Clin. Pract., Volume 10, Issue 2 (May 2020) – 8 articles

Immune mediated necrotizing myopathy (IMNM) is part of the inflammatory myopathies group of diseases and presents with muscle weakness, myalgias and elevated serum creatine phosphokinase (CPK). Statin-induced IMNM is a rare complication. We present a patient with IMNM secondary to simvastatin use. The patient presented with proximal myopathy, dysphagia, and elevated creatinine kinase levels, and was subsequently found to have anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) autoantibodies with a necrotizing process on muscle biopsy. This patient’s case was further complicated by sequelae of multiple disease processes, ultimately leading to deterioration of his health. Full article

Acute esophageal necrosis, also known as black esophagus or acute necrotizing esophagitis is a rare condition with roughly 154 cases reported in the literature. This condition is classically diagnosed on its endoscopic findings of a circumferentially black esophagus that abruptly ends at the gastroesophageal junction and transitions to normal gastric mucosa. When present, acute esophageal necrosis potentially signifies a poor prognosis with an overall mortality up to 36%. This case report describes a critically ill patient with multiple comorbidities that was found to have acute esophageal necrosis the entire length of the esophagus secondary to ischemia/hypoperfusion that was caused by diabetic ketoacidosis. The patient had a prolonged hospitalization but was ultimately discharged in stable condition. We also review the literature of this rare esophageal condition. Full article

Brunner’s gland hyperplasia (BGH) is an unusual entity that presents with large duodenal polyp or mass and rarely causes gastrointestinal bleeding. It is usually asymptomatic and often an incidental finding during the esophagoduodenoscopy (EGD). However, most of the cases are benign. We encounter a 40 years old man who presented in Emergency Room with melena. EGD revealed solitary, large duodenal polyp in second part of duodenum and later histopathological findings were consistent with BGH. Full article

Odontogenic keratocyst (OKC) and ameloblastomas are distinct histopathologically diagnosed odontogenic lesions of the oral cavity. Both are primarily located in the posterior regions of the mandible, however, they can involve the maxilla as well. The occurrence of both an OKC and ameloblastoma in a patient is very uncommon. This case demonstrated such a lesion in the mandible of a 57 years old female. The diagnostic work-up and features of both lesions are illustrated with special focus on histopathological variances distinguishing OKC from ameloblastoma with the support of immunohistochemistry. This case highlights the importance of identifying accurate diagnoses for such lesions which may prompt clinical implications. Clinico-pathologic understanding of both lesions signifies the need for careful management plan and prevention of recurrence. Previously reported simultaneous occurrences of odontogenic cysts and/or tumors in the oral cavity are also reviewed. Full article

Nivolumab-induced immune thrombocytopenia (ITP) is a rare process with few reported cases. We present a 67-year-old man with advanced non-small cell lung cancer who was hospitalized with severe thrombocytopenia. Physical exam was notable for petechiae across his chest and extremities as well as bullae in his oral cavity. The patient initially received high-dose glucocorticoids and intravenous immuno - globulin, but did not respond to treatment. He was then started on weekly rituximab and after three doses, there was complete resolution of his thrombocytopenia. Altogether, his presentation was an extreme case and rare side effect of immune checkpoint therapy, known as nivolumab-induced ITP. Diagnosis of nivolumab-induced ITP is challenging given the lack of specific testing and a wide differential diagnosis. There are few cases reporting severe ITP following nivolumab treatment. We highlight the importance of recognizing and treating this rare complication of immunotherapy. Full article

Acute upper airway obstruction can be fatal. Early recognition of airway distress followed by diagnostic laryngoscopy and prompt intervention to secure airway control is crucial. We here present a 62-year old male patient who presented with cough and increasing respiratory distress for three weeks. Within the next 24 h, he developed symptoms of critical upper airway obstruction, endotracheal intubation was not possible, and an acute surgical tracheotomy was performed to retain patent airways. A computer tomography scan revealed severe laryngopharyngeal soft tissue thickening and upper airway obstruction caused by leukemic infiltration. He was diagnosed with acute leukemia and responded to induction chemotherapy. This case report points out the importance of establishing the diagnosis of critical upper airway obstruction in patients presenting with respiratory symptoms, and highlights the emergency management of airway obstruction due to malignant infiltration of leukemic blasts. Full article

Coronavirus disease-19 (COVID-19) has been regarded as an infective-inflammatory disease, which affects mainly lungs. More recently, a multi-organ involvement has been highlighted, with different pathways of injury. A hemoglobinopathy, hypoxia and cell iron overload might have a possible additional role. Scientific literature has pointed out two potential pathophysiological mechanisms: i) severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) interaction with hemoglobin molecule, through CD147, CD26 and other receptors located on erythrocyte and/or blood cell precursors; ii) hepcidin-mimetic action of a viral spike protein, inducing ferroportin blockage. In this translational medicine-based narrative review, the following pathologic metabolic pathways, deriving from hemoglobin denaturation and iron metabolism dysregulation, are highlighted: i) decrease of functioning hemoglobin quote; ii) iron overload in cell/tissue (hyperferritinemia); iii) release of free toxic circulating heme; iv) hypoxemia and systemic hypoxia; v) reduction of nitric oxide; vi) coagulation activation; vii) ferroptosis with oxidative stress and lipoperoxidation; viii) mitochondrial degeneration and apoptosis. A few clinical syndromes may follow, such as pulmonary edema based on arterial vasoconstriction and altered alveolo-capillary barrier, sideroblastic-like anemia, endotheliitis, vasospastic acrosyndrome, and arterio- venous thromboembolism. We speculated that in COVID-19, beyond the classical pulmonary immune-inflammation view, the occurrence of an oxygen-deprived blood disease, with iron metabolism dysregulation, should be taken in consideration. A more comprehensive diagnostic/therapeutic approach to COVID-19 is proposed, including potential adjuvant interventions aimed at improving hemoglobin dysfunction, iron over-deposit and generalized hypoxic state. Full article

Ameloblastoma is the only odontogenic tumor that displays diversified histomorphological features with subtypes like follicular, plexiform, acanthomatous, granular cell, clear cell, desmoplastic etc. In this paper we presented an extremely unusual presentation of ameloblastoma, which is characterized by desmolysis or acantholysis of stellate reticulum-like cells caused due to keratinocyte dissociation. A 35-year-old male patient presented with a painless hard 3×3 cm swelling in the mandibular right posterior region in the past 4-5 months. Radiographic examination revealed a multilocular radiolucent lesion in the body of mandible with resorption of the roots. Histopathological examination revealed ameloblastic follicles with central cells showing keratinocyte dissociation leading to desmolysis/acantholysis. Desmolytic cells were seen as an isolated entity in the follicular space with round to polygonal shaped morphology. Future retrospective studies on archival samples of ameloblastoma are recommended to relook into identification of such rare phenomenon. This will help in better understanding of the incidence rate and biological behavior of this rare variant of ameloblastoma. Full article