Malignant Acute Disseminated Encephalomyelitis (ADEM) Presenting as a Stroke Alert: A Case Report.

Acute disseminated encephalomyelitis (ADEM) is a rare illness. Generally characterized by encephalopathy and non-specific, heterogeneous neurological deficits depending on the location of the demyelinated lesions, ADEM is considered a clinical diagnosis with radiological findings that may or may not have supportive features based on the temporal relationship of an inciting factor and symptom onset. Even rarer, hyperacute or malignant ADEM can be defined by rapid symptom onset followed by catastrophic brain edema and its sequelae. We present a case of a patient who presented with an acute stroke with activation of a rapid sequence care pathway (stroke alert protocol) to mobilize resources that could expedite his care to determine eligibility for thrombolysis. ADEM was the definitive diagnosis with a subsequent rapid and treatment-refractory decline.

Trastuzumab Associated With Recurrent Severe Thrombocytopenia and Successful Use of Pertuzumab Monotherapy.

Trastuzumab is a mainstay chemotherapeutic agent used in the treatment of human epidermal growth factor receptor 2 (HER2)/neu-positive breast cancer that, though generally well-tolerated, is classically associated with side effects like cardiotoxicity. Cytopenias can be seen but are generally secondary to other chemotherapeutic agents used in conjunction with trastuzumab. Herein, we present a case of recurrent severe thrombocytopenia following trastuzumab use that resolved following discontinuation. Our patient then finished a year of maintenance therapy with pertuzumab alone and is still in remission four years later. This is the eleventh report of this severe adverse effect described in the literature. This report contributes to the body of work describing this severe side effect by illustrating a clear temporal relationship between trastuzumab and severe thrombocytopenia, while also providing an alternate treatment option with chemotherapy and pertuzumab monotherapy. Given that pertuzumab is typically only used in addition to trastuzumab, evidence of its successful independent use is of clinical value to patients who may not be able to tolerate trastuzumab.

Incidence of Secondary Hemophagocytic Lymphohistiocytosis in Critically-Ill COVID-19 Patients.

Objective Coronavirus disease 2019 (COVID-19) is associated with diffuse lung injury that can progress to acute respiratory distress syndrome, multisystem-organ failure, and death. The inflammatory storm seen in many COVID-19 patients closely resembles secondary hemophagocytic lymphohistiocytosis (sHLH) which has been described in other virus-associated severe sepsis. We sought to describe the incidence of sHLH in COVID-19 infected patients. Design In this retrospective study, we reviewed the records of critically ill COVID-19 positive patients to determine the incidence of sHLH. An H-score for sHLH diagnosis was determined for each study participant, with a score greater than 169 points needed for diagnosis. Setting A quaternary referral center in suburban Pennsylvania, USA. Patients All study participants had a positive COVID-19 test, and were deemed critically ill defined as receiving invasive mechanical ventilation and/or who expired. Measurements and Main Results Of the 246 records identified, 242 records met inclusion criteria and were reviewed. Eighty five patients were excluded from analysis due to missing H-score data parameters. Overall, 32 of 157 (20.38%, 95% CI:14.38-27.54%) patients met diagnostic criteria for sHLH. The average age was 69.42 years (standard deviation (SD) 14.81). Patients diagnosed with sHLH were more likely to be younger (61.09 years vs 69.38 years, P = 0.0036), male (71.88% vs 52.00%, P = 0.0433), and require mechanical ventilation (96.88% vs 72.80%, P = 0.0035). Conclusions Among critically ill COVID-19 positive patients, the incidence of sHLH is higher than previously reported in patients with non-COVID-19 related sepsis. Clinicians caring for COVID-19 patients should consider this secondary diagnosis and subsequent appropriate treatments, especially in those requiring mechanical ventilation.

Central neurogenic hyperventilation secondary to suspected metastatic renal cell carcinoma.

Central neurogenic hyperventilation (CNH) is a neurogenic disorder rarely described within Emergency Medicine literature. CNH is a primary cause of hyperventilation, most commonly due to primary central nervous system neoplasms. Patient presentation varies based on the underlying cause, and may present with a sole chief complaint of dyspnea. We present a case of an adult male with a history of deep vein thrombosis, anticoagulated on apixaban, and extensively metastatic renal cell carcinoma who presented with a two-week history of dyspnea. Evaluation in the emergency department showed a primary respiratory alkalosis with a compensatory metabolic acidosis. Diagnostic work-up failed to reveal a cardiac, pulmonary, metabolic, or toxic cause. During the emergency department course, the patient became dysarthric and altered, at which point, computed tomography scan of the head revealed a pontine hemorrhage. The hemorrhage was stabilized with prothrombin complex concentrate, but the patient's dyspnea and mental status deteriorated throughout the course of his hospitalization. While the cause of the patient's hemorrhage was not elucidated, given the patient's widely metastatic disease, it was presumed to be secondary to metastasis. Our case highlights both a unique cause of a rare disorder of hyperventilation, and a diagnostic challenge to the emergency medicine provider. It is important to consider central causes of hyperventilation in patients with dyspnea and neurologic symptoms.

Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report.

INTRODUCTION: Hyperhemolysis syndrome (HHS) is a rare complication of repeat blood transfusions in sickle cell disease (SCD). This can occur acutely or have a delayed presentation and often goes unrecognized in the emergency department (ED) due to its rapid progression and similarity to acute chest syndrome and other common complications of SCD. CASE REPORT: We present a case of a 20-year-old male with SCD who presented to the ED with pain and tenderness in his lower extremities one day after discharge for a crisis. Unbeknownst to the ED team, during his admission he had received a blood transfusion. On presentation he was noted to have hyperkalemia, hyperbilirubinemia, anemia, and uncontrolled pain, and was admitted for sickle cell pain crisis. Over the next 36 hours, his hemoglobin dropped precipitously from 8.9 grams per deciliter (g/dL) to 4.2 g/dL (reference range: 11.5-14.5 g/dL), reticulocyte count from 11.7 % to 3.8% (0.4-2.2%), and platelets from 318,000 per cubic centimeter (K/cm3) to 65 K/cm3 (140-350 K/cm3). He also developed a fever, hypoxia, transaminitis, a deteriorating mental status, and severe lactic acidosis. Hematology was consulted and he was treated with methylprednisolone, intravenous immunoglobulin, two units of antigen-matched red blood cells, fresh frozen plasma, and cryoprecipitate. He was transferred to an outside hospital for exchange transfusion and remained hospitalized for 26 days with acute liver failure, bone marrow necrosis, and a fever of unknown origin. CONCLUSION: Because of the untoward outcomes associated with delay in HHS diagnosis and the need for early initiation of steroids, it is important for emergency providers to screen patients with hemoglobinopathies for recent transfusion at ED presentation. Asking the simple question about when a patient's last transfusion occurred can lead an emergency physician to include HHS in the differential and work-up of patients with sickle cell disease complications.

Spontaneous Tumor Lysis Syndrome in an Adenocarcinoma of Unknown Origin.

Spontaneous tumor lysis syndrome (STLS) is a rare oncologic emergency caused by massive cancer cell lysis or necrosis without a precipitating factor. Although tumor lysis syndrome (TLS) is most commonly associated with hematologic malignancies, a small number of cases in solid tumor malignancies have been reported. We present a case of spontaneous tumor lysis syndrome in a 77-year-old female with a widely metastatic, poorly differentiated adenocarcinoma of unknown origin. She presented in distributive shock, and laboratory testing at admission revealed acute renal failure, high anion gap metabolic acidosis, hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia. Rasburicase and continuous renal replacement therapy were initiated, however, her condition deteriorated. Treatment was withdrawn and she died four days after admission.