Hemoglobin SE (HbSE) disease is a hemoglobinopathy resulting from the combination of hemoglobin S (HbS) and hemoglobin E (HbE) genotypes. It may present as a vaso-occlusive crisis (VOC) in the setting of an acute stressor. Herein, we present a case of undiagnosed HbSE disease presenting as a massive splenic infarct in the setting of high-altitude exposure. A 55year-old female of South Asian descent presented with acute left upper quadrant abdominal pain after hiking in the Swiss Alps four days previously. Laboratory testing revealed that she had hemolytic anemia, and computed tomography (CT) imaging showed a greater than 50% splenic infarction. After the initiation of anticoagulation, she experienced a hemorrhagic conversion of the initial splenic infarct resulting in acute hemodynamic decompensation. She initially underwent vascular intervention with arterial plugging, coiling, and embolization but ultimately required a splenectomy and partial colectomy upon developing a large splenic hematoma. Hemoglobin electrophoresis was consistent with hemoglobin SE disease. Hemoglobin variants, especially combined heterozygosity, are rare and have the potential to present as a vaso-occlusive crisis in the setting of acute chemical and physiological stresses. Only 43 cases of hemoglobin SE disease have been previously reported and one other occurrence in the setting of high altitude. Conservative management is recommended when a diagnosis of sickle cell trait (SCT) is definite, in comparison with cardioembolic phenomena, in which antiplatelet and anticoagulant therapy should be initiated. Hemoglobin SE disease is a rare heterozygous hemoglobinopathy resulting from the combination of hemoglobin variants geographically separated by thousands of miles. Currently, there are no strict guidelines supporting anticoagulation for the management of VOC in hemoglobinopathies. Splenic infarct in HbSE disease should be managed similarly to SCT/sickle cell disease (SCD) with fluids and analgesia, and anticoagulation should be limited to confirmed thromboembolic events and with the insight of an anticoagulant specialist.
Background: E-cigarette or vaping associated lung injury (EVALI) is a lung disease associated with an inflammatory response to the vaping fluid. Currently, diagnosis remains elusive without definitive biomarkers. Case presentation: Herein, we describe three cases of EVALI among 18-to 21-year-old patients ranging from mild to severe. All cases presented with a combination of respiratory, gastrointestinal, and constitutional symptoms. Oxygen support and level of medical care varied based on disease severity. Bilateral pulmonary opacities were observed on chest imaging in each case. Additionally, each case had markedly elevated inflammatory markers, specifically C-reactive protein (CRP). None of these patients improved with intravenous (IV) antibiotics and all required IV corticosteroid therapy to achieve clinical improvement. Conclusion: EVALI should be suspected among young, otherwise healthy patients who present with newonset hypoxia, non-specific gastrointestinal symptoms, and endorse a history of vaping. Though considered a diagnosis of exclusion, diagnosing EVALI requires thorough history taking. Inflammatory studies, CRP, and erythrocyte sedimentation rate (ESR) should be considered adjunctive biomarkers to aid clinicians when the diagnosis remains unclear. Corticosteroids are the mainstay of treatment and patients should have close follow-up whether or not they require hospitalization.
Primary tracheal tumors are quite rare with an annual incidence of .1 per 100,000 people, with 90% being malignant in nature and commonly found in children. We present an exceedingly rare case of a benign tracheal hemangioma in an adult who presented with recurrent hemoptysis. CASE PRESENTATION: A 55-year-old female with no known past medical history presented to the emergency department with sudden onset large volume hemoptysis. She was a recent immigrant from Nigeria and was undergoing outpatient diagnostic workup for intermittent hemoptysis. On admission, she was hemodynamically stable with initial lab work significant for mild anemia (9.9g/dL) and moderate thrombocytopenia (52,000mm3). CT scan with contrast enhancement was concerning for an exophytic lesion involving the posterior trachea at the level of the aortic arch measuring 8x8x7mm. Rigid bronchoscopy was performed with complete resection of a pink-tan to red-brown polypoidal tumor involving the mid-trachea with 60% airway occlusion. Cytology results confirmed an ulcerated tracheal hemangioma without signs of malignancy. Six months post-operative images have not shown any further growth and complete patency of the trachea.
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