Rapid Hepatomegaly From Ruxolitinib Discontinuation Syndrome.

Introduction: Ruxolitinib (RUX) is a Food and Drug Administration-approved Janus Kinase (JAK) inhibitor shown to be effective in improving hypercatabolic symptoms and splenomegaly in patients with myelofibrosis (MF). RUX therapy provides symptomatic benefits for MF patients but is often discontinued for various reasons including worsening cytopenias. Ruxolitinib Discontinuation Syndrome (RDS) involves an acute cytokine-storm rebound phenomenon that can manifest as an acute relapse of symptoms, worsening splenomegaly, respiratory distress, systemic inflammatory response syndrome, or disseminated intravascular coagulopathy. Case Presentation: We present the case of a patient with JAK2-positive post-polycythemia vera MF, whose RUX therapy was discontinued due to an active gastrointestinal (GI) bleed and worsening cytopenias. The patient had recently started azacitidine and was on the drug combination prior to hospitalization. The patient developed what appears to be the first case of acute onset accelerated massive hepatomegaly, a previously undescribed clinical manifestation of RDS. Conclusion: Although rare, medical professionals should maintain a high suspicion of RDS in hospitalized patients following the discontinuation of RUX.

Acute Esophageal Necrosis and Duodenal Disease in the Setting of Recently Initiated Chemotherapy.

Introduction: Acute esophageal necrosis (AEN), commonly referred to as "black esophagus" or Gurvits syndrome, is a rare condition characterized by diffuse black mucosa in the distal esophagus. Most often, the patient is an older male with multiple comorbidities, presenting with upper gastrointestinal bleeding. The exact pathogenesis is unclear, but it is often thought to be secondary to acute vascular hypo-perfusion or ischemia of the esophageal mucosa in critically ill patients with certain secondary comorbid conditions such as renal insufficiency, diabetes mellitus, dyslipidemia, coronary artery disease, malnourishment, alcohol abuse, or association with an underlying malignancy. Case Presentation: We present a case of AEN in a 78-year-old female following the recent start of a chemotherapy regimen with carboplatin and paclitaxel two weeks prior. The patient underwent EGD and was found to have AEN throughout the entirety of her esophagus with necrosis and eschars seen up to the second part of the duodenum. The patient initially improved after receiving blood transfusions, being made nil-per-os, and starting proton pump inhibitor (PPI) therapy, but she ultimately died given the severity of her clear cell uterine cancer and other comorbidities. Conclusion: Although it is rare that initiation of chemotherapy leads to AEN, it should be considered as a potential etiology.