A Rare Case of Hereditary Hemochromatosis Presenting With Porphyria Cutanea Tarda.

Hereditary hemochromatosis is an autosomal recessive condition with incomplete penetrance that is most commonly caused by a mutation in the HFE gene. Hereditary hemochromatosis can remain asymptomatic in some patients until triggered by certain events. Porphyria cutanea tarda is a condition that can lead to iron overload due to defective synthesis of heme and can cause the onset of adult-onset hereditary hemochromatosis. Herein, we present a case where a 77-year-old man presented with painful blisters on the sun-exposed areas of his hands and was diagnosed with porphyria cutanea tarda. Further testing for mutations in the HFE gene given elevated ferritin was performed and returned positive, which confirmed the diagnosis of adult-onset hereditary hemochromatosis. The patient received serial therapeutic phlebotomy for iron overload and adopted lifestyle modifications such as avoiding sun exposure of upper extremities. The patient's blisters and laboratory iron panel parameters improved with continued phlebotomy. Therapeutic phlebotomy has been demonstrated to be an effective first-line therapy in patients with dual diagnosis. Our case highlights that cutaneous symptoms due to porphyria cutanea tarda may be the first presenting symptom in patients with underlying hemochromatosis.

Lower Fracture Rates in Patients Treated with Radium-223, Abiraterone or Enzalutamide, When Given Concurrently with Bone Health Agents: A Real-World Analysis.

BACKGROUND: The phase 3 trial ERA223 demonstrated an increased fracture rate and no survival advantage for metastatic castration resistant prostate cancer (mCRPC) patients on Radium-223 (Ra-223) with abiraterone, leading to regulatory restrictions on combination therapy. However, less than half of trial patients received bone health agents (BHA). We reviewed electronic health record (EHR) data evaluating fracture rates for patients on BHA receiving Ra-223, androgen deprivation therapy and either abiraterone or enzalutamide. PATIENTS AND METHODS: We conducted a retrospective, cohort analysis of EHR data of mCRPC patients on Ra-223 treated at a single community center by a single provider between 2010 and 2018. The primary objective was evaluating fracture rates for patients on BHA receiving Ra-223 and abiraterone. We conducted a secondary analysis for enzalutamide. RESULTS: One hundred seventy-seven patients received Ra-223 concurrently with abiraterone or enzalutamide between November 2010 and August 2018. The median age was 73 at first Ra-223 dose (range 40-93). The median follow-up time from last Ra-223 dose was 30 months (range 2-106). One hundred sixty-four patients (93%) received BHAs. One hundred fifty-nine patients (89%) were on a BHA before and/or during Ra-223. Sixty-seven patients received denosumab (38%), 63 received zoledronic acid (36%), and 29 received both nonconcurrently (16%). Eleven patients (6.2%) experienced a fracture after starting Ra-223, 9 of which occurred while on prior and/or concurrent BHA. We observed a 5.7% fracture rate for mCRPC patients who received combination therapy and denosumab or zoledronic acid. CONCLUSION: This real-world analysis demonstrating a low fracture rate in patients with mCRPC receiving a BHA while on Ra-223 and abiraterone or enzalutamide may inform current clinical practice.

Horner Syndrome induced by toxoplasmosis infection in a patient with AIDS and disseminated herpes simplex virus.

PURPOSE: This case report describes a case of Horner syndrome resulting from central nervous system (CNS) toxoplasmosis in an immunocompromised patient. Horner Syndrome is a neurological condition characterized by unilateral miosis, ptosis with apparent enophthalmos, and anhidrosis due to inhibition of the sympathetic pathway. The ocular sympathetic pathway runs from the posterolateral hypothalamus to the ophthalmic branch of the trigeminal nerve (cranial nerve V1). Central nervous system (CNS) toxoplasmosis infection is typically only seen in immunocompromised patients. To our knowledge, toxoplasmosis has never been reported as a cause of Horner syndrome. OBSERVATIONS: A forty-four-year-old Caucasian male was admitted to the hospital for left upper extremity paresthesias, gait instability, and painful vesicular skin lesions, and Horner syndrome. Upon review, he had an 18-year history of HIV initially controlled on anti-retrovirals but had been lost to follow-up for several years until he developed severe headaches determined to be caused by Toxoplasmosis lesions in his brain. Over several months he was treated for the Toxoplasmosis but had poor adherence to medications. After subsequent admission and workup, we found multi-focal ring enhancing lesions on MRI in the basal ganglia, hypothalamus, thalamus, and internal capsule. We postulated that the hypothalamic lesion was the cause of his Horner syndrome. After treatment for both toxoplasmosis and HSV his Horner syndrome and other neurologic symptoms resolved. CONCLUSIONS AND IMPORTANCE: This is the first reported case of Horner syndrome resulting from CNS toxoplasmosis. This case report and the accompanying questions provide an opportunity to review and explore the neuroanatomy and subtle symptomatic differences between various etiologies of Horner syndrome (primary, secondary, tertiary) in the context of a novel presentation. In conclusion, toxoplasmosis should be considered when investigating Horner syndrome in immunocompromised patients.

Agranulocytosis with ceftaroline high-dose monotherapy or combination therapy with clindamycin.

INTRODUCTION: The only clinically available cephalosporin with in vivo and in vitro activity against methicillin-resistant Staphylococcus aureus is ceftaroline, which is approved for the treatment of soft tissue infection and community-acquired pneumonia in doses of 600 mg intravenously every 12 hours for 2 weeks or less. However, many clinicians use ceftaroline to treat more serious infections and dose more frequently (every 8 hours) for longer periods of time. METHODS: A retrospective medication safety assessment was performed at two centers where agranulocytosis was observed in four patients (two at each center) who were treated with ceftaroline. The cases were reviewed by the treating physicians for common features, and the frequency of agranulocytosis was calculated based on the total number of treated patients. RESULTS: We report four cases of agranulocytosis associated with ceftaroline use, highlighted by prolonged use (more than 14 days) and 8-hour dosing intervals or 12-hour dosing intervals with concomitant clindamycin therapy. When ceftaroline (600 mg every 12 hours) and clindamycin (900 mg every 8 hours) were coadministered for more than 2 weeks, the frequency of agranulocytosis was 18% (2 of 18 patients treated). When ceftaroline alone was administered for more than 2 weeks at 600 mg every 8 hours, agranulocytosis occurred in 5.4% (2 of 37 treated patients). No cases of ceftaroline-related agranulocytosis were seen that did not have these features. In these patients, granulocyte-colony stimulating factor therapy usually resulted in rapid myeloid recovery. CONCLUSIONS: Clinicians should have a heightened awareness of agranulocytosis when using ceftaroline in such settings and monitor complete blood counts at least once/week.