Management of Hyperhemolysis in ß-thalassemia With Multiple Immunosuppressives, Including Complement Blockade.

Hyperhemolysis is a life-threatening condition of exaggerated hemolysis of red blood cells which occurs in patients receiving chronic transfusion therapy. We present a 19-year-old male with the ß-thalassemia major with an episode of hyperhemolysis. Hemolysis was initially unresponsive to immunosuppression but responded after the addition of eculizumab. Several weeks after stabilization, hemolysis returned; which was also managed with immunosuppression and eculizumab. Hyperhemolysis presents significant challenges in ß-thalassemia due to the underlying dysfunctional erythropoiesis and transfusion dependence. Aggressive immunosuppression combined with eculizumab successfully slowed the hemolysis and allowed for the resumption of transfusions.

A Retrospective Review of Mercaptopurine Metabolism Reveals High Rate of Patients With Suboptimal Metabolites Successfully Corrected With Allopurinol.

Skewed drug metabolism of 6-mercaptopurine (6-MP) can jeopardize antileukemic effects and result in toxicities during the treatment of acute lymphoblastic leukemia and lymphoblastic lymphoma. Allopurinol can alter 6-MP metabolism to maximize therapeutic effects while reducing toxicities. Over 75% of our patients with acute lymphoblastic leukemia or lymphoblastic lymphoma experienced a 6-MP-related toxicity. Review of metabolite date a showed 6-methylmercaptopurine nucleotide levels were >10,000 in 55% of the cohort, suggesting 6-MP shunting. Allopurinol was initiated in 12 of 23 shunters with resolution of toxicities. We propose an algorithm to incorporate allopurinol into chemotherapy regimens for patients with inappropriate 6-MP metabolism.

Survival in Pediatric, Adolescent, and Young Adult Patients With Sarcoma in the Military Health System: Comparison With the SEER Population.

BACKGROUND: We sought to compare survival outcomes of sarcomas in the pediatric and adolescent/young adult populations with universal care access in the Military Health System (MHS) to those from the United States general population. METHODS: We compared data from the Department of Defense's (DoD) Automated Central Tumor Registry (ACTUR) and the National Cancer Institute's (NCI) Surveillance, Epidemiology, and End Results (SEER) program on the overall survival of patients 24 years or younger with histologically or microscopically confirmed sarcoma between diagnosed between January 1, 1987, and December 31, 2013. The Kaplan-Meier survival curves were used to compare survival between the 2 patient populations. Cox proportional hazard models were used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) comparing ACTUR relative to SEER. RESULTS: The final analysis included 309 and 1236 bone sarcoma cases and 465 and 1860 soft tissue sarcoma cases from ACTUR and SEER, respectively. Cox proportional hazards analysis showed soft tissue sarcoma patients in ACTUR had significantly better overall (HR=0.73, 95% CI=0.55-0.98) and 5-year overall (HR=0.63, 95% CI=0.46-0.86) survival compared with SEER patients, but no significant difference in overall or 5-year overall survival between ACTUR and SEER patients with bone sarcoma. CONCLUSION: Survival data from the ACTUR database demonstrated significantly improved overall survival for soft tissue sarcomas and equivalent survival in bone sarcomas compared with that reported by SEER.

Hemolytic anemia as a result of piperacillin/tazobactam administration: a case report and discussion of pathophysiology.

We report a case of a 19-year-old woman with cystic fibrosis who presented with hemolytic anemia during a course of piperacillin/tazobactam. The patient was initially managed with the replacement of blood products; however, she continued to show signs of hemolysis. Laboratories were obtained confirming antibody formation to piperacillin/tazobactam, and she was given a single infusion of intravenous immunoglobulin. Following the infusion, the patient did not require administration of any further blood products and achieved stable red blood cell counts. Early recognition of hemolytic anemia secondary to piperacillin/tazobactam with the administration of intravenous immunoglobulin may shorten the duration of hospitalization and quantity of blood products required for the stabilization of red blood cell counts.