Search

von Willebrand Disease (VWD) is a coagulopathy related to decreased or dysfunctional von Willebrand Factor (VWF) levels resulting in a myriad of bleeding manifestations, typically menorrhagia, postoperative bleeding, or mucocutaneous bleeding. VWD can be acquired or inherited and is estimated to have a prevalence of one percent. It has been well established in the literature that individuals with blood type O are more commonly affected by VWD type 1 as blood type O shortens the half-life of VWF. We describe a healthy 51-year-old female who presented with persistent headaches. Imaging of the head demonstrated bilateral subdural hematomas (SDH) in the absence of a traumatic event. An extensive workup was otherwise unremarkable, which prompted an investigation for a coagulopathy as a potential cause of her extreme presentation. Hematologic studies disclosed a partial deficiency of VWF consistent with VWD type 1. Given the self-limited nature of her symptoms and the spontaneous regression of the hematomas, our patient was treated conservatively. Our case is unique with respect to bilateral nontraumatic subdural hematomas as a primary clinical manifestation of VWD and blood type B as opposed to the more commonly encountered blood type O in patients with a more extreme presentation of type 1 VWD. [ABSTRACT FROM AUTHOR]

Background: For a number of decades, hairy cell leukemia (HCL) has been linked with polyarthritis, vasculitis, Symptomatic cytopenias and thrombosis in the medical literature. Notwithstanding, the significance of these associations has not been well understood. Therefore, we have decided to analyze them further. Methods: We provide herein a comprehensive literature review of the prevalence of autoimmune disorders in patients with HCL. Most relevant publications were identified through searching the PubMed/Medline database for articles published from inception to February 2014. Findings: Perhaps due to the rarity of HCL, scientific literature on autoimmune conditions in patients with HCL consists mainly of published case series and isolated reports. Our analysis identified increased prevalence of various autoimmune conditions in patients with HCL, including various vasculitides, immune cytopenias and antiphospholipid antibody syndrome (APS) among others. Conclusions: Presence of certain autoimmune disorders should increase the suspicion of HCL in an appropriate clinicolaboratory context. Conversely, the diagnosis of HCL should prompt early recognition of certain autoimmune disorders if clinical suspicion exists. While some of these autoimmune diseases are thought to be secondary to the dysfunctional immune response associated with underlying malignant process, others could be primary and might even play a role in the HCL pathogenesis. The autoimmune complications can pose important clinical problems for the HCL patients. Therefore, a catalogue of these problems is important for alerting physicians to watch for them and diagnose them promptly. [ABSTRACT FROM AUTHOR]

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limited disease of unknown etiology. This condition is most commonly encountered in Asian and Caucasian females of childbearing age who usually present with cervical lymphadenopathy and fever. Though rarely, KFD has been described in younger African-American females as well. It often mimics more serious conditions such as lymphoma, metastatic solid malignancy, HIV infection, tuberculosis, sarcoidosis, or systemic lupus erythematosus. Although its etiopathogenesis has not been fully elucidated, literature suggests viral or possibly autoimmune components to play a role. We describe a 34-year-old African-American female who presented with constitutional symptoms and polyadenopathy on clinical examination and imaging, of which the portacaval and portahepatis lymph nodes were most prominent. An extensive workup was otherwise unremarkable, and biopsy showed histiocytic necrotizing lymphadenitis. Initially, her clinical condition improved spontaneously, and she required only a short course of oral steroids. Three months later, she relapsed with bilateral cervical adenopathy and constitutional symptoms and was successfully managed again with steroids. Our case is unique with respect to (a) portahepatis and portacaval node enlargement as the dominant adenopathy and (b) her underlying conditions of fibromyalgia and chronic fatigue syndrome. [ABSTRACT FROM AUTHOR]