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A 46-year-old Dominican man, known to have HIV, presented with constitutional symptoms of two week's duration. The patient was found to have cytopenias, significantly elevated ferritin level and lymphadenopathy. Biopsies and laboratory studies met the criteria for hemophagocytic lymphohistiocytosis (HLH). A concomitant diagnosis of histoplasmosis was confirmed as the trigger for HLH and treatment resulted in clinical improvement and resolution of symptoms.

Purpose To evaluate sphere concentration delivered to tumor and non-tumor tissue using voxel-based dosimetry as it relates to treatment, pathologic outcomes, and adverse events.Methods A retrospective, single-center analysis of patients (n = 57) with solitary HCC who were treated with Y90 radiation segmentectomy with Y90 glass microsphere infusion (TheraSphere; Boston Scientific, Marlborough, MA, USA) from 2020 to 2022 was performed. Post-treatment dosimetry was evaluated using Mirada DBx Build 1.2.0 Simplicit90Y dosimetry software. Voxel-based dosimetry and MIRD formula were utilized to calculate sphere concentration to tumor and non-tumor tissue. Time to progression (TTP), treatment response, pathologic response, and adverse events were studied.Results Fifty-seven patients with solitary tumors were analyzed with a median tumor diameter of 3.4cm (range 1.2-6.8cm). The median tumor absorbed dose was 692Gy (range, 256-1332Gy) with a median perfused treatment volume of 113mL (range, 33.6-442mL). Median sphere activity (SA) at time of delivery was 1428Bq (range, 412-2589Bq). Using voxel-based dosimetry and the MIRD formula, median tumor sphere concentration was 12,339 spheres/mL (range, 2,689 − 37,649 spheres/mL). Sphere concentration to tumor exhibited a weak, inverse correlation with perfused treatment volume (R2 = 0.25). However, tumor sphere concentration and non-tumor sphere concentration exhibited a direct, positive correlation (R2 = 0.72). Of the 52 tumors with post-treatment imaging, objective response was noted in 50 patients (96%) and complete response in 41 patients (79%). 98% of all treated tumors demonstrated a durable response at 2 years. The median time to progression for all patients was not reached with a 2-year progression rate of 11%. Multivariate analysis demonstrated target dose as the only statistically significant variable associated with TTP (p = 0.033). 14 patients underwent liver transplant. Median tumor necrosis was 99% (range, 80–100%).Conclusion Voxel-based dosimetry following Y90 radioembolization can be utilized to measure sphere concentration into tumor and non-tumoral tissue. Higher SA allows increased tumor absorbed dose with limited sphere/mL tumor capacity.

Background: Calciphylaxis is most commonly seen in end-stage renal disease and hemodialysis (uremic calciphylaxis). Nonuremic calciphylaxis is described, but large studies are lacking. Design: All calciphylaxis cases from 2010-2019 were identified. Uremic calciphylaxis cases (patients with ESRD, chronic kidney disease with creatinine level ≥ 3 mg/dl, acute kidney injury requiring renal replacement therapy or transplantation) were excluded. Charts and available biopsies were reviewed. Results: 24/68 (35%) were nonuremic calciphylaxis. Median age was 66 years (range 31-88) with an F: M ratio of 11:1. 19/24 (79%) had BMI >30 (median, 35.75). Patients presented with ulcers (17), retiform purpura (4) or indurated plaques (3) involving the thigh (9), lower leg (7), calf (6), abdomen (2), and axilla (1) Most had multiple medical conditions, including hypertension (19), diabetes mellitus (12), hyperlipidemia (10), autoimmune diseases (8), coronary artery disease (7), deep vein thrombosis (4), and atrial fibrillation (4). 3 had hyperparathyroidism at presentation. All had multiple medications, including diuretics (15), warfarin (11), antiplatelet drugs (10), corticosteroids, statins, opioids (8) and vitamin D supplements (14). Five had corrected serum calcium level > 10 mg/dl (median, 9.51 mg/dl). Seven had elevated parathyroid hormone level (>64 pg/ml, median, 51 pg/ml). 18 biopsies were available. Calcification was apparent on H&E in all cases and confirmed on 10 with von Kossa stains. All had calcification of small-medium caliber vessel walls. The calcification was predominantly stippled in 5, chunky in 2 cases and mixed stippled and chunky in 10. Calcification associated with thrombosis was seen in 11 and vessel wall necrosis in 4. Extravascular calcification was present in 10, involving subcutaneous tissue interstitium (7), perieccrine deposition (4), and dermis (1). Epidermal changes included full-thickness necrosis (3), ulceration (2), and erosion (1) to crust and vesicle formation (2). Dermal changes included reactive angioendotheliomatosis (4), perivascular chronic inflammation (4) and hemorrhage (2). Fat necrosis, present in 14, had septal fibrosis (3) and septal panniculitis (2) in a subset Conclusions: Nonuremic calciphylaxis is more common than recognized, accounting for >1/3 of our cases and disproportionately affects women. Obesity is the most common predisposing factor, but most patients had multiple comorbidities that could contribute to calciphylaxis.

2016-12-23T18:44:59