Heterozygous plasminogen deficiency was found in 2 patients (mother and daughter). The mother, aged 55 years, was symptomatic while the daughter, aged 10 years, was asymptomatic so far. The thrombotic tendency presented by the proposita (mother) was severe and included recurrent superficial, portal, mesenteric, subclavian thrombophlebitis. No arterial thrombosis was noted. Oral anticoagulants have been of some benefit. The main laboratory features were: plasminogen activity about 50% of normal in two amidolytic methods and in a caseinolytic method. Plasminogen antigen was also about 50% of normal using electroimmunoassay and radial immunodiffusion. Crossed immunoelectrophoresis revealed a normal, even though reduced pattern, thus excluding dysplasminogenemia. Routine coagulation tests were negative. Euglobulin lysis time, fibrinogen level and fibrinogen degradation products (FDP) were within normal limits. Antithrombin III, protein C and protein S were also within normal limits.

Familial persistent atrial standstill (PAS) has been rarely documented. Five patients, three male and two female (mean age 46 years at first observation), with familial 'complete' PAS, all from a small mountain community (900 residents) have been studied. Diagnostic criteria were: absence of P wave in any lead of the standard electrocardiogram; no electrical activity of the atria with extremely slow junctional escape rhythm on the endocavitary recordings; lack of atrial excitability; absence of atrial wall movement at fluoroscopy; no mitral A wave on the echocardiogram. All patients had marked cardiac enlargement primarily due to atrial enlargement and all had impaired functional class. Some had bradycardia for many years but none had syncope. Permanent cardiac pacing was carried out in all. Two had cerebral embolism. In the same community there were three patients, one male and two female (mean age 34 years at first observation), with 'partial' atrial standstill characterized by absence of P waves in any lead of the standard electrocardiogram and by endocavitary recording of electrical activity limited to a localized region of the atrium. These three patients also had cardiac enlargement primarily due to atrial enlargement but to a lesser degree than the patients with complete PAS. One patient had cerebral embolism. The familial and endemic character of the disease is stressed. Cardiac enlargement due primarily to atrial enlargement seems to be a common feature of both the complete and partial form of the PAS syndrome.

A family with a new congenital abnormality of antithrombin III (AT III) is presented. 5 members, all females, were affected. The proposita has had several thrombotic manifestations. The other patients, so far, are asymptomatic. Antithrombin activities were all decreased regardless of the method used (chromogenic or clotting) and regardless of the presence or absence of heparin in the assay system. AT III antigen, on the contrary, was normal in all patients regardless of the method used (electroimmunoassay, radial immunodiffusion or Laser nephelometer). The crossed immunoelectrophoresis without heparin showed in plasma the presence of an abnormal peak which was more anodal than the normal counterpart. The same pattern was seen in serum. In the heparin-modified cross-immunoelectrophoresis a normal pattern was seen in plasma and an abnormal one in serum. In the latter the anodal peak was in fact larger than the normal counterpart. Chromatographic studies using Heparin-Sepharose column failed to show changes in heparin affinity, and indicated that both the normal and the abnormal peak were contained in the major AT III area. The toponym AT III Trento is proposed to describe this abnormality. These studies further emphasize the great heterogeneity of AT III defects. This is the first AT III abnormality to show an abnormal crossed-immunoelectrophoresis in the absence of heparin.

New systems for collection of platelet concentrate (PC) and platelet poor plasma (PPP) are presently available. The aim of our work was to test the possibility of preparing PC routinely from normal plasma donors in a minimum amount of time and, at the same time, providing a second product that can be used as source-plasma or fresh-frozen plasma. Over a 3 year period (from 1990 to 1992) we performed 3503 procedures using 2 Haemonetics PCS machines (1236 procedures) and 3 Autopheresis-C (2267 procedures). With the PC produced we were able to satisfy all the requests coming from the hospitals of our region. The platelet yield was 1.95 x 10(11) with PCS and 3.2 x 10(11) with Auto-C in a PC volume of 150 and 200 ml respectively; collection times were quite similar (56 and 63 min). The results show that plasma-plateletpheresis is an efficient and competitive system. Regarding platelet yield, the best results were obtained with the Auto-C.