Analysis of outcome of laparoscopic splenectomy for idiopathic thrombocytopenic purpura by platelet count

Laparoscopic splenectomy (LS) is now performed routinely in patients with idiopathic thrombocytopenic purpura (ITP) refractory to the medical treatment. Low preoperative platelet count was deemed to be a contraindication for a laparoscopic approach; however, there is no data reporting the outcome in those patients. We aimed to evaluate the influence of the preoperative platelet count on the operative and postoperative course and complication rate. Retrospective cohort study that was conducted in tertiary care university-affiliated medical center and included 110 consecutive patients who underwent LS. All patients were divided into three groups by their preoperative platelet counts: 50 x 10(9)/L (n = 80). The outcome and the influence of preoperative factors predictive of complications, blood transfusion, and length of stay were compared between the groups. Patients with a platelet count of 20 x 10(9)/L before surgery. Patients with counts >20 x 10(9)/L can safely undergo LS.     

Idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia in Hodgkin's disease

Idiopathic thrombocytopenic purpura (ITP) and Coombs' positive hemolytic anemia (AIHA) were found, respectively, in 5 (1%) and 1 (0.2%) of 492 patients with Hodgkin's disease (HD). 33 cases of ITP associated with HD reported in the literature are reviewed. Of our cases, ITP was coincident with the diagnosis of HD in 1 patient. In another patient ITP preceded the diagnosis of HD by 41 months and in the remaining 3 patients the diagnosis of ITP was established after they had been successfully treated for HD. A herpes zoster infection preceded ITP by 1 month in 1 patient and another had herpes zoster at the time of diagnosis of ITP. AIHA had preceded the diagnosis of HD by 8 months in 1 case. In patients with ITP the prognosis seems to be related only to the status of the underlying HD.     

Predictive factors of renal involvement or relapsing disease in children with Henoch-Schönlein purpura

Predictive factors of renal involvement or relapsing disease in children with Henoch-Schönlein purpura (HSP) are poorly known. We examined clinical and laboratory variables concerning HSP evolution in children hospitalized in the last decade to predict the possibility of renal complications or relapse occurrence. Periodic clinical evaluations with urinalysis were performed for 6±3.6 years. Simple logistic regression showed that persistent rash and severe abdominal pain at the onset of HSP are significantly related to renal involvement, while persistent rash is significantly related to relapsing disease. Multiple logistic regression showed that persistent rash remains related to renal involvement when adjusted for sex, age, and severe abdominal pain and conversely that severe abdominal pain is not related to renal involvement when adjusted for sex, age, and persistent rash. In conclusion, persistent rash over a period longer than 1 month seems to represent a significant predictor of renal sequelae and disease relapse in children with HSP.     

Antiphospholipid antibodies in adults with immune thrombocytopenic purpura

To determine the clinical significance of antiphospholipid antibodies (aPL) in patients with immune thrombocytopenic purpura (ITP), anticardiolipin (aCL) (IgG and IgM) and lupus anticoagulant (LA) were sought at diagnosis in 215 ITP adults with platelets <50 × 10⁹/l. aPL (aCL and/or LA) were detected in 55 patients (26%): aCL alone in 39 (18%), aCL and LA in 15 (7%) and LA alone in one (0·5%). LA was significantly associated with high IgG-aCL levels ( P  =   0·001). Among age, sex, initial platelet count, bleeding score, acute or chronic ITP outcome, only younger age was significantly associated with LA-positivity (mean age 29 ± 14 years vs. 45 ± 20 years, P  =   0·002). After a median follow-up of 31 months, 14/215 (7%) patients developed thrombosis (four arterial, 10 venous and/or pulmonary embolism); four of them (29%) had high aCL levels and LA. Multivariate analysis significantly associated thrombosis events only with age [hazard ratio (HR)   =   1·6; 95% confidence interval (CI): 1·2–2·4], LA (HR: 9·9; 95% CI: 2·3–43·4) or high IgG-aCL level (HR: 7·5; 95% CI; 1·8–31·5). Although the thrombosis rate was low, the significant associations between thrombosis and LA or high aCL level suggest that aPL should be tested at ITP diagnosis.     

Specific antiplatelet glycoprotein autoantibodies are associated with the thrombocytopenia of primary antiphospholipid syndrome

Thrombocytopenia is a frequent complication of primary antiphospholipid syndrome (PAPL) and has been attributed to antibodies directed against platelet glycoproteins (Gp) and also to antiphospholipid antibodies. We tested patients with PAPL with and without thrombocytopenia for specific antiplatelet autoantibodies. Platelet autoantibodies were detected by means of platelet immunoassays which included MAIPA with a panel of monoclonal antibodies directed against all the platelet Gps known to be possible targets for platelet autoantibodies. A high prevalence of serum platelet antibodies was found in patients with thrombocytopenia (73%, 11/15 patients) whereas antiplatelet antibody was detected in only one of the 10 control patients ( P  < 0.01). The antibodies mainly recognized GpIIbIIIa ( n  = 7), but also CD9 ( n  = 5), GpIaIIa ( n  = 4), GpIbIX ( n  = 3) and GpIV ( n  = 3). Platelet-Gp antibodies eluted from the platelet surface had the same reactivity as those found in the original sera from three of the four patients tested, whereas no anticardiolipin activity was found in the platelet eluates, suggesting the absence of cross-reactivity between anticardiolipin and antiplatlet antibodies. The MAIPA assay was also performed with F(ab')2 fragments obtained by pepsin digestion of serum IgG from four patients. The same results were obtained with F(ab')2 fragments and the original serum, demonstrating that platelet antibodies specifically bind in vivo to platelet Gps via their F(ab')2 fragments. Our results suggest a link between specific platelet antibodies and the thrombocytopenia of PAPL.     

Atypical presentations of thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) usually presents in a fulminant fashion with marked hematologic abnormalities often associated with fever and neurologic and renal abnormalities. Two cases of TTP are reported in which neurologic deficits were initially the dominant findings, followed weeks later by the pronounced hematologic manifestations. Young patients with unexplained neurologic findings, even if there is only minimal anemia or thrombocytopenia, should be monitored for subsequent manifestations of TTP so that treatment may be instituted promptly.     

Nine years of cascade filtration for thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) continues to attract physicians' attention because of its unpredictable course and underlying pathogenetic mechanisms. It is also attractive because of its optimal response to therapeutic plasma exchange (PE) even in the most severe cases. The usual approach to its treatment is conventional PE with plasma substitution and only recently fresh frozen plasma (FFP) has been substituted by cryodepleted or virally inactivated plasma with clinical results that are, if not better, at least comparable with the ones attained when FFP is employed. Nonetheless, no consensus exists regarding the optimal PE fluid and this is not of marginal interest as even after PE treatment mortality is still from 15 to 20%. On the contrary, some consensus exists on the pathogenetic relevance of the exceedingly large Von Willebrand (vWF) multimers whose presence parallels the clinical course and appears to be determined by the abnormalities in the production or function and survival of vWF-cleaving proteases which is auto-antibody mediated. In both cases plasmapheresis without plasma infusion is relatively ineffective, perhaps because it does not increase protease activity. Paradoxically, cascade filtration (CF) can produce the same favorable results without replacing any protease activity. As with CF, the replacement is the autologous plasma with approximately 20% levels of vWF, fibrinogen, fibronectin, IgM and circulating immune complexes and 75% of albumin, IgGs, AT III and proteins whose molecular weight exceeds 250-300000 Da. Our experience with CF for TTP began in 1994. Since then, 16 patients have undergone CF combined with decreasing amounts of FFP supplementation and since 1998 without any allogeneic FFP supplementation in 9 cases. Twenty-four patients (96%) treated with no or minimal amounts of FFP survived but four (16%) experienced from one to four recurrences associated with cutaneous, paradental, cholecystic and vaginal infections. Only one patient died (5%) after the second CF procedure. There were no untoward effects related to the procedure itself and up to 18 procedures in one patient were carried out over 16 months, 10 with the patient in her sixth month of pregnancy and four in the post partum period in preparation for a splenectomy. Remission was achieved after an average number of treatments (10.7 +/- 6.8); a result that compares favorably with those of our historical control group of 47 cases (14 +/- 13). The patient's exposure to allogenic plasma which was 10.8 +/- 4.6 plasma U/session was reduced to 0 in 10 patients, to 1.4 +/- 1.2 and 4.4 +/- 2.3 plasma U/session, respectively, for seven and nine patients receiving PE + CF and CF with same plasma supplementation as described in our previous article. Based on our experience, we believe CF is presently the optimal treatment for patients with classic, sporadic TTP.     

Randomized study of Helicobacter pylori eradication therapy and proton pump inhibitor monotherapy for idiopathic thrombocytopenic purpura

Helicobacter pylori (HP) eradication therapy is a useful treatment for idiopathic thrombocytopenic purpura (ITP). Some investigators have also reported the effects of proton pump inhibitor (PPI) monotherapy on ITP. We performed a randomized study of HP eradication therapy and PPI monotherapy on ITP. Four of nine patients achieved complete remission (CR), two of nine achieved partial remission (PR) in HP eradication therapy, three of eight achieved CR, and two of eight achieved PR in PPI monotherapy. No significant differences were observed in the CR + PR of these patients between HP eradication therapy and PPI monotherapy. As for cost comparisons, HP eradication therapy is cheaper than PPI monotherapy, but it is less effective.     

Efficacy of high-dose methylprednisolone as a first-line therapy in adult patients with idiopathic thrombocytopenic purpura

Fifty-seven adult patients with idiopathic thrombocytopenic purpura (ITP) were treated with either conventional-dose prednisolone (CDP) (1 mg/kg/d, 36 patients) or high-dose methylprednisolone (HDP) (30 mg/kg/d, 21 patients), as first-line treatment. Patients in the HDP arm responded more rapidly (4.7 v 8.4 d), with a higher response rate (80% v 52.7%), and without severe side-effects. One quarter of the patients (3/12) who were non-responsive to CDP achieved complete remission when they were treated with HDP. The findings suggest that HDP may be a more effective first-line treatment than CDP for adult ITP, and it may also be preferred for life-threatening cases of ITP. However, these results must be confirmed by a randomized study prior to any change in the current practice of employing CDP as first-line treatment for adult ITP.     

Autoimmune-prone (NZW × BXSB)F1 (W/BF1) mice escape severe thrombocytopenia after treatment with deoxyspergualin, an immunosuppressant

Summary. Male (NZW × BXSB)F₁ mice spontaneously develop a disease which closely resembles human systemic autoimmune disease, involving idiopathic thrombocytopenic purpura and glomerulonephritis. We investigated whether autoimmune thrombocytopenia in the mice responded to deoxyspergualin, as immunosuppressant. Deoxyspergualin completely prevented the development of thrombocytopenia and suppressed the increase in circulating autoantibodies against platelets. This agent also ameliorated lupus nephritis. These findings suggest that deoxyspergualin may be effective in the prevention of idiopathic thrombocytopenic purpura.