Amyloidosis Presenting as Severe Bleeding Diathesis

Bleeding is one of the rare presentations of Amyloidosis. The mechanism behind spontaneous or peri- interventional bleeding in patients of amyloidosis is complex and involves multiple co-existing factors like coagulation factor deficiency, abnormal synthesis of coagulation factors due to advanced liver dysfunction, acquired Von Willebrand disease, platelet dysfunction, amyloid angiopathy and other unknown mechanisms. We present a case of middle aged female, presenting with spontaneous retroperitoneal haemorrhage, on further investigations was found to have systemic amyloidosis and secondary severe factor X deficiency (2.7 % of normal by one stage factor assay method). Factor X deficiency (both inherited and acquired) is known to present with the most severe bleeding phenotype. The management option for such acute spontaneous haemorrhage is limited and mostly supportive in nature. Definitive treatment is directed towards the primary pathology and requires chemotherapy and hematopoietic stem cell transplantation.

Progress of platelets in liver and renal diseases / 白血病·淋巴瘤

Recent studies have shown that platelets have a variety of important functions, and play an important role in the pathophysiology of liver and renal diseases. The quantitative and functional changes of platelets, could affect the bleeding complications in patients with liver and renal diseases. This article reviews the progress of the effects of thrombocytopenia and platelet dysfunction on patients with liver and renal diseases, and the role of platelets in the pathophysiology of liver and renal diseases in the 58th American Society of Hematology Annual Meeting.

The role of point-of-care assessment of platelet function in predicting postoperative bleeding and transfusion requirements after coronary artery bypass grafting.

Objective: Objective platelet function assessment after cardiac surgery can predict postoperative blood loss, guide transfusion requirements and discriminate the need for surgical re‑exploration. We conducted this study to assess the predictive value of point‑of‑care testing platelet function using the Multiplate® device. Methods: Patients undergoing isolated coronary artery bypass grafting were prospectively recruited (n = 84). Group A (n = 42) patients were on anti‑platelet therapy until surgery; patients in Group B (n = 42) stopped anti‑platelet treatment at least 5 days preoperatively. Multiplate® and thromboelastography (TEG) tests were performed in the perioperative period. Primary end‑point was excessive bleeding (>2.5 ml/kg/h) within first 3 h postoperative. Secondary end‑points included transfusion requirements, re‑exploration rates, intensive care unit and in‑hospital stays. Results: Patients in Group A had excessive bleeding (59% vs. 33%, P = 0.02), higher re‑exploration rates (14% vs. 0%, P < 0.01) and higher rate of blood (41% vs. 14%, P < 0.01) and platelet (14% vs. 2%, P = 0.05) transfusions. On multivariate analysis, preoperative platelet function testing was the most significant predictor of excessive bleeding (odds ratio [OR]: 2.3, P = 0.08), need for blood (OR: 5.5, P < 0.01) and platelet transfusion (OR: 15.1, P < 0.01). Postoperative “ASPI test” best predicted the need for transfusion (sensitivity ‑ 0.86) and excessive blood loss (sensitivity ‑ 0.81). TEG results did not correlate well with any of these outcome measures. Conclusions: Peri‑operative platelet functional assessment with Multiplate® was the strongest predictor for bleeding and transfusion requirements in patients on anti‑platelet therapy until the time of surgery. Study registration: ISRCTN43298975 (http:// www.controlled‑trials.com/ISRCTN43298975/).