Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality.

While platelets are primary mediators of hemostasis, there is emerging evidence to show that they may also mediate pathologic thrombogenesis. Little data are available on risks and benefits associated with platelet transfusions in thrombotic thrombocytopenic purpura (TTP), heparin-induced thrombocytopenia (HIT) and immune thrombocytopenic purpura (ITP). This study utilized the Nationwide Inpatient Sample to evaluate the current in-hospital platelet transfusion practices and their association with arterial/venous thrombosis, acute myocardial infarction (AMI), stroke, and in-hospital mortality over 5 years (2007-2011). Age and gender-adjusted odds ratios (adjOR) associated with platelet transfusions were calculated. There were 10 624 hospitalizations with TTP; 6332 with HIT and 79 980 with ITP. Platelet transfusions were reported in 10.1% TTP, 7.1% HIT, and 25.8% ITP admissions. Platelet transfusions in TTP were associated with higher odds of arterial thrombosis (adjOR = 5.8, 95%CI = 1.3-26.6), AMI (adjOR = 2.0, 95%CI = 1.2-3.3) and mortality (adjOR = 2.0,95%CI = 1.3-3.0), but not venous thrombosis. Platelet transfusions in HIT were associated with higher odds of arterial thrombosis (adjOR = 3.4, 95%CI = 1.2-9.5) and mortality (adjOR = 5.2, 95%CI = 2.6-10.5) but not venous thrombosis. Except for AMI, all relationships remained significant after adjusting for clinical severity and acuity. No associations were significant for ITP. Platelet transfusions are associated with higher odds of arterial thrombosis and mortality among TTP and HIT patients.

Predictive factors for successful laparoscopic splenectomy in immune thrombocytopenic purpura: study of clinical and laboratory data.

BACKGROUND: Laparoscopic splenectomy (LS) offers better short-term results than open surgery for the treatment of immune thrombocytopenic purpura (ITP), but long-term follow-up is required to ensure its efficacy. The remission rate after splenectomy ranges from 49 to 86% and the factors that predict a successful response to surgical management have not been clearly defined. The goal of this study was to determine the preoperative factors that predict a successful outcome following LS. METHODS: From February 1993 to December 2003, LS was consecutively performed in a series of 119 nonselected patients diagnosed with ITP (34 men and 85 women; mean age, 41 years), and clinical results were prospectively recorded. Postoperative follow-up was based on clinical records, follow-up data provided by the referring hematologist, and a phone interview with the patient and/or relative. Univariate and multivariate analyses were performed for clinical preoperative variables to identify predictive factors of success following LS. RESULTS: Over a mean period of 33 months, 103 patients (84%) were available for follow-up with a remission rate of 89% (92 patients, 77 with complete remission with platelet count > 150,000). Eleven patients did not respond to surgery (platelet count < 50,000). Mortality during follow-up was 2.5% (two cases not related to hematological pathology and one case without response to splenectomy). Preoperative clinical variables evaluated to identify predictive factors of response to surgery were sex, age, treatment (corticoids alone or associated with Ig or chemotherapy), other immune pathology, duration of disease, and preoperative platelet count. In a subgroup of 52 patients, we also evaluated the type of autoantibodies and corticoid doses required to maintain a platelet count > 50,000. Multivariate analysis showed that none of the variables evaluated could be considered as predictive factors of response to LS due to the high standard error. CONCLUSION: Long-term clinical results show that LS is a safe and effective therapy for ITP. However, a higher number of nonresponders is needed to determine which variables predict response to LS for ITP.

Prognostic factors of severe infectious purpura in children.

The French Club of Pediatric Intensive Care has prospectively studied 90 cases of infectious purpura which were hospitalized in 1981; the purpose of this study was to determine prognostic factors. The statistical study (X2 test) of all these cases is in agreement with data in the literature and shows that the mortality is significantly higher when there is: shock (p less than 0.001), coma (p less than 0.05), ecchymotic or necrotic purpura (p less than 0.01), temperature less than 36 degrees C (p less than 0.05), no clinical meningism (p less than 0.001), white cell count less than 10,000/mm3 (p less than 0.05), thrombocytopenia less than 100,000 (p less than 0.01), fibrinogen less than 1.5 g/l (p less than 0.001), kalemia greater than 5 mEq/l (p less than 0.01), spinal fluid cell count less than 20/mm3 (p less than 0.01). Because shock is one of the main prognostic factors (23 deaths in 55 shocked patients, versus 2 in 35 non-shocked) we have performed another statistical study (with the Benzecri method) to determine a prognostic index for patients in shock. For its determination, five initial parameters are used: age, kalemia, white cell count, clinical meningism, platelet count. The predictive value for survival is 91%. The predictive value for death is 87%. The score was applied on the patients hospitalized in shock in 1982: the predictive value for survival is 75%, the predictive value for death is 61%.

Onyalai at Rundu, Namibia 1981-1988: age, sex, morbidity, mortality and seasonal variation of 612 hospitalized patients.

Of 51,263 admissions to Rundu State Hospital in Namibia between 1981 and 1988, 612 (1.19%) were diagnosed as onyalai. The annual incidence varied between 0.96% and 1.66% of all admissions. The female to male ratio was 3:2. The mean age at presentation was 24.8 years (range 6 months to 80 years) and the mean hospital stay (and duration of clinical bleeding) for the years 1981 to 1982 and 1985 to 1988 was 7.68 d (range 1-38 d). Although the highest number of cases occurred during the months March, April and May a statistically significant monthly variation was not found. The treatment policy of commencing intravenous fluid on admission and a blood transfusion whenever the haemoglobin dropped below 10 g/dl in patients with active bleeding was associated with a mortality rate of 2.78% compared to 9.8% in cases recorded up to 1981.

Idiopathic thrombocytopenic purpura in the older adult patient.

Forty adults with idiopathic thrombocytopenic purpura (ITP), aged over 45 years, were seen from March 1954 to December 1983 at the Medical College of Georgia. All patients had bleeding manifestations at presentation. Twenty-one of 40 (52.5%) during the follow-up period had either life-threatening or fatal bleeding episodes. There were no significant differences for the presence of any presenting clinical or laboratory feature for patients who achieved a complete remission compared with those who did not. A complete response to therapy, younger age, higher presenting hemoglobin level, and absence of central nervous system bleeding favorably influenced overall survival. Therapy was ineffective in this age group, with only 12 patients (30%) achieving a permanent complete remission. Fourteen patients (35%) died either from bleeding or from direct complications of therapy. This analysis of ITP in the older adult suggests a disease refractory to therapy that is associated with major morbidity and mortality.

Thrombocytopenic purpura in lambs given bovine colostrum.

Newborn lambs which had been given colostrum from a single cow developed thrombocytopenia and seven died. The clinical signs included purpura, haemoglobinuria, melaena, dullness and lethargy. The lambs died at one to two days old. Post mortem examination revealed widespread haemorrhages throughout the body. Lambs given small amounts of colostrum developed a transient thrombocytopenia but did not die.

[Splenectomy in idiopathic thrombopenic purpura and in myelofibrosis. A retrospective study of platelet increase, hemorrhagic complications and mortality]. / La splenectomia nella porpora piastrinopenica idiopatica e nella mielofibrosi. Une studio retrospettivo dell'incremento piastrinico, complicanze emorragiche e mortalità.

In a retrospective study of 39 splenectomies, patients with increased blood cell breakdown (13 cases of idiopathic thrombocytopenic purpura (ITP), 5 cases of hereditary spherocytosis, 2 of Felty's syndrome and 2 of autoimmune hemolytic anemia) were compared with those patients also presenting decreased blood cell production [14 cases of myelofibrosis (MF) with splenomegaly and 3 cases of advanced chronic myelogeneous leukemia (CML)]. Platelet regeneration post-operatively was significantly (p less than 0.01) more rapid in the ITP than in the MF group. Only 1/22 patients in the ITP group had major post-operative complications as compared to 10/17 in the MF group. None of the patients in the ITP group died within 25 days of operation as compared to 5/17 in the MF group. Only 3/22 patients in the ITP group lost more than 800 ml of blood during the operation as compared to 8/17 with MF. No statistically significant higher blood loss was found in patients with less than 30 x 10(9) platelets/l preoperatively, compared to those with higher platelet counts. However, correlation between the splenic weights and amount of blood loss was statistically significant (p less than 0.01). Thus, splenectomy seems much better tolerated in patients with ITP, even if platelets are low, than in patients with myelofibrosis.

[Splenectomy in idiopathic thrombocytopenic purpura]. / Esplenectomía en púrpura trombocitopénica idiopática.

Between January 1976 and December 1987, 44 patients with idiopathic thrombocytopenic purpura were submitted to splenectomy. This study analyzes the results of treatment and the usefulness of several prognostic markers. There were 38 female patients and six males with a median age of 28.5 years (range 11 to 66 years). Forty two out of 44 patients received preoperative steroids. Seventy five percent achieved a satisfactory response to this treatment but 31 relapsed (in eight recurrence was observed when the steroid dose was tapered). Eleven patients persisted symptomatic notwithstanding medical treatment. In two cases splenectomy was performed as an emergency procedure, and in one patient who was already in remission with previous steroid treatment, splenectomy was performed for other medical reasons. Operative mortality rate was 4 percent, and five patients developed postoperative complications (three had pneumonia, one pulmonary thromboembolism and one would infection). Eighty four percent of the patients were free of disease at one year followup survival at 10 year period was 80 percent. No correlation was found between age, duration of symptoms, previous response to steroids or time-period of medical treatment with the results obtained with splenectomy.

[Thrombotic microangiopathies]. / De trombotiske mikroangiopatier.

The thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive pathophysiological defect in most cases of thrombotic microangiopathic diseases. Early recognition and a secure distinction from other conditions that resemble TMA diseases is essential in order to institute adequate treatment and to obtain a favourable outcome in terms of both survival and development of serious sequelae.

Prognosis of chronic thrombocytopenic purpura.

A multicenter prospective study on the treatment of chronic idiopathic thrombocytopenic purpura (ITP), conducted by the Idiopathic Disorders of Hematopoietic Organ Research Committee, the Ministry of Health and Welfare of Japan, is currently in progress. In this study we analyzed the clinical records of 256 patients with chronic ITP in order to define the prognostic factors. As of November, 1988 after a median observation period of 34 months, 174 of the 256 patients (68%) were alive, 11 (4%) dead and 71 (28%) lost to follow-up. Bleeding was a direct cause of death in only one patient. Assessment of the status of patients based on platelet count at the final observation revealed that 48% of patients were in remission, 21% showed improvement, and 31% remained unchanged or worsened. Univariate analyses identified 4 parameters associated with favorable prognosis: presenting platelet count less than 2 x 10(4)/microliters, platelet count greater than 10 X 10(4)/microliters after one-year follow-up, maximal platelet count greater than 10 X 10(4)/microliters during administration of the initial dose of corticosteroids and splenectomy.

United States Thrombotic Thrombocytopenic Purpura Apheresis Study Group (US TTP ASG): multicenter survey and retrospective analysis of current efficacy of therapeutic plasma exchange.

Thrombotic thrombocytopenic purpura (TTP) remains enigmatic from the perspective of its etiology, pathophysiology, and treatment. Once recognized, the accepted standard of care for TTP is daily therapeutic plasma exchange (TPE). However, the diversity in TPE treatment protocols has made comparisons of clinical research between institutions difficult. This study strived to assess the current practice of TPE in order to provide direction for prospective controlled clinical trials. Twenty large apheresis centers within the United States comprising the US TTP ASG responded to a survey to establish the current status of TPE in TTP. A retrospective analysis from data provided by 14 of 20 centers included 115 initial presentations of primary TTP with an overall mortality rate of 10% and relapse rate of 37%. The majority of deaths (58%) occurred within 48 hours of presentation. Variation in therapeutic targets (platelet count [plt] and serum LDH) and the number of plasma volumes exchanged per procedure did not affect the relapse rate. Initial plt and LDH were not predictive of mortality. Response, relapse, and mortality rates with the combination of 5% albumin for the initial 50% of TPE followed by plasma for the final 50% of TPE as replacement were comparable or possibly better than plasma-only replacement strategies. Forty percent of centers routinely used a TPE taper; however, there was no statistical difference in relapse rates comparing the taper and non-taper sub-groups. By controlling for adjunctive modalities such as steroids and anti-platelet agents, it is hoped that future prospective clinical trials may optimize the role of TPE in TTP, minimize patient exposure to blood products and procedures, shorten the clinical course of TTP, and reduce mortality.

Therapeutic experience on 934 adults with idiopathic thrombocytopenic purpura: Multicentric Trial of the Cooperative Latin American group on Hemostasis and Thrombosis.

In order to analyze the usefulness of different types of treatment in relation to the interval since the onset of idiopathic thrombocytopenic purpura (ITP), a collaborative study of 934 adult patients was undertaken. Prednisone was administered to 818 patients, and 32% of them achieved prolonged complete remission (PCR). However, only 14% of patients who had ITP for more than six months achieved a prednisone-induced PCR (P less than .01). Splenectomy was done in 399 patients, and 65% of them achieved PCR; the remission rate did not vary with the interval since the onset of ITP. Of 120 patients with chronic ITP that was refractory to corticosteroids and splenectomy, 91 received either azathioprine or cyclophosphamide; 21% of them achieved PCR and 55% had a favorable response. None of 19 patients treated with vincristine and only one of ten patients treated with vinblastine-loaded platelets achieved PCR.

Chronic idiopathic thrombocytopenic purpura: incidence, treatment, and outcome.

In order to determine incidence, outcome, trends in management and natural history, data on 92 children with chronic idiopathic thrombocytopenic purpura (ITP), comprising 66 from a single centre's experience between 1950 and 1980 and all 26 presenting from a defined population between 1984 and 1994, have been analysed. Its incidence, calculated from the population based group, is 0.46/10(5) children per year. Twenty nine of 34 (85%) remitted after splenectomy. Short initial histories predicted response to splenectomy. Splenectomy was offered only half as frequently in the last 10 years as in the 30 year, single centre group of children. Most (39 cases) of those not offered or successfully treated by splenectomy recovered spontaneously. The predicted spontaneous remission rate in 85 with adequate follow up data is 61% after 15 years. No other form of active treatment was of lasting benefit. No death solely attributable to chronic ITP occurred. The high spontaneous recovery rate, low mortality, and generally benign outcome may encourage a less interventionist approach to management.