Hepatic Langerhans Cell Histiocytosis (LCH) Mimicking Antimitochondrial Antibody (AMA)–Negative Primary Biliary Cholangitis (PBC), Presenting as a Harbinger of Multisystem LCH

Abstract Objectives LCH is a rare systemic disorder characterized by an infiltration of CD1a+/Langerin+ histiocytes. LCH commonly involves bone, skin, and lymph nodes in children. Hepatic involvement is rarely observed in multisystem LCH. We describe an exceptional case of hepatic LCH in an adult, mimicking AMA-negative PBC, preceding the diagnosis of multisystem LCH. Methods A 65-year-old man presented with intermittent pruritus, weakness, dyspnea, fever, and chills that have been progressive for 4 years. Physical examination was unremarkable. Laboratory work revealed elevated alkaline phosphatase (471 U/L; ref 40-120 U/L), GGT (271 U/L; ref 0-41 U/L), total bilirubin (2.0 mg/dL; ref 0.0-1.2 mg/dL), and direct bilirubin (1.1 mg/dL; ref 0.0-0.3 mg/dL). Indirect bilirubin, AST, and ALT were within normal limits. Liver biopsy was performed. Results Liver biopsy showed lobular and portal nonnecrotizing granulomas with one “florid duct lesion” with duct injury, suggestive of PBC. Tests for autoimmune diseases including AMA were negative. ERCP was negative for biliary obstruction. One month after the liver biopsy, he developed flaking, red, and burning rash on the right scalp, forehead, and epigastric skin. A skin biopsy at an outside institution revealed dermal and epidermal infiltration of CD1a-positive histiocytes with indented nuclei and pale eosinophilic cytoplasm, consistent with LCH. Subsequent reexamination of the liver biopsy showed that the histiocytes within the florid duct lesion were positive for CD1a and S-100, while the remaining granulomas were negative. The following day, a small focus of LCH was noted in his gastric biopsy performed for gastritis symptoms. Conclusion Hepatic LCH may mimic AMA-negative PBC histologically and clinically, and may present as a harbinger of multisystem LCH. While rendering the diagnosis would be challenging without prior history of LCH and with focal involvement, awareness of such presentation and communication with clinical colleagues may be helpful. </jats:sec

Polyarteritis Nodosa Following Vaccination Against Hepatitis B Virus with Lower-Extremity Manifestations: A Case Report

Polyarteritis nodosa is a progressive, often life-threatening, vasculitis affecting multiple organs, including the skin and peripheral nerves. We report a patient presenting with systemic features of the disease and with characteristic lesions in the feet 3 weeks after vaccination against hepatitis B virus infection.</jats:p

Successful Surgical Intervention for the Management of Endocarditis due to Multidrug Resistant Candida parapsilosis: Case Report and Literature Review

Candida parapsilosis is an uncommon cause of invasive endocarditis. This pathogen induces severe complications and carries a high mortality rate. We describe a case of C. parapsilosis endocarditis in a 54-year-old man with a history of HIV and Hepatitis C infection who previously underwent prosthetic valve replacement due to bacterial endocarditis. The patient presented with prolonged febrile episodes and fungemia with repeat blood cultures positive for C. parapsilosis. The patient failed multiple regimens of antifungal therapy and the C. parapsilosis isolate progressively acquired resistance to a number of drugs. Due to the multidrug resistant nature of the isolate, replacement of the infected valve was required to resolve his fungemia, and the patient remained asymptomatic for two years. This case is unusual due to the multidrug resistant nature of the isolate requiring both combined medical and surgical intervention. A review of published reports indicates that endocarditis due to C. parapsilosis responds well to a combination of medical and surgical interventions; the latter is particularly suitable for immunocompromised hosts

Definitive urine drug testing in emergency medicine: Recreational and psychiatric drug findings

Introduction: Clinical management of drug-related emergency department (ED) visits relies on available history, toxidrome findings and drug screening. In this study, definitive drug testing is used to assess ED drug prevalence and immunoassay drug screening performance. Methods: Definitive testing for 116 drugs and metabolites was performed on urine from 400 ED patients, with comparison to immunoassay drug screening. Results: Definitive testing resulted in 1,350 drug findings with prevalent use of nicotine (63%), cocaine (34%), ∆9 tetrahydrocannabinol (34%), fentanyl (17%), morphine or heroin (11%) and methamphetamine (6%). Forty percent of patients were also positive for antidepressants and 24% positive for antipsychotics. Significant patterns of co-drug use were found for cocaine, fentanyl, morphine and nicotine. Multi-serotonergic drug use was frequent, suggesting a risk for serotonin syndrome. Immunoassay performance showed high false negative rates for benzodiazepines (40%), amphetamines (38%), barbiturates (33%), opiates (25%), methadone (20%) and cocaine (16%), along with inaccuracy in phencyclidine detection. Immunoassay missed 890 of the 1,350 drug findings by definitive testing, due to either high cutoff thresholds or limited testing scope. Discussion: A high prevalence of drugs use by ED patients is evidenced with frequent co-use of illicit and therapeutic drugs and with potential for unrecognized multi-serotonergic drug interactions. This study also shows the limitations of immunoassay drug testing in both scope and sensitivity, with a high rate of undetected drug use. Conclusion: The study provides evidence-based support for recommended implementation of definitive drug testing in emergency medicine as a guide to clinical management in drug-related ED visits

A Poorly Differentiated Malignant Neoplasm Lacking Lung Markers Harbors an EML4-ALK Rearrangement and Responds to Crizotinib

Suspected metastatic site lesions that are poorly differentiated present a diagnostic challenge when morphologic and immunohistochemical profiling cannot establish the primary tumor site. Here we present a patient diagnosed with both a malignant neoplasm in the lung and a right upper extremity (RUE) neoplasm of unclear histogenetic origin. Immunohistochemical staining performed on the latter specimen was inconclusive in determining the site of origin. Although the lung biopsy sample was insufficient for molecular testing, hybrid capture-based comprehensive genomic profiling (FoundationOne) identified an EML4-ALK rearrangement in the RUE lesion. Crizotinib treatment resulted in a major response in both the RUE and the lung lesions. This report illustrates the utility of comprehensive genomic profiling employed at the initial presentation of an unknown primary malignant neoplasm, which resulted in the front-line use of targeted therapy and a significant and sustained antitumor response