re-categorized into low-risk and high-risk phenotypes, and kappa statistic for concordance on high-risk BCC was determined. RESULTS: The overall κ statistic was 0.301, indicating fair agreement among the six observers. Superficial and fibroepithelial BCC had the highest individual kappa statistics. When subtypes were re-classified into a two-tier system of high-risk and low-risk phenotypes, there was substantial interobserver agreement on high-risk BCC with a κ statistic of 0.699. CONCLUSION: These results suggest only fair agreement among dermatopathologists on specific BCC subtypes, but substantial agreement on superficial, fibroepithelial and high-risk BCC growth patterns. A simplified classification system comprised of superficial, fibroepithelial, nodular and infiltrative subtypes would increase interobserver precision and facilitate clinical decision-making.