SummaryLiposomes of pure phospholipids were used in a modified APTT test system and the role of phosphatidyl serine (PS) in determining the sensitivity of the test system to the presence of lupus anticoagulants was assessed. Six consecutive patients with lupus anticoagulants and seven haemophiliacs with anticoagulants directed at specific coagulation factors, were studied. Increasing the concentration of phospholipid in the test system markedly reduced the sensitivity to lupus anticoagulants but had marginal effect on the specific factor inhibitors. The same effect was achieved when the content of PS alone was increased in a vehicle liposome of constant composition.The results suggest that the lupus anticoagulants can best be detected by a screening method using an APTT test with a reagent of low PS content. The use of a reagent rich in PS will largely abolish the lupus anticoagulant’s effect on the APTT. An approach using the two different types of reagent may facilitate differentiation of lupus inhibitors from other types of anticoagulant.

A 68-year-old previously well woman developed sudden onset of limb gangrene in association with liver dysfunction. An immediately acting inhibitor to factor V with some of the features of lupus anticoagulant was demonstrated. The patient required limb amputation within 2 weeks and activity of the anticoagulant seemed to be on the decline 6 months later.

No abstract

No abstract

No abstract

SUMMARY A retrospective study of 211 bone marrow aspirates from patients with chronic myeloid leukaemia (CML) was undertaken to assess the incidence ofGaucher cells and sea-blue histiocytes. A significant correlation between the presence of these cells and prolonged survival was seen. Such storage histiocytes occurred most often during periods of relapsed chronic phase. This study shows that Gaucher cells and sea blue histiocytes are a common feature ofCML and that their accumulation seems to be associated with a prolonged increase in leucocyte turnover.

Factor XI1 plays a part in both the contact activation of blood coagulation and in fibrinolysis (Murano 1978). It is known that deficiency of Factor XI1 does not usually lead to a bleeding diathesis although a few cases have been reported with a mild haemorrhagic tendency. An association between this deficiency and both venous and arterial thromboembolism has been the subject of several reports (Ratnoff 1980) but a definite correlation remains unproven. Case historyA 47 year old Pakistani male with no history of unusual bleeding presented with a 3 year history of angina pectoris and previous myocardial infarction. Coronary angiography was carried out demonstrating severe triple vessel coronary disease and impaired left ventricular function (ejection fraction < 30%). Coronary artery bypass grafting was recommended and a routine pre-operative coagulation screen revealed the following: prothrombin time 14 s (Control 13.5); APTT 110 s (Control 34); thrombin time 18.5 s (Control 20); fibrinogen 2.75 g/dl; whole blood clotting time 8.5 min; euglobin clot lysis time >360 min; inhibitor screening negative. Full blood count and platelet count were normal. Further investigation revealed the following: factor XI1 < 1.0 u/dl; factor VIII 240 u/dl; factor IX 68 u/dl; factor XI 100 u/dl. The glucose tolerance test and the lipoprotein electrophoresis were normal. The other lipid parameters were: total cholesterol 6.2 mmol/l (normal range 3.6-7.2); triglycerides 1.7 mmol/l (normal range 0.8-1.8); HDL-cholesterol 1.18 mmol/l (normal range 0.7-1.65); LDLcholesterol 3.93 mmol/l (normal range 2. 27-5.19).At operation both the right and left ventricles showed extensive diffuse scarring. There was severe coronary atheroma and massive, non-coronary, collateral circulation. Triple coronary artery bypass grafting was carried out. Before cannulation the patient was anticoagulated with 4 mg/kg of heparin.

Although hairy cell leukaemia was first described 40 years ago, it is only in the last decade that newer therapeutic agents have enabled effective treatment. The purine nucleoside analogue, 2-chlorodeoxyadenosine (2-CdA) is currently considered as first-line therapy with a very high rate of complete remission. Although adverse events with 2-CdA are increasingly recognized, severe cutaneous reactions have been reported rarely. We describe two consecutive patients treated with 2-CdA for hairy cell leukaemia who both suffered extremely severe cutaneous reactions, one of which was life-threatening.