Chronic opioid therapy for pain in sickle cell disease (SCD) carries risks of tolerance, hyperalgesia, and morbidity. Buprenorphine offers a safer alternative, yet optimal induction strategies for SCD patients dependent on full agonists remain unclear and traditionally require mild withdrawal prior to initiation. Three adult SCD patients transitioned from full opioid agonists (morphine equivalent daily dose 90-410 mg) to buprenorphine using a cross-titration strategy stratified by baseline opioid use to avoid withdrawal and maintain analgesia. These cases support an individualized buprenorphine induction approach that continues full agonists, expanding strategies to improve tolerability and transition success for adults with SCD.

Lupus mesenteric vasculitis (LMV) is a rare but potentially life-threatening complication of systemic lupus erythematosus, with reported mortality rates of up to 13.4% despite aggressive immunosuppression. Standard management relies on high-dose glucocorticoids (GCs) combined with cyclophosphamide (CYC) or mycophenolate mofetil (MMF); however, prolonged glucocorticoid dependence and disease recurrence remain significant clinical challenges. Telitacicept, a recombinant transmembrane activator and calcium-modulating cyclophilin ligand interactor (TACI)-Fc fusion protein that simultaneously neutralizes B-lymphocyte stimulator and a proliferation-inducing ligand, has demonstrated efficacy in active SLE, yet evidence for its use in LMV is limited to a single case report. We describe a 27-year-old female with severe, glucocorticoid-dependent LMV complicated by autoimmune hemolytic anemia (AIHA), who received pulse methylprednisolone (MP) and intravenous immunoglobulin (IVIG) for acute induction, followed by telitacicept 160 mg weekly as part of a combination maintenance regimen. After conventional induction, telitacicept was associated with successful prednisone tapering from 60 mg/day to 10 mg/day over approximately 6 months without disease flare, accompanied by sustained normalization of hemoglobin and complement levels, and radiological improvement. This case represents the second report of telitacicept for LMV and the first to document sustained resolution of AIHA maintained during telitacicept-containing therapy. The induction with pulse MP followed by telitacicept maintenance and accelerated steroid taper" strategy may offer a promising therapeutic option for refractory LMV warranting further prospective investigation.

Glycated albumin (GA), the product of nonenzymatic glycation of serum albumin, reflects the mean glucose levels over the preceding approximately 2 weeks, bridging the gap between daily self-monitoring of blood glucose and the roughly 3-month window covered by glycated hemoglobin (HbA1c) measurement. First quantified in the early 1980s, GA was long overshadowed by HbA1c, but at present, interest has revived for two reasons. First, HbA1c assessment proves unreliable when the erythrocyte lifespan or hemoglobin glycation is perturbed, as in patients with renal failure, hemolytic anemia, hemoglobin variants, and iron deficiency. Second, continuous glucose monitoring has drawn attention to postprandial excursions and shortterm glucose variability that are not captured by HbA1c. This review revisits GA's biology, measurement, standardization, clinical utility, limitations, and evidence linking it to diabetic complications. Because it responds within 2 weeks to treatment and is markedly influenced by postprandial glucose excursions, GA supports early monitoring of therapy, and its ratio to HbA1c serves as an index of postprandial glycemia and insulin secretion. However, it must also be recognized that conditions that alter albumin turnover change GA levels independently of blood glucose levels. The prognostic value of GA is strongest in patients on maintenance hemodialysis, although recent studies in various cohorts and a meta-analysis have expanded situations in which GA measurement is of prognostic value. New measurement approaches, including finger-prick sampling, weekly home measurement, and noninvasive saliva and tear assays, point toward a renewed role for GA measurement in everyday glucose monitoring in individuals with diabetes.

Thrombotic thrombocytopenic purpura (TTP), the most common type of which is immune-mediated (iTTP), is a rare, life-threatening hematologic disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and end-organ damage. Prompt recognition and early treatment are essential for survival. Early diagnosis and initiation of treatment are critical in iTTP. Nurses play a key role in recognizing symptoms, supporting patients through treatment, and monitoring for relapse. This review outlines the pathophysiology and clinical presentation of iTTP, along with criteria for diagnosis, with an emphasis on the role of the von Willebrand factor-cleaving protease ADAMTS-13. Current evidence-based treatment strategies are discussed, including therapeutic plasma exchange and immunosuppression with corticosteroids and monoclonal antibody-based therapies. A case study of a middle-aged patient who experienced a relapse of iTTP is presented, illustrating key clinical decision points, challenges in long-term management, and the need for interdisciplinary collaboration.

Sickle cell disease (SCD) is a debilitating hereditary hematologic disorder characterized by severe physical complications, including painful vaso-occlusive crises, chronic pain, multi-organ damage, and reduced life expectancy. Diverse medications are prescribed to treat SCD or manage its associated complications. However, no comprehensive resource for concomitant medication and drug-drug interaction (DDI) risk exists for people living with SCD. Here, we utilized real-world claims data from the Merative Commercial MarketScan Research Database to identify the 100 most prescribed drugs for adolescents and adults living with SCD in the United States with commercial health insurance. DDI risks were assessed by leveraging the CERTARA Drug Interaction Database. In total, 43/100 drugs on the list were associated with DDI risks. Inhibitors were more frequently represented than inducers, suggesting that interaction risk in this population may be driven primarily by inhibition-mediated mechanisms. CYP3A4 accounted for the largest proportion of identified interactions. The high prescription rates of the CYP3A4 substrates oxycodone (40%) and fentanyl (18.5%), associated with potentially serious clinical consequences due to DDIs, highlight the importance of considering CYP3A4 interaction risk during development of novel therapies for SCD. Overall, this analysis provides a population-level insight into prescription patterns and subsequent DDI risks for people living with SCD. We expect these findings to (1) create clinical DDI risk awareness for this population and (2) inform strategic planning of DDI assessments during development of novel therapies indicated for SCD.

The once-weekly combination of rifapentine and isoniazid (3HP regimen) is a preferred treatment for latent tuberculosis infection (LTBI). Although generally well tolerated, rare but severe systemic adverse reactions may occur. We report the case of a 57-year-old woman receiving once-weekly rifapentine, isoniazid, and pyridoxine for LTBI who developed recurrent chest pain, generalized weakness, nausea, and vomiting within a short time after taking each weekly dose. The evaluation demonstrated multiorgan involvement, including acute liver injury, hyperbilirubinemia, hyperferritinemia, markedly elevated lactate dehydrogenase (LDH), undetectable haptoglobin, thrombocytopenia, and acute kidney injury. An extensive evaluation for infectious, cardiac, hematologic, and hepatic etiologies was unrevealing. The temporal relationship to medication administration, recurrence with re-exposure, and improvement following discontinuation of the rifapentine and isoniazid regimen supported a diagnosis of severe drug-induced hypersensitivity with hepatic, hematologic, renal, and systemic inflammatory involvement. The patient was managed with supportive care, resulting in progressive improvement of laboratory abnormalities, although renal recovery remained incomplete at discharge. This case highlights a rare but clinically significant multiorgan adverse reaction associated with the 3HP regimen. Clinicians should maintain a high index of suspicion for drug-induced hypersensitivity in patients who develop recurrent systemic symptoms after dosing, as early recognition and prompt discontinuation of therapy may prevent significant morbidity.

Hereditary thrombotic thrombocytopenic purpura (hTTP) is a thrombotic microangiopathy due to severe ADAMTS13 deficiency. hTTP typically presents in adulthood with thrombocytopenia, hemolytic anemia, and/or neurologic abnormalities. However, nearly half of the patients with hTTP are symptomatic at birth, most commonly presenting as severe jaundice secondary to hemolysis. Turbulent blood flow during birth puts neonates at high risk for thrombotic events and subsequent hemolytic anemia. We present a case series of 3 siblings with ADAMTS13 loss-of-function variants, all of whom required neonatal exchange transfusion. These siblings are now in their 30s and were diagnosed with hTTP between the ages of 13 and 18. One sibling experienced a neonatal stroke, while another sibling required a leukemia workup in her childhood because of hTTP. They remain in good health as adults. Two of them receive recombinant ADAMTS13 treatment for their deficiency. A recent report found that only 5% of patients with hTTP were correctly diagnosed at birth-underscoring the importance of having a high index of suspicion in the neonatal period for the anemic and thrombocytopenic newborn with nonimmune hemolytic anemia. With newly Food and Drug Administration-approved recombinant ADAMTS13 therapy and rapid diagnostic testing for ADAMTS13 levels, it is imperative that pediatricians and neonatologists recognize this rare but treatable form of recalcitrant jaundice. We propose a diagnostic algorithm to assist clinicians in neonatal diagnosis of hTTP in hopes of mitigating the need for exchange transfusions and extensive hematologic workups later in life. Timely diagnosis would prompt targeted treatment, which can prevent long-term neurologic and renal sequelae.