Perioperative therapeutic plasma exchange in a patient with rare Factor XIII inhibitor.

INTRODUCTION: Factor XIII deficiency is a rare bleeding disorder which could be severe if inherited or less severe if acquired. We report a case of acquired Factor XIII inhibitor in a 75-year-old male with a suspicious left renal mass treated perioperatively with therapeutic plasma exchange (TPE). PATIENT AND METHOD: To perform kidney biopsy and ablation of the renal mass, six daily TPE treatments were performed before and after biopsy to minimize bleeding risk because the patient did not respond to drug therapy. Both thromboelastography (TEG) and laboratory-based coagulation tests were performed to assess coagulation status prior to and after TPE. RESULTS: The biopsy indicated oncocytoma which was removed by surgical procedure. Factor XIII activity remained below 15 % throughout TPE treatments, but Factor XIII inhibitor titer reduced from initial positive value of 1:40 to negative following the third TPE and remained negative through the sixth TPE. Unfortunately, the inhibitor titer was positive at 1:20 in the fifth month and 1:5 in the sixth month during follow-up. CONCLUSIONS: TPE is useful in removing XIII inhibitory factor, but the effects are only short term.

Severe complications in a 25-year-old male after brown recluse spider bite treated by therapeutic plasma exchange: A case report and review of other case studies.

Although in the majority of patients (90%), the bite wound of brown recluse spider resolves spontaneously, some patients may experience a severe reaction requiring hospitalization. A 25-year-old male developed severe hemolytic anemia, jaundice, and other complications following a brown recluse spider bite on his posterior right thigh. He was treated with methylprednisolone, antibiotics, and red blood cells (RBCs) transfusion without response. Therapeutic plasma exchange (TPE) was added to the treatment regimen, and his hemoglobin (Hb) was eventually stabilized, leading to significant clinical improvement. The beneficial effect of TPE in the current case was compared to three other reported cases. We recommend close monitoring of Hb levels in patients with systemic loxoscelism during the first week after brown recluse spider bite and early implementation of TPE in the management of severe acute hemolysis when patients do not respond to usual treatment modalities and RBC transfusion.

Concordance between i-STAT Contemporary Troponin I and Beckman High Sensitivity Troponin I Only when Both Tests Were Performed within Two Hours: A Preliminary Study.

OBJECTIVE: Often in the Emergency Department (ED) of our hospital, troponin I is measured by the i-STAT (contemporary troponin I) followed by analysis of high sensitivity troponin I (hs-TnI) using the Beckman analyzer in the clinical laboratory. In this study, contemporary troponin I concentrations obtained by the i-STAT were compared with the Beckman hs-TnI concentrations in patients with myocardial infarction. METHODS: Troponin I concentrations were determined by both methods in 56 specimens collected from 56 patients admitted to the ED (time between both measurements; <1 h to up to 16 h). RESULTS: When troponin I concentrations determined by the iSTAT-1 were repeated in the laboratory within 2 h, there was concordance between both values using standard regression analysis (y=1.14 x-0.56, n=18, r=0.98; hs-TnI values converted into ng/mL) as well as Passing-Bablock regression analysis (y=0.89 x-0.006). However, overall correlation when all 56 data points were considered was very poor. In addition, we also observed very poor correlation in another 38 specimens when laboratory determinations of hs-TnI were conducted >2 h up to 16 h. CONCLUSION: We concluded that the iSTAT-1 contemporary troponin I concentrations were concordant with hs-TnI values only if measured within 2 h.

Urine Protein Immunofixation Electrophoresis: Free Light Chain Urine Immunofixation Electrophoresis Is More Sensitive than Conventional Assays for Detecting Monoclonal Light Chains and Could Serve as a Marker of Minimal Residual Disease.

BACKGROUND: Immunoglobulin monoclonal light chains (MLCs) in serum and urine are markers for monoclonal gammopathy and could serve as markers of minimal residual disease (MRD) in multiple myeloma (MM). Excretion of MLCs in urine is known to result in renal damage and shorter survival in patients with LC-predominant MM. METHODS: Retrospective review of urine immunofixation in 1738 specimens at 3 medical centers was conducted to assess the utility of urinalysis for diagnosis and monitoring of monoclonal gammopathy. We tested 228 stored urine specimens via the modified urine immunofixation method, using antisera to assay free LCs (FLCs). RESULTS: Our review of urine immunofixation results and medical records validated the theory that the only meaningful value-added finding was detection of monoclonal free light chains. Examination of 228 urine specimens using our novel method revealed 18.4% additional positive results. The rate of incremental findings for lambda LCs was nearly 3-fold higher than for kappa LCs. CONCLUSIONS: The new method of urine immunofixation is significantly more sensitive and more efficient than the conventional method for detecting MLCs in urine. The new assay appears to be sensitive enough to prove that MLCs serve as a marker of MRD in MM.