Beyond Midostaurin: Role of Avapritinib in Managing Systemic Mastocytosis.

We present a case of an adult male who presented with pancytopenia accompanied by symptomatic anemia, necessitating chronic transfusions. He was diagnosed with systemic mastocytosis with an associated hematologic neoplasm. Following an inadequate response to midostaurin therapy, the patient was initiated on the newly approved avapritinib. The patient showed significant improvements in all three blood cell lines; however, he developed leg edema, blepharedema, and gum bleeding on this medication. This case underscores the intricacies of managing a patient with advanced systemic mastocytosis, the emerging role of highly selective KIT inhibition in its treatment, and the practical management of adverse medication effects.

The Utility of Echocardiography in Estimating Fluid Responsiveness.

Assessing the volume status in critically ill patients is the key to maintain the stability of the hemodynamics; however, it can be challenging to view the complexity of cases and the diversity of shock etiology. Multiple noninvasive means have been used to study the effectiveness of volume resuscitation, but none of them have been used as gold standard. We aim to illustrate the most used techniques: left ventricular outflow tract velocity time integral versus inferior vena cava compressibility index, and highlight their limitations and strengths. These tools are both operator-dependent and might be affected by several factors including ventilator settings.

A Rare Case of High-Grade Atrioventricular Block in Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA) is an autoimmune disease that affects small and medium-sized vessels. It is classically known to present with renal and respiratory tract symptoms. However, the disease can manifest in other organ systems, especially cardiovascular involvement. Though there are multiple reports of cardiac involvement in GPA, it is not commonly evaluated and is often overlooked in patients with GPA. Heart disease in GPA has a wide range of presentations ranging from subacute and silent to severe abnormalities, which can prove fatal if not identified and treated appropriately. Identifying cardiac involvement early in patients with no apparent signs can help with prevention strategies and follow-up to avoid significant complications. Pericarditis is the most common pathology noted in GPA, followed by cardiomyopathy, coronary artery disease, valvular disease, and conduction abnormality. In our report, we present a case of GPA in a young male with asymptomatic conduction abnormality of the heart. Although it was silent at the presentation, identifying the initial electrocardiogram (ECG) changes prompted us to admit him to the telemetry floor. Continuous telemetry monitoring helped us identify the progression of the conduction abnormality, which otherwise could have been missed. This led us to correlate to his symptoms which he later developed during his admission course. His symptoms subsided after prompt treatment. If not identified early, these cardiac abnormalities can delay management, leading to increased disease burden and morbidity. Hence, essential cardiac work with at least ECG and continuous telemetry monitoring is recommended.

Lactococcus endocarditis after Bentall procedure presenting with intracranial hemorrhage.

Background: Endocarditis is more common in patients with cardiac prostheses. A Bentall procedure entails surgical replacement of the aortic valve, aortic root, and ascending aorta with re-implantation of coronary arteries into the graft. Case: 65-year-old male with history of atrial fibrillation on rivaroxaban, bicuspid aortic valve, and ascending aortic aneurysm with a history of a Bentall procedure two years prior, presented with headache and dysarthria for one day. National Institutes of Health Stroke Scale was 3 and CT head showed 2.7 cm left frontal hematoma with extension into the subarachnoid space. Andexanet alfa was given for rivaroxaban reversal followed by cerebral angiogram which showed 5 mm intracranial inferior MCA aneurysm and embolization and coil placement was done. Blood cultures grew Lactococcus garvieae and transesophageal echocardiogram revealed aortic valve thickening and vegetation on the non-coronary cusp. He was subsequently treated with six weeks of IV ceftriaxone and Gentamycin. Conclusion: With increasing use of bioprosthetic valves, the possibility of infective endocarditis with uncommon pathogens should be kept in mind. Lactococcus commonly affects native valves, however it can affect bioprosthetic valves and can present with mycotic aneurysms.

Human Herpes Virus 6-Associated Meningitis in an Immunocompetent Adult With a History of Plaque Psoriasis: A Case Report.

Human herpes virus-6 (HHV-6) is a common cause of viral infection in humans, recognized for causing exanthema subitem during the first two years of life. Chronic inflammatory states in different autoimmune diseases can be associated with an immunosuppressed microenvironment. We report a case of HHV-6-associated meningitis in a patient with psoriasis. A 36-year-old man with a history of psoriasis presented with worsening bifrontal headache as well as painful oral lesions on the tongue and soft palate. Computed tomographic (CT) scan of the brain was nondiagnostic. Cerebrospinal fluid (CSF) analysis was diagnostic for HHV-6 infection and was treated with intravenous ganciclovir for two weeks. This case report highlights the emergence of HHV-6 infections in people with underlying mild immune disorders, such as psoriasis. Plaque psoriasis, such as in this patient, could be related to viral infections not typically seen among adults.