Basic characteristics and safety of donation in related and unrelated haematopoietic progenitor cell donors - first 10 years of prospective donor follow-up of Swiss donors.

Since July 2007 prospective life-long follow-up (FU) for unrelated (URD) and related donors (RD) is mandatory in Switzerland and data on every allogeneic haematopoietic progenitor cell (HPC) donation are collected prospectively. We report the real-world experience of HPC donation during a 10-year study period (01.07.2007-30.06.2017) with basic characteristics and FU data. 1105 donors underwent 1155 HPC donation procedures. Eighty percent of first donations performed by 802 (73%) RDs and 303 (27%) URDs were peripheral blood stem cells (PBSC), 20% bone marrow (BM). Male donors were over-represented as URD (60% male vs 40% female). Main differences between RDs and URDs concerned age and pre-existing health disorders. RDs were significantly older at first donation (median age 48 years) compared to URD (34 years, p < 0.0001) and had more pre-existing health problems: 25% vs 9% in URD (p < 0.0001). No fatal complications occurred, collection related severe adverse events (SAE) after first donation were not significantly different between groups (RD 1.2%, URD 0.99%), incidence rates for neoplastic and autoimmune diseases did not exceed the rates of the general population. RDs are a more heterogeneous and potentially more vulnerable group, but if donor evaluation is performed appropriately, HPC donation is still safe.

[Hemostasis parameters in 55 patients with venous and/or arterial thromboembolisms]. / Hämostaseparameter bei 55 Patienten mit venösen und/oder arteriellen Thromboembolien.

55 consecutive patients (28 males and 27 females) who had suffered from (recurrent) venous and/or arterial thromboembolism were evaluated for laboratory findings of thrombophilia. At the time of investigation, 15 patients were taking oral anticoagulants. In addition to patient and family history and clinical examination, a coagulation profile, euglobulin clot lysis time before and after venous occlusion, assays of plasminogen, antithrombin III, protein C (PC), free protein S (PS[f]), and C4b binding protein bound protein S (PS[b]) were obtained. 2 patients not orally anticoagulated had partial PD deficiency with functional PC activity values of 53% and 57% respectively, as compared to normal human plasma (NHP). Functionally active PS(f) was found to be lower than normal in 14 patients not taking oral anticoagulants and without signs of hepatopathy. In 4 of these 14 patients partial PS deficiency was present (PS[f] between 7 and 37% of NHP, and PS[b] between 51 and 86% of NHP). In one of these 4 subjects the hereditary nature of PS deficiency was proven by investigation of family members. In 3 of the 14 patients a shift from PS(f) (between 34 and 66%) towards PS(b) (124-141%) was found. The remaining 7 patients showed subnormal PS(f) values (56-64%) and normal PS(b) values (78-105%). In the patients receiving oral anticoagulant therapy, PC and PS levels were diminished. Statistically there was no close relationship between PC and PS levels on the one hand and prothrombin time values on the other. In anticoagulated patients, therefore, PC or PS deficiency can only be diagnosed by investigation of members of the propositus's family.(ABSTRACT TRUNCATED AT 250 WORDS)

[Measurement of bleeding time and study of thrombocyte aggregation. Standardization of methods, normal values and results in patients with suspected hemorrhagic diathesis]. / Messung der Blutungszeit und Untersuchung der Thrombozytenaggregation. Standardisierung der Methoden, Normalwerte und Resultate bei Patienten mit Verdacht auf hämorrhagische Diathese.

Bleeding time measurement and investigation of platelet aggregation in platelet rich plasma (PRP) are routine procedures for the diagnosis of defects in primary hemostasis. These tests are subject to methodological difficulties and should be well standardized in each individual laboratory. - In the present study, bleeding time was measured using the Simplate II device in 40 normal subjects. Furthermore, platelet aggregation in PRP induced by ADP, collagen, arachidonate, and ristocetin was examined. 26 patients referred for investigation of a suspected mild bleeding disorder, who had a normal plasmatic coagulation profile, a normal von Willebrand factor activity, and a normal platelet count, were similarly studied. - Based on the reference values established in the 40 normal subjects, platelet aggregation was found to be pathologic in 7 patients and normal in 12. In 7 patients platelet aggregation was considered to be borderline-pathologic as defined by the range of platelet aggregability found in the 10% of our normal subjects showing the weakest aggregation responses. Bleeding time was prolonged in only 3 patients whereas it was normal in the remaining 23. There was strong evidence of a hemostatic defect as assessed by systematic patient history in 6 out of 7 patients with pathologic platelet aggregation, but in only 3 out of 19 showing normal or borderline-pathologic aggregation. - Pathologic platelet aggregation, therefore, represents not only an abnormal laboratory finding but is likely to be associated with a hemorrhagic diathesis. Platelet aggregation studies do not permit etiologic diagnosis of the thrombocytopathy except for the well-defined membrane glycoprotein deficiencies. The bleeding time appeared to be of low sensitivity for the diagnosis of mild platelet dysfunction.