Thromboprophylaxis With Heparin During Orthotopic Liver Transplantation: Comparison of Hepcon HMS Plus and Anti-Xa Assays for Low-Range Heparin.

OBJECTIVES: The purpose of this study was to compare the agreement between two heparin assays, Hepcon HMS plus/Kaolin-ACT and Anti-Xa, and their predictive power in detecting circulating heparin levels post-reperfusion of the liver graft when compared with thromboelastogram (TEG) r time ratio in patients undergoing orthotopic liver transplantation (OLT). DESIGN: Prospective, observational cohort study design. SETTING: Single center, university hospital. PARTICIPANTS: Thirty-eight consecutive adults who had undergone liver transplant. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Paired arterial blood samples were collected before surgical incision, 5 minutes after administration of an average dose of 2,054±771 units of intravenous unfractionated heparin before caval cross-clamping, 5 minutes after portal reperfusion, 5 minutes after hepatic artery reperfusion, and 1 hour after hepatic artery reperfusion. The observations that heparin assay measurements were within the predetermined limits of agreement, strongly suggested the two heparin assays (Hepcon HMS plus and Anti-Xa assay) are interchangeable during prophylactic heparin dose therapy during OLT. Post-reperfusion, receiver operating characteristic curve analysis revealed high accuracy in measuring circulating heparin levels with both Anti-Xa and Hepcon HMS assays when compared with the TEG r time ratio assay. CONCLUSIONS: The point-of-care Hepcon HMS plus/Kaolin-ACT (activated clotting time) assay appeared to be a reliable alternative to the more expensive and laboratory-required Anti-Xa assay in monitoring the response to intravenous heparin in patients undergoing OLT.

Combined mycophenolate mofetil and prednisone therapy in tamoxifen- and prednisone-resistant Reidel's thyroiditis.

BACKGROUND: Mycophenolate mofetil is a recently identified therapy for disorders associated with systemic fibrosis, but has never been reported in the treatment of Reidel's thyroiditis. We report the first case of Reidel's thyroiditis that became resectable after treatment with mycophenolate and prednisone. SUMMARY: A 27-year-old woman presented to an outside hospital with thyromegaly associated with compressive symptoms. The patient underwent a neck exploration with thyroid biopsy that revealed evidence of fibrosing variant Hashimoto's thyroiditis. The patient was then treated with tamoxifen and prednisone at an outside hospital without resolution. After initial evaluation she underwent an open thyroid wedge biopsy that revealed Reidel's thyroiditis. She was subsequently treated with both 1 g mycophenolate twice daily and 100 mg prednisone daily. The patient experienced immediate subjective improvement of compressive symptoms and objective decrease in mass size at 30 days, as seen by serial computed tomography examination. By 90 days of therapy the mass had decreased to a size small enough to allow subtotal thyroidectomy, which was completed over two staged procedures. CONCLUSIONS: Reidel's thyroiditis remains a primarily surgical disease. Patients who are not surgical candidates have been treated with tamoxifen and prednisone with equivocal results. Our report is the first to suggest the combination of mycophenolate and prednisone as a viable treatment option for patients with Reidel's thyroiditis.

Comparison of two new classifications for pediatric myelodysplastic and myeloproliferative disorders.

BACKGROUND: The category, cytology, cytogenetics (CCC) system for myelodysplastic syndrome (MDS) and the pediatric WHO system for MDS/myeloproliferative disorder (MPD) have recently been proposed to characterize these diseases in pediatrics. OBJECTIVE: We compare the CCC and pediatric WHO systems against each other and against the French, American, British (FAB) and adult WHO classifications in order to determine which more accurately classifies these diseases and predicts outcome. METHODS: An 18-year retrospective review identified patients less than 18 years of age meeting CCC and/or pediatric WHO criteria for the diagnosis of MDS or MPD. Resolution, stability, progression, and death in the subcategories of each system were compared. RESULTS: Twenty-eight patients were included in the study. Pediatric WHO: 17 patients met criteria, 10 died. Eight developed acute myelogenous leukemia (AML) (seven died), one juvenile myelomonocytic leukemia (JMML) (died), one chronic myelomonocytic leukemia (CMML) (currently in relapse), two died of complications, two responded to BMT, three have stable disease, one resolved. Eleven patients were not classifiable by the pediatric WHO system, one of which progressed to AML and died. CCC: 26 patients met criteria, 9 died. Nine developed AML (8 died), 1 died of complications, 10 responded to treatment (BMT and/or chemotherapy). Four are stable without treatment, two resolved. Two patients with MPD were not classifiable by the CCC system. CONCLUSIONS: Both the pediatric WHO and CCC systems are better able to classify MDS in children than the adult WHO and FAB classifications. The pediatric WHO system is more exclusive. Children meeting these criteria are more likely to progress to AML or death. The restrictive nature of the pediatric WHO system was unable to classify one case of fatal MDS. The CCC system is more inclusive and can stratify patients into a neutral or poor prognosis based upon outcome. However, the CCC system ignores those diseases with a myeloprolifferative component. This resulted in two cases of MPD that were unclassifiable by the CCC system. One of these patients died, the other is currently in relapse.

Giant cell tumor of tendon sheath arising in the toe.

Giant cell tumor of the tendon sheath (nodular synovitis) is a benign soft tissue tumor, usually affecting older women, that most often occurs in the interphalangeal joints of the fingers. Occurrence is rare in children. We present a 12-year-old boy and a 6-year-old girl with giant cell tumors of tendon sheaths occurring in the toes. The tumor from the 12-year-old demonstrated the cytogenetic finding of t(1;2)(p13;q37), which previously has been associated with giant cell tumor of tendon sheath in adults. Giant cell tumors of the tendon sheath occurring in children have similar histological and cytogenetic features as adults, however, there may be a predilection for lower extremity involvement.