Polyarteritis Nodosa with Bilateral Asynchronous Testicular Necrosis: A Case Report

Polyarteritis nodosa (PAN) is a systemic vasculitis which may result in thrombosis or aneurysm formation in any organ of the body. We report a case polyarteritis nodosa (PAN) resulting in bilateral asynchronous testicular necrosis. A 55-year-old male developed acute onset of left testicular pain resulting in a left orchiectomy and right orchidopexy for an ischemic left testicle without evidence of torsion. Three weeks later, the patient developed acute right-sided scrotal pain, and surgical exploration revealed a right necrotic testicle resulting in a right orchiectomy. Pathologic evaluations demonstrated benign testes with acute interstitial hemorrhage and focal atrophy. The patient also experienced abdominal skin necrosis, penile pain and swelling, and temporary loss of vision. This is a unique case of PAN and the only case of asynchronous testicular necrosis in the medical literature

Polyarteritis nodosa with bilateral asynchronous testicular necrosis: a case report.

Polyarteritis nodosa (PAN) is a systemic vasculitis which may result in thrombosis or aneurysm formation in any organ of the body. We report a case polyarteritis nodosa (PAN) resulting in bilateral asynchronous testicular necrosis. A 55-year-old male developed acute onset of left testicular pain resulting in a left orchiectomy and right orchidopexy for an ischemic left testicle without evidence of torsion. Three weeks later, the patient developed acute right-sided scrotal pain, and surgical exploration revealed a right necrotic testicle resulting in a right orchiectomy. Pathologic evaluations demonstrated benign testes with acute interstitial hemorrhage and focal atrophy. The patient also experienced abdominal skin necrosis, penile pain and swelling, and temporary loss of vision. This is a unique case of PAN and the only case of asynchronous testicular necrosis in the medical literature

Venetoclax for Children and Adolescents with Acute Lymphoblastic Leukemia and Lymphoblastic Lymphoma

Venetoclax is approved for adult patients with chronic lymphocytic leukemia and acute myeloid leukemia. Expanding its use to the pediatric population is currently under investigation, but more robust data are needed. We retrospectively analyzed the safety and efficacy of venetoclax in children/AYA with ALL/LBL. We identified 18 patients (T-cell ALL, n = 7; T-cell LBL, n = 6; B-cell ALL, n = 5) aged 6–22 years. No new venetoclax safety signals were identified; the most common toxicity was myelosuppression. No deaths occurred within 30 days from the start of the therapy. A mean of 2.6 (range 0–8) prior lines of therapy were given. The mean duration of venetoclax was 4.06 months (range 0.2–24.67 months). Complete remission was achieved in 11 (61%) patients. Of the eight patients who remain alive, four are continuing on venetoclax combination therapy, and four proceeded to hematopoietic stem cell transplantation. Three patients who initially achieved CR, later relapsed, and are deceased. Nine patients are deceased, and one patient was lost to follow-up. Overall survival is 9.14 months (range 1.1–33.1), and progression-free survival is 7.34 months (range 0.2–33.1). This is the largest cohort of pediatric/AYA patients who received venetoclax for ALL/LBL. Our data support the consideration of venetoclax-based regimens in pediatric patients with R/R ALL/LBL and its investigation as upfront therapy for T-cell ALL/LBL

Venetoclax for Acute Myeloid Leukemia in Pediatric Patients: A Texas Medical Center Experience

The BCL-2 inhibitor venetoclax improves survival for adult patients with acute myeloid leukemia (AML) in combination with lower-intensity therapies, but its benefit in pediatric patients with AML remains unclear. We retrospectively reviewed two Texas Medical Center institutions’ experience with venetoclax in 43 pediatric patients with AML; median age 17 years (range, 0.6–21). This population was highly refractory; 44% of patients (n = 19) had ≥3 prior lines of therapy, 37% (n = 16) had received a prior bone marrow transplant, and 81% (n = 35) had unfavorable genetics KMT2A (n = 17), WT1 (n = 13), FLT3-ITD (n = 10), monosomy 7 (n = 5), TP53 (n = 3), Inv(3) (n = 3), IDH1/2 (n = 2), monosomy 5 (n = 1), NUP98 (n = 1) and ASXL1 (n = 1). The majority (86%) received venetoclax with a hypomethylating agent. Grade 3 or 4 adverse events included febrile neutropenia in 37% (n = 16), non-febrile neutropenia in 12% (n = 5), anemia in 14% (n = 6), and thrombocytopenia in 14% (n = 6). Of 40 patients evaluable for response, 10 patients (25%) achieved complete response (CR), 6 patients (15%) achieved CR with incomplete blood count recovery (CRi), and 2 patients (5%) had a partial response, (CR/CRi composite = 40%; ORR = 45%). Eleven (25%) patients received a hematopoietic stem cell transplant following venetoclax combination therapy, and six remain alive (median follow-up time 33.6 months). Median event-free survival and overall survival duration was 3.7 months and 8.7 months, respectively. Our findings suggest that in pediatric patients with AML, venetoclax is well-tolerated, with a safety profile similar to that in adults. More studies are needed to establish an optimal venetoclax-based regimen for the pediatric population

Nonmechanical Revision Indications Portend Repeat Limb-Salvage Failure Following Total Femoral Replacement

BackgroundThere is scant evidence to guide decision-making for patients considering total femoral replacement (TFR). We aimed to identify the indication, patient, disease, and surgical technique-related factors associated with failure. We hypothesized that failure occurs more frequently in the setting of revision surgical procedures, with infection as the predominant failure mode.MethodsWe performed a retrospective cohort study of patients receiving total femoral endoprostheses for oncological and revision arthroplasty indications; 166 patients met these criteria. Our primary independent variable of interest was TFR for a revision indication (arthroplasty or limb salvage); the primary outcome was failure. Analyses were performed for patient variables (age, sex, diagnosis group, indication), implant variables (model, decade, length, materials), and treatment variables. We analyzed TFR failures with respect to patient factors, operative technique, and time to failure. We conducted bivariate logistic regressions predicting failure and used a multivariate model containing variables showing bivariate associations with failure.ResultsForty-four patients (27%) had treatment failure. Failure occurred in 24 (23%) of 105 primary TFRs and in 20 (33%) of 61 revision TFRs; the difference was not significant (p = 0.134) in bivariate analysis but was significant (p = 0.044) in multivariate analysis. The mean age at the time of TFR was 37 years in the primary group and 51 years in the revision group (p = 0.0006). Of the patients who had mechanical failure, none had reoccurrence of their original failure mode, whereas all 8 patients from the nonmechanical cohort had reoccurrence of the original failure mode; this difference was significant (p = 0.0001).ConclusionsTFR has a high failure rate and a propensity for deep infection, especially in the setting of revision indications and prior infection. All failed TFRs performed for revision indications for infection or local recurrence failed by reoccurrence of the original failure mode and resulted in amputation.Level of evidenceTherapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence