Kyaw Zin Thein

A Rare Concurrence of Leiomyomatosis Peritonealis Disseminata, Leiomyosarcoma of the Pelvis and Leiomyomatous Nodule of the Liver

Leiomyomatosis peritonealis disseminata (LPD) is a rare entity that is characterized by the presence of multiple subperitoneal or peritoneal smooth muscle nodules throughout the peritoneal surface mimicking a malignant process. LPD follows a benign course in general, and it is often found incidentally during abdominal surgery. There have been reported cases of LPD with malignant degeneration although the association is uncertain. Concurrent finding of LPD and leiomyosarcoma of the pelvis is very rare that could be coincidental, malignant transformation of LPD to leiomyosarcoma, or progression of undetected primary leiomyosarcoma. There are only a few previously reported cases in the literature. Herein, we report a case of 56-year-old woman with a history of leiomyoma of uterus who presented with progressive abdominal swelling secondary to mass lesions in the pelvis. The patient underwent exploratory laparotomy and debulking of the tumors, and the histologic examination of the tumors revealed coexistence of LPD and leiomyosarcoma. After recovery from the operation, core needle biopsy of the superficial, residual liver mass was obtained to investigate potential liver metastasis, and the histopathologic findings are consistent with leiomyoma which represents the first simultaneous occurrence of LPD, leiomyosarcoma, and leiomyomatous nodule of the liver.

Acute symptomatic hypocalcemia from immune checkpoint therapy-induced hypoparathyroidism

Background Ipilimumab (a monoclonal antibody against CTLA‐4) and nivolumab (a humanized antibody against PD‐1) target these immune checkpoint pathways and are used for treatment of melanoma and an increasing number of other cancers. However, they may cause immune‐related adverse effects (IRAEs). Although many endocrinopathies are known to be IRAEs, primary hypoparathyroidism with severe hypocalcemia has never been reported. This is the first case of hypoparathyroidism as an IRAE presenting to an Emergency Department with acute hypocalcemia. Case description A 73‐year‐old man with metastatic melanoma presented to the Emergency Department for the chief complaints of imbalance, general muscle weakness, abdominal pain and tingling in extremities. He had wide spread metastasis, and begun immunotherapy with concurrent ipilimumab and nivolumab 1.5 months ago. At presentation, he had ataxia, paresthesia in the hands and feet, and abdominal cramping. Magnetic resonance imaging of the brain was unremarkable. He was found to be hypocalcemic with undetectable plasma parathyroid hormone. He was admitted for treatment of symptomatic hypocalcemia and was diagnosed with primary hypoparathyroidism. Shortly afterwards, he had thyrotoxicosis manifesting as tachycardia and anxiety, followed by development of primary hypothyroidism. At 4 months after the Emergency Department visit, his parathyroid function and thyroid function had not recovered, and required continued thyroid hormone replacement and calcium and vitamin D treatment for hypocalcemia. Conclusions Primary hypoparathyroidism caused by ipilimumab and nivolumab may acute manifest with severe symptomatic hypocalcemia. Emergency care providers should be aware of hypoparathyroidism as a new IRAE in this new era of immuno‐oncology.

Thrombosis in Philadelphia negative classical myeloproliferative neoplasms: a narrative review on epidemiology, risk assessment, and pathophysiologic mechanisms

Thrombosis is common in cancer patients and is associated with increased morbidity and mortality. Myeloproliferative neoplasms (MPN) are common malignancies in elderly individuals and are known for a high incidence of thrombotic complications. Different risk factors have been identified in studies, and risk models have been developed to identify patients with MPN at higher risk for thrombosis. Several pathophysiological mechanisms help explain the increased likelihood of thrombosis in these patients. Factors, such as leukocyte and platelet activation leading to the formation of leukocyte–platelet aggregates, activation of the coagulation cascade by microparticles, high levels of inflammatory cytokines, and endothelial dysfunction have a crucial role in thrombosis in MPN patients. Recent studies have demonstrated a significant association between the allele burden of specific genetic mutations (mainly JAK2V617F) associated with MPN and the incidence of thrombotic events, thus suggesting a possible role for these mutations in thrombogenesis.

Copper deficiency anemia: review article

Copper is a crucial micronutrient needed by animals and humans for proper organ function and metabolic processes such as hemoglobin synthesis, as a neurotransmitter, for iron oxidation, cellular respiration, and antioxidant defense peptide amidation, and in the formation of pigments and connective tissue. Multiple factors, either hereditary or acquired, contribute to the increase in copper deficiency seen clinically over the past decades. The uptake of dietary copper into intestinal cells is via the Ctr1 transporter, located at the apical membrane aspect of intestinal cells and in most tissues. Copper is excreted from enterocytes into the blood via the Cu-ATPase, ATP7A, by trafficking the transporter towards the basolateral membrane. Zinc is another important micronutrient in animals and humans. Although zinc absorption may occur by direct interaction with the Ctr1 transporter, its absorption is slightly different. Copper deficiency affects physiologic systems such as bone marrow hematopoiesis, optic nerve function, and the nervous system in general. Detailed pathophysiology and its related diseases are explained in this manuscript. Diagnosis is made by measuring serum copper, serum ceruloplasmin, and 24-h urine copper levels. Copper deficiency anemia is treated with oral or intravenous copper replacement in the form of copper gluconate, copper sulfate, or copper chloride. Hematological manifestations are fully reversible with copper supplementation over a 4- to 12-week period. However, neurological manifestations are only partially reversible with copper supplementation.

Adverse Effects of Immune Checkpoint Therapy in Cancer Patients Visiting the Emergency Department of a Comprehensive Cancer Center

Study objective Cancer immunotherapy is evolving rapidly and is transforming cancer care. During the last decade, immune checkpoint therapies have been developed to enhance the immune response; however, specific adverse effects related to autoimmunity are increasingly apparent. This study aims to fill the knowledge gap related to the spectrum of immune‐related adverse effects among cancer patients visiting emergency departments (EDs). Methods We performed a retrospective review of patients treated with immune checkpoint therapy who visited the ED of a comprehensive cancer center between March 1, 2011, and February 29, 2016. Immune‐related adverse effects from the ED visits were identified and profiled. We analyzed the association of each immune‐related adverse effect with overall survival from the ED visit to death. Results We identified 1,026 visits for 628 unique patients; of these, 257 visits (25.0%) were related to one or more immune‐related adverse effects. Diarrhea was the most common one leading to an ED visit. The proportions of ED visits associated with diarrhea, hypophysitis, thyroiditis, pancreatitis, or hepatitis varied significantly by immune checkpoint therapy agent. Colitis was significantly associated with better prognosis, whereas pneumonitis was significantly associated with worse survival. Conclusion Cancer patients treated with ipilimumab, nivolumab, or pembrolizumab may have a spectrum of immune‐related adverse effects that require emergency care. Future studies will need to update this profile as further novel immunotherapeutic agents are added.

Pernicious Anemia: Fundamental and Practical Aspects in Diagnosis

BACKGROUND Pernicious Anemia (PA), the most common cause of cobalamin deficiency anemia worldwide, is an autoimmune disease of multifactorial etiologies involving complex environmental and immunological factors. Although it was first reported by Addison in 1849 with subsequent advances in understanding of pathogenesis and molecular biology, diagnosis of PA is still challenging for clinicians because of its complexity and diverse clinical presentations. CONCLUSION Herein, we provide an overview of PA, mainly focusing on its scientific and practical aspects in diagnosis. We also discuss the limitations of currently available diagnostic tools for the evaluation of cobalamin deficiency and PA.

Mushroom Poisoning Mimicking Painless Progressive Jaundice: A Case Report with Review of the Literature

Mushroom poisoning is common in the United States. The severity of mushroom poisoning may vary, depending on the geographic location, the amount of toxin delivered, and the genetic characteristics of the mushroom. Though they could have varied presentation, early identification with careful history could be helpful in triage. We present a case of a 69-year-old female of false morel mushroom poisoning leading to hepatotoxicity with painless jaundice and biochemical pancreatitis.

A Systematic Review and Meta-Analysis of Randomized Controlled Trials to Evaluate the Risk of Hematological Toxicities in Patients with Multiple Myeloma Treated with Daratumumab

S242 p 1⁄4 0.70); asthenia, 0.84 (95% CI: 0.38 e 1.85, p 1⁄4 0.66); and insomnia, 1.05 (95% CI: 0.80 e 1.36; p 1⁄4 0.71). The RR of highgrade adverse effects were as follows: nausea, 1.92 (95% CI: 0.17 e 21.60; p 1⁄4 0.59); diarrhea, 1.43 (95% CI: 0.75 e 2.74, p 1⁄4 0.27); fatigue, 1.87 (95% CI: 0.99 e 3.52; p 1⁄4 0.051); asthenia, 0.81 (95% CI: 0.30 e 2.14, p 1⁄4 0.67); and insomnia, 0.30 (95% CI: 0.04 e 1.93; p 1⁄4 0.20). Conclusions: Addition of daratumumab did not increase the risk of gastrointestinal toxicities and healthrelated quality of life events in patients with multiple myeloma compared to control arm, while contributing substantial survival benefits.

Primary thromboprophylaxis (PTP) in ambulatory patients with lung cancer receiving chemotherapy: A systematic review and meta-analysis of randomized controlled trials (RCTs)

Thromboembolism (TE) is a leading cause of death in cancer patients. Primary thromboprophylaxis (PTP) in ambulatory cancer patients receiving chemotherapy has been debated and considered to potentially improve survival by reducing TE occurrence.

Primary esophageal diffuse large B cell lymphoma presenting with tracheoesophageal fistula: A rare case and review

Primary non-Hodgkin lymphomas in the esophagus are rare. Tracheoesophageal fistulas mainly arise from solid esophageal carcinoma or mediastinal malignancies. Our patient presented with cough, dysphagia and weight loss, and upon initial computed tomography imaging and esophagogastroduodenoscopy, a malignant mass in the middle third of esophagus with tracheoesophageal fistula was found. The location of the mass and presence of malignant tracheoesophageal fistula were strongly suggestive of squamous cell carcinoma. However, tumor biopsy revealed diffuse large B-cell lymphoma. This case report details a rare incident of a primary diffuse large B-cell lymphoma presented as tracheoesophageal fistula and reviews previous literature.