Open reduction and internal fixation with precontoured distal humerus anatomical locking plate system is a good method of treatment for complex Supra- intercondylar fracture of distal humerus with good functional outcome and low rates of complications. Even though early results are promising, longer term investigations and larger patient groups are necessary to confirm the presented data.

The discovery that acquired TTP results from autoantibodies to ADAMTS-13, a von Willebrand factor-cleavage protein (vWF-CP), provided rationale for rituximab immunosuppression as an adjunct treatment. We previously reported our experience with 5 refractory TTP patients treated with rituximab (Ann Hematology 2005, 84:232). Rituximab was administered at 375 mg/m2 IV weekly for 4 weeks. Therapeutic plasma exchanges (TPE) were held at least 24 hours post rituximab. All patients attained durable remissions with a median response of 15 months (range: 10–21). Literature review revealed 11 other studies reporting longest response duration of 23 months. Since our initial report, we treated 5 additional patients with TTP: 1 refractory and 4 newly diagnosed. Of the previously reported 5 patients with refractory TTP, 3 were female and 2 were male patients with a median age of 37 years (27–70). All patients achieved complete responses (CR). The median duration of response has increased to 32 months with a follow up period of 25 to 51 months. The longest response has been sustained 44 months after the first dose of rituximab. One patient developed clinical relapse after 26 disease-free months. She achieved CR2 after another 4 doses of rituximab with continued response 12 months after re-treatment. Time to response was shorter with re-treatment, 4 weeks vs. 5 weeks at initial therapy. Our first male patient with refractory TTP who was treated in 4/02 remained in remission for 25 months until his death from unrelated causes. One additional male patient with refractory TTP was treated preemptively due to recurrent loss of vWF-CP activity after achieving initial response with TPE and steroids. Treatment resulted in reappearance of vWF-CP activity, with continued response 30 months from first dose of rituximab. Four newly diagnosed female patients were treated: 3 had idiopathic TTP, and 1 had associated SLE. Median age was 20 years (range: 16–30). The cohort had a median platelet count of 28×109/L (range: 11–56), median hemoglobin of 10g/dL (range: 6–12) and a median lactate dehydrogenase of 868 IU/L (range: 618–1737). The median TPE’s received prior to rituximab therapy was 6 (range: 4–9) and after initiation of rituximab was 17 (range: 12–19). All patients achieved CR with median time to response of 4.5 weeks (range: 3–8). Responses are maintained 13 to 27 months after initial rituximab dose. The patient with SLE attained a complete clinical response but developed persistently low platelets unrelated to her TTP. Among the 10 patients treated, decreased or absent vWF-CP activity with presence of inhibitor were noted in 6 patients, 3 had normal levels and 1 was not tested. Our experience suggests that rituximab is safe and effective in TTP patients with refractory and newly diagnosed disease. Time to response was similar among refractory and newly diagnosed patients. To our knowledge, 44 months is the longest remission duration reported in the literature among refractory TTP patients treated with rituximab. Extended follow up is necessary to determine durable remissions among patients with newly diagnosed disease.

No abstract

.Purpose. The most recent International Society for Heart and Lung Transplantation (ISHLT) biopsy scale classifies cellular and antibody-mediated rejections. However, there are cases with acute decline in left ventricular ejection fraction (LVEF ≤ 45%) but no evidence of rejection on biopsy. Characteristics and treatment response of this biopsy negative rejection (BNR) have yet to be elucidated. Methods. Between 2002 and 2012, we found 12 cases of BNR in 11 heart transplant patients as previously defined. One of the 11 patients was treated a second time for BNR. Characteristics and response to treatment were noted. Results. 12 cases (of 11 patients) were reviewed and 11 occurred during the first year after transplant. 8 cases without heart failure symptoms were treated with an oral corticosteroids bolus and taper or intravenous immunoglobulin. Four cases with heart failure symptoms were treated with thymoglobulin, intravenous immunoglobulin, and intravenous methylprednisolone followed by an oral corticosteroids bolus and taper. Overall, 7 cases resulted in return to normal left ventricular function within a mean of 14 ± 10 days from the initial biopsy. Conclusion. BNR includes cardiac dysfunction and can be a severe form of rejection. Characteristics of these cases of rejection are described with most cases responding to appropriate therapy.

No abstract