MANAGEMENT OF SPLENECTOMY FAILURES IN CHRONIC IMMUNE THROMBOCYTOPENIC PURPURA: ROLE OF ACCESSORY SPLENECTOMY

Abstract Accessory splenic tissue was demonstrated in four of eight patients with chronic immune thrombocytopenic purpura investigated following post-splenectomy relapses. Time from initial splenectomy to relapse of thrombocytopenia ranged from immediately to eight years and post-splenectomy changes were present on the peripheral blood films of all patients at time of relapse. Three scanning techniques were employed to demonstrate and localise residual splenic tissue. Conventional ^99mTc scans were positive in three of the four patients whilst ^99mTc-heat damaged red cell scans and computerised tomographic scans were each positive in three out of three patients including the one patient in whom a conventional ^99mTc scan was negative. Histological confirmation of splenic tissue was obtained in all cases with the weights of the accessory spleens ranging from 0.6 g to 2 g. Two patients responded to accessory splenectomy and, without immunosuppressive therapy, have remained well with normal platelet counts for over four years. There was no correlation between length of initial remission and the response to removal of the accessory spleen. The presence of a functioning accessory spleen should be considered in all patients with chronic ITP who fail to respond to, or relapse following, initial splenectomy. (Aust NZ J Med 1986; 16: 695–698.)     

PROTEOLYTIC DEGRADATION OF HIGH MOLECULAR WEIGHT KININOGEN IN ACUTE THROMBOTIC THROMBOCYTOPENIC PURPURA

Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by thrombocytopenia and schistocytic haemolytic anaemia. The majority of patients have normal coagulation parameters including the activated partial thromboplastin time (APTT). An intracellular cysteine protease, calpain, has been found in the plasma of many patients with acute TTP, and we hypothesize that it participates in the pathogenesis of the disorder. However, certain aspects of the disorder remain unresolved. For example, high molecular weight kininogen (HK) is one of the primary plasma inhibitors of calpain, and it also can act as a substrate for calpain. Consequently, one might anticipate that the HK could be defective or altered in TTP. In this report we describe the analysis of HK in plasma from five patients with acute TTP and following recovery. The HK was studied immunogenically and functionally. We observed that the HK in plasma samples from patients with acute TTP was proteolysed. This degradation was not observed in remission samples from the same patients. However, both acute and remission TTP samples had normal HK coagulant activity (acute samples, x  =0.84 ±0.26 U/ml; remission samples, x  = 0.89 ± 0.21 U/ml; control samples, x  = 0.87 ± 0.05 U/ml). Although the TTP plasmas were able to inhibit calpain activity, less inhibition activity was found in the acute samples (acute: mean inhibition 71 ±2.4%; remission: mean 92 ± 2.1%; control samples: mean 93 ± 5.4%: P <0.001). Normal HK treated with calpain also had reduced calpain inhibition capacity (mean 58%). This study suggests that although HK is proteolysed during acute TTP, the proteolysis occurs without a major loss in the coagulation function or depletion of the protease inhibitory activity of HK.     

慢性血吸虫病并发伤寒引起长期高热1例

慢性血吸虫病并发伤寒引起长期高热1例@熊思贤$江西省高安市血吸虫病防治站!高安330800~~     

INCIDENCE OF VERRUCOUS VEGETATIONS IN SYSTEMIC LUPUS ERYTHEMATOSUS. THE ASSOCIATION WITH ANTIPHOSPHOLIPID ANTIBODIES

Background: Valvular heart disease is prevalent in systemic lupus erythematosus (SLE) and is a common cause of morbidity and mortality. Valvular thickening and verrucous valvular vegetations (Libman–Sacks) are the most common lesions. The purpose of this prospective study was to analyze the incidence of Libman–Sacks vegetations in patients with SLE. Methods: Two hundred and seventeen consecutive patients (196 females, 21 males, aged 53 ± 11 years) with SLE were evaluated with M‐mode, 2D, and Doppler echocardiography. Libman–Sacks vegetations were defined as distinct localized masses of varying size and shape on the surface of the valve leaflets and exhibiting no independent motion. In 76 (35%) patients with SLE there were antiphospholipid antibodies (APL) present. Results: Thirty‐two (14.7%) patients with SLE had Libman–Sacks vegetations and in 21 (65.6%) of them there was a presence of antiphospholipid antibodies. In 22 patients the location of the masses was in the mitral, in 9 in the aortic, and in 1 in the tricuspid valve, whereas in 4 cases in BOTH valves. Valvular regurgitation was the predominant lesion in 18 mitral, 9 aortic, and 1 tricuspid, but only 2 cases had severe mitral regurgitation. Valvular stenosis was detected in 5 cases. Three patients had severe mitral stenosis and one of them died, while in the other two patients mitral stenosis was reversed with treatment of the underlying disease. The remaining two cases had severe aortic stenosis but one of them, also having severe mitral regurgitation, died suddenly. Conclusion: Our results show that Libman–Sacks vegetations are quite frequent in patients with SLE and are related to the presence of antiphospholipid antibodies. The most common lesion is valvular regurgitation and rarely valvular stenosis.     

ANTIBODIES TO INFLUENZA A IN A CLUSTER OF CHILDREN WITH JUVENILE CHRONIC ARTHRITIS

In this study of 41 patients with progressive juvenile chronicarthritis (JCA), born between 1946 and 1970, it was noticedthat 14 were born in the same year (1963). The possibility ofa common environmental factor was therefore investigated. Recordsshowed that an epidemic of influenza A H₂N₂ was present in thatyear, and the study shows that JCA patients born in 1963 stillhave a higher level of antibody to influenza A H₂N₂ than JCApatients born in other years or age-matched controls. This elevationis not seen in a survey of three control viruses. Since thisgroup developed their clinical JCA after the appearance of influenzaA H₂N₂ in 1977, it is suggested that these patients developeda progressive arthropathy because they had been pre-sensitizedto influenza A by contact with an earlier strain when in utero. KEY WORDS: Influenza A, Juvenile chronic arthritis, Birthdate, Clustering