Antiplatelet therapy in thrombotic thrombocytopenic purpura.
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Biomedical subjects
Publications and source records attributed to N G Slater.
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Histamine-releasing autoantibodies have been identified in chronic idiopathic urticaria. 8 patients with severe disease and histamine-releasing activity in their sera underwent plasmapheresis. Symptoms were abolished for 2 months in 1 patient and for 3 weeks in another, 2 showed almost complete resolution of symptoms, 2 had temporary relief, and the other 2 showed little change. Further investigation in 4 of the patients showed significantly reduced skin-test responses to fresh post-exchange autologous sera after plasmapheresis compared with stored pre-exchange sera, but the response to intradermal histamine remained unchanged. Blood cellular histamine increased as in-vitro serum histamine-releasing activity fell after plasmapheresis. These results favour a pathogenetic role for histamine-releasing autoantibodies in patients with chronic urticaria.
The haemodynamic effects of the venesection of one unit (450 ml) of blood over 9 min were measured using non-invasive techniques, in 14 healthy controls and 18 patients with coronary heart disease or hypertension. Venesection was associated with significant reductions in supine and standing systolic and diastolic blood pressure, stroke volume index and cardiac index, and increases in standing heart rate, in both patient groups. No significant differences were observed between the responses of subjects with and without cardiovascular disease. The authors conclude that, in contrast to established teaching, blood loss can be detected in its early stages by careful haemodynamic monitoring and that venesection can be performed safely without volume replacement in patients with stable cardiovascular disease.
We compared the plasma cell light chain ratios in the bone marrows of 13 patients with multiple myeloma (MM), with those of 13 patients with monoclonal gammopathy of undetermined significance (MGUS). The mean light chain ratio in favour of the paraprotein isotype in the myeloma group was 51.83 (95% confidence limits (CL) 29.52-74.14), while in the MGUS group it was 5.30 (CL 2.07-8.52). The difference between the MGUS and MM groups was significant (p = 0.0005). Neither the bone marrow plasma cell count nor the paraprotein level were significantly correlated with the light chain ratio in either of these two groups. We found a cut-off ratio of 8 to be the most useful in differentiating between myeloma and MGUS. Only one patient with myeloma had a ratio below 8, and one MGUS patient had a ratio above this cut-off point. We conclude that determination of the bone marrow plasma cell light chain ratio is a simple and useful test in differentiating between myeloma and MGUS in difficult cases.
Autologous blood transfusion--the transfusion to a patient of his or her own blood--is increasingly being recognised as a useful adjunct to traditional transfusion practice. The reasons include fears of disease transmission, the potential adverse effects on the recipient's immune system of transfused donor blood, and logistical factors. The three main techniques of autologous transfusion are: (i) pre-deposit, in which patients donate blood over a period of time in preparation for elective surgery; (ii) pre-operative isovolaemic haemodilution, in which blood is removed immediately before surgery and volume replacement is given, the blood being reinfused post-operatively; and (iii) salvage transfusion--the collection of blood shed at surgery or in similar circumstances, which is reinfused immediately or after concentration and purification. All three techniques can help to improve the safety of transfusion and economise on scarce supplies of donor blood.
Thrombotic thrombocytopenic purpura is a rare but serious haematological disease for which first-line therapy is medical, but not always successful. The role of splenectomy in the management of such patients is unclear. This paper reports three patients with the condition who went into remission following splenectomy after other forms of therapy had failed. Because thrombotic thrombocytopenic purpura is nearly always fatal if a remission is not obtained, splenectomy should be considered in patients who prove to be resistant to medical therapy.
Lymphocyte survival changes observed at 1, 2 and 3 days as responses to 3 doses of ionising radiation in vitro (40, 100, and 500 cGy) are analysed by computer according to a simple (single cell population) mathematical model. Intrinsic radiosensitivity, the susceptibility to lethal injury, which is expressed as the D37 value (the radiation dose permitting 37% survival), is estimated separately from the kinetics of subsequent death of lethally-irradiated cells (expressed as their half-life, or t1/2 value). Among the 35 patients with B-cell CLL studied (15 were never treated), both parameters varied widely and independently of one another. t1/2 ranged from 9-200 h and above, D37 from 14-500 cGy or above. Twenty-three patients were deemed 'radiosensitive' (D37 below 110 cGy). D37 level did not correlate with treatment status, mode of treatment, clinical staging (Rai) or lymphocyte count. With some exceptions, D37 remained relatively constant for individual patients with increasing duration of disease or alterations in treatment status. The assay method may prove useful as an aid in predicting response to low-dose splenic irradiation (SI) in CLL.
A direct chemiluminescence test (CLT) employing autologous monocytes and granulocytes was used to assay platelet-bound antibody in comparison to the direct platelet suspension immunofluorescence test (PSIFT) in 50 patients. Twenty-six of the 30 immune thrombocytopenic purpura (ITP) patients, including all 21 chronic ITP patients with platelet counts below 100 x 10(9)/l, showed positive results with at least one type of phagocytic cell; so did seven of the eight patients with other presumed autoimmune thrombocytopenias, and three out of 12 patients with thrombocytopenia associated with non-immune disorders. This test appears potentially valuable in the clinical diagnosis of immune thrombocytopenias.
Autologous blood transfusion--transfusion to the patient of his or her own blood--is of increasing importance in transfusion practice. There are three main techniques: pre-deposit, in which patients donate blood over a period of time in preparation for elective surgery; preoperative isovolaemic haemodilution, in which blood is removed in theatre immediately preoperatively and volume replacement is given, the blood being reinfused postoperatively; and salvage transfusion - the harvesting of blood shed at surgery or in similar circumstances, which is reinfused immediately or after concentration and purification. All these techniques can play a part in rendering transfusion safer and in economising on scarce supplies of donor blood.
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Demethoxydaunorubicin (DMDR), a new anthracycline available both for intravenous and oral administration, was given in 14 cases of leukaemia, non-Hodgkin's lymphoma (NHL) and multiple myeloma (MM) replacing either daunorubicin (DNR) or doxorubicin (DOX) in conventional chemotherapy regimes. In acute leukaemia (6 myeloblastic and 1 common lymphoblastic) there were 5 complete (CR) and 2 partial (PR) remissions; one patient, previously brought into remission with a regime including i.v. DMDR was thereafter maintained in CR with oral DMDR. Among the patients treated with the oral DMDR, 2 NHL cases were treated; 1 patient had a sustained remission of 12 months so far, with DMDR alone; another patient had a CR with a combined regime. In MM, one patient with very advanced disease treated with i.v. DMDR/CHOP did not respond, but three cases treated with oral DMDR plus other drugs showed a partial remission. Toxic effects were limited to brief episodes of nausea and vomiting in a few i.v. treated patients; a prolonged bone marrow depression was observed in one case only. No cardiotoxic effect was recorded.
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