The Prevalence of Pelvic Floor Hematoma After Vaginal Delivery.

OBJECTIVE: The goal of this study was to evaluate differences in levator ani hematoma formation within 3 days of delivery between adult women after their first vaginal delivery and adult women who have had multiple vaginal deliveries. METHODS: This was a cross-sectional study at a single institution from 2013 to 2015 using a high-resolution endovaginal ultrasound transducer to identify postvaginal delivery hematoma formation. Logistic regression was used to examine the association between hematoma formation and vaginal parity while considering potential confounders including induction, vaginal operative delivery, vaginal birth after cesarean, fetal weight, fetal head circumference, race and ethnicity, body mass index, age at delivery, gestational age, and length of second-stage labor. RESULTS: Ninety women (46 vaginal-primiparous; 44 vaginal-multiparous) were included in this study. After adjusting for oxytocin use, length of second-stage labor, and body mass index, the odds of pelvic floor hematoma of 1000 mm3 or greater were 2.93 (95% confidence interval, 0.78-10.91) times greater in women after their first vaginal delivery compared with women with a history of multiple vaginal deliveries. The adjusted odds of pelvic floor hematoma of 1500 mm3 or greater were 6.02 (95% confidence interval, 1.09-33.24) times greater in vaginal-primiparous compared with vaginal-multiparous women. CONCLUSIONS: Although the prevalence of pelvic floor hematoma was higher in vaginal-primiparous women than vaginal-multiparous women after vaginal delivery, hematomas were present in both groups. Future prospective studies are needed to evaluate the additive effect of multiple vaginal deliveries on the pelvic floor.

Immune Thrombocytopenia (ITP): Current Limitations in Patient Management.

Primary immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by isolated thrombocytopenia caused by increased platelet destruction and impaired platelet production. First-line therapies include corticosteroids, intravenous immunoglobulin, and anti-D immunoglobulin. For patients who are refractory to these therapies, those who become corticosteroid dependent, or relapse following treatment with corticosteroid, options include splenectomy, rituximab, and thrombopoietin-receptor agonists, alongside a variety of additional immunosuppressive and experimental therapies. Despite recent advances in the management of ITP, many areas need further research. Although it is recognized that an assessment of patient-reported outcomes in ITP is valuable to understand and guide treatment, these measures are not routinely measured in the clinical setting. Consequently, although corticosteroids are first-line therapies for both children and adults, there are no data to suggest that corticosteroids improve health-related quality of life or other patient-related outcomes in either children or adults. In fact, long courses of corticosteroids, in either children or adults, may have a negative impact on a patient's health-related quality of life, secondary to the impact on sleep disturbance, weight gain, and mental health. In adults, additional therapies may be needed to treat overt hemorrhage, but unfortunately the results are transient for the majority of patients. Therefore, there is a need to recognize the limitations of current existing therapies and evaluate new approaches, such as individualized treatment based on the probability of response and the size of effect on the patient's most bothersome symptoms and risk of adverse effects or complications. Finally, a validated screening tool that identifies clinically significant patient-reported outcomes in routine clinical practice would help both patients and physicians to effectively follow a patient's health beyond simply treating the laboratory findings and physical symptoms of ITP. The goal of this narrative review is to discuss management of newly diagnosed and refractory patients with ITP, with a focus on the limitations of current therapies from the patient's perspective.

Tobacco Use Patterns among GED Recipients.

U.S. General Educational Development diploma (GED) recipients have the highest smoking prevalence of any education level. This paper describes demographic characteristics and tobacco use patterns and examines effect modification and confounding as potential explanations for higher crude prevalence of smoking. METHODS: The study population included adults aged 25 and older in the 2013 National Health Interview Survey. We estimated adjusted prevalence ratios and 95% CIs for smoking and quitting behaviors using weighted multivariable logistic regression. RESULTS: Among women with a GED, adjusted prevalence of ever use (58.7%) and smoking (32.4%) was 1.50 and 1.52 times the prevalence among high school dropouts (39.1%, 21.3%). Female GED recipients had a significantly higher prevalence of ever smoking compared with dropouts. We found no significant educational differences in smoking prevalence among men or quit behaviors for either sex. CONCLUSIONS: More research is needed to identify targeted interventions to prevent smoking in this disparate population.

Management of antithrombotic therapy in adults with immune thrombocytopenia (ITP): a survey of ITP specialists and general hematologist-oncologists.

While patients with immune thrombocytopenia (ITP) and low platelet counts are at risk for bleeding, they are not protected against arterial and venous thrombotic events. Frequently, hematologists are asked to consult on a patient with ITP requiring an antiplatelet (AP) agent or anticoagulant (AC). No direct evidence exists to guide hematologists in weighing the risk of thrombosis against the risk of bleeding in patients with ITP. Therefore, we performed a survey to determine the preferred management of AP/AC therapy in ITP patients. The survey described hypothetical patient scenarios and asked respondents to recommend a minimum platelet count for initiation of AP/AC therapy. We surveyed both hematologists with an international reputation in treatment of ITP (n = 48) and also general hematologist-oncologists in Oklahoma (n = 97). Response rates were 38/48 (79%) for the ITP specialists and 46/97 (47%) for general hematologist-oncologists. Overall, recommended platelet thresholds for antithrombotic therapy were similar between ITP specialists and general hematologist-oncologists. Although both groups recommended a minimum platelet count of 50 × 109/L for AP and AC therapy in most scenarios, there was great variability in individual practice patterns among respondents. This study highlights the need for studies of patients with ITP who require AP/AC therapy to provide high-quality evidence for establishing optimal management strategies.

A structural equation modeling approach to understanding pathways that connect socioeconomic status and smoking.

The inverse association between socioeconomic status and smoking is well established, yet the mechanisms that drive this relationship are unclear. We developed and tested four theoretical models of the pathways that link socioeconomic status to current smoking prevalence using a structural equation modeling (SEM) approach. Using data from the 2013 National Health Interview Survey, we selected four indicator variables (poverty ratio, personal earnings, educational attainment, and employment status) that we hypothesize underlie a latent variable, socioeconomic status. We measured direct, indirect, and total effects of socioeconomic status on smoking on four pathways through four latent variables representing social cohesion, financial strain, sleep disturbance, and psychological distress. Results of the model indicated that the probability of being a smoker decreased by 26% of a standard deviation for every one standard deviation increase in socioeconomic status. The direct effects of socioeconomic status on smoking accounted for the majority of the total effects, but the overall model also included significant indirect effects. Of the four mediators, sleep disturbance and psychological distress had the largest total effects on current smoking. We explored the use of structural equation modeling in epidemiology to quantify effects of socioeconomic status on smoking through four social and psychological factors to identify potential targets for interventions. A better understanding of the complex relationship between socioeconomic status and smoking is critical as we continue to reduce the burden of tobacco and eliminate health disparities related to smoking.

Changes in bone marrow morphology in adults receiving romiplostim for the treatment of thrombocytopenia associated with primary immune thrombocytopenia.

The effects of romiplostim on bone marrow morphology were evaluated in adults with immune thrombocytopenia (ITP). Patients with platelet counts <50 × 10(9)/L, ≥1 prior ITP therapies, and no collagen at baseline received weekly subcutaneous romiplostim starting at 1 µg/kg, adjusted to maintain platelet counts between 50 and 200 × 10(9)/L. Biopsies were scheduled after 1, 2, or 3 years of romiplostim (cohorts 1, 2, and 3, respectively). Irrespective of scheduled time, biopsies were performed earlier if patients discontinued or failed to achieve/maintain a response to romiplostim. Reticulin (silver stain) and collagen (trichrome stain) were graded by two hematopathologists using the modified Bauermeister scale (0-4). Of 169 patients, 131 had evaluable biopsies; 9/131 (6.9 %) had increases of ≥2 grades on the modified Bauermeister scale (cohort 1: 0/34; cohort 2: 2/39; cohort 3: 7/58), including two with collagen. Three of the nine patients had follow-up biopsies, including one patient with collagen; changes were reversible after romiplostim discontinuation. Of the nine patients, one had neutropenia detected by laboratory test and two had adverse events of anemia, both non-serious and not treatment-related. By actual exposure (as some biopsies did not occur as scheduled), the number of patients with grade increases ≥2 were year 1: 3/41, year 2: 1/38, year 3: 5/52. Twenty-four patients sustained platelet counts ≥50 × 10(9)/L for ≥6 months with no ITP medications after discontinuing romiplostim, i.e., they entered clinical remission of their ITP. In conclusion, in patients with ITP receiving romiplostim, bone marrow changes were observed in a small proportion of patients.ClinicalTrials.gov identifier: NCT#00907478.

Barriers And Motivators for Smoking Cessation in Patients With Systemic Lupus Erythematosus (SLE).

UNLABELLED: Although studies have shown that smoking is detrimental to the health of patients with systemic lupus erythematosus (SLE), studies regarding barriers and motivators for smoking cessation are lacking. The purpose of this study was to generate hypotheses regarding the barriers and motivators for smoking cessation in SLE patients. METHODS: This study was based on the theoretical framework of the stages of change model. All participants met SLE classification criteria. Interviews were conducted with 16 current and 10 former smokers. RESULTS: Motivators included: medical reasons, readiness, and concern for others. Barriers included: enjoyment, coping mechanism, and an emotional connection. Participants were unsure of the impact of smoking on their medication and disease, and had mixed feelings regarding the impact on pain. CONCLUSION: The main motivator for cessation in this population was concern for one's health. Rheumatologists need to include disease specific harms and assess pain management strategies as part of cessation counseling.

Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura.

UNLABELLED: Pregnancy may precipitate acute episodes of thrombotic thrombocytopenic purpura (TTP), but pregnancy outcomes in women who have recovered from acquired TTP are not well documented. We analyzed pregnancy outcomes following recovery from TTP associated with acquired, severe ADAMTS13 deficiency (ADAMTS13 activity <10%) in women enrolled in the Oklahoma TTP-HUS Registry from 1995 to 2012. We also systematically searched for published reports on outcomes of pregnancies following recovery from TTP associated with acquired, severe ADAMTS13 deficiency. Ten women in the Oklahoma Registry had 16 subsequent pregnancies from 1999 to 2013. Two women had recurrent TTP, which occurred 9 and 29 days postpartum. Five of 16 pregnancies (31%, 95% confidence interval, 11%-59%) in 3 women were complicated by preeclampsia, a frequency greater than US population estimates (2.1%-3.2%). Thirteen (81%) pregnancies resulted in normal children. The literature search identified 382 articles. Only 6 articles reported pregnancies in women who had recovered from TTP associated with acquired, severe ADAMTS13 deficiency, describing 10 pregnancies in 8 women. TTP recurred in 6 pregnancies. CONCLUSIONS: With prospective complete follow-up, recurrent TTP complicating subsequent pregnancies in Oklahoma patients is uncommon, but the occurrence of preeclampsia may be increased. Most pregnancies following recovery from TTP in Oklahoma patients result in normal children.

Management of primary immune thrombocytopenia, 2012: a survey of oklahoma hematologists-oncologists.

BACKGROUND: Management options for patients with primary immune thrombocytopenia (ITP) have increased, and treatment of patients with ITP has changed during the past 10 years. METHODS: To document current practice and to determine how current practice is related to recommendations of 2 recent practice guidelines for ITP, an International Consensus report and an American Society of Hematology (ASH) guideline, the authors surveyed practicing hematologists-oncologists in Oklahoma. Surveys were specific for children or adults. Each survey had 3 questions describing patients with a new diagnosis and patients who had not achieved remission with initial treatment. Questions were adapted from the clinical scenarios of the ASH guideline. RESULTS: Twelve (92%) Oklahoma pediatric hematologists-oncologists responded; 82 (81%) Oklahoma adult hematologists-oncologists responded. For a child with a new diagnosis of ITP, a platelet count of 8000/µL and minor bleeding, 5 (42%) hematologists-oncologists selected observation without drug treatment (recommended by both guidelines). For an adult with a platelet count of 9000/µL who had failed to respond to initial treatment with corticosteroids and IVIg, 32 (39%) selected splenectomy (recommended by the ASH guideline); 30 (37%) selected rituximab and 13 (16%) selected thrombopoietin-receptor agonists (both recommended by the International Consensus report). Hematologists-oncologists who had more years in practice were more likely to select splenectomy (P = 0.047). CONCLUSIONS: In a time of changing management for patients with ITP, these data document reported current management in Oklahoma and provide a basis for serial comparisons across time and for comparisons with other regions and comparison of management with patient outcomes.

Drug-induced thrombocytopenia in children.

BACKGROUND: Acute, immune-mediated thrombocytopenia may be caused by many different approved drugs as well as by other substances including vaccines, complementary and alternative medicines, herbal remedies, nutritional supplements, foods and beverages. All causes are described as drug-induced thrombocytopenia (DITP). Often the cause is not recognized, resulting in recurrent thrombocytopenia and inappropriate treatments. Systematic analysis of children (age less than 18 years) with suspected DITP has not been previously reported. PROCEDURES: (1) We searched 15 databases to identify articles describing children with thrombocytopenia as an adverse effect of drugs and other substances. Articles were reviewed to assign levels of evidence for an association of the suspected substance with thrombocytopenia. (2) Data from the BloodCenter of Wisconsin were reviewed to identify reports of drug-dependent, platelet-reactive antibodies in children with suspected DITP. RESULTS: Of 2,191 articles identified, 242 were selected for review. Seventy-two articles reporting 74 individual patients and nine groups of patients had evaluable data. Eleven individual patients and one group had definite evidence and 40 patients and three groups had probable evidence for an association of the suspected substance with thrombocytopenia. Thirty-two substances had a definite or probable association with thrombocytopenia. During 2008-2012, sera from 91 children with suspected DITP were tested and 21 had drug-dependent, platelet-reactive antibodies involving six substances. CONCLUSIONS: Drugs and other substances must be considered as potential causes of thrombocytopenia. Evidence from published reports and data for drug-dependent, platelet-reactive antibodies can help clinicians evaluate of children with unexpected thrombocytopenia.

Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) associated with severe, acquired ADAMTS13 deficiency is uncommonly reported in children. The incidence, demographic, and clinical features of these children, compared to adults, have not been described. PROCEDURES: This study focused on children (<18 years old) and adults with TTP associated with severe, acquired ADAMTS13 deficiency, defined as activity <10%. The incidence rates for TTP in children and adults were calculated from patients enrolled in the Oklahoma TTP-HUS (Hemolytic-Uremic syndrome) Registry, 1996-2012. To describe demographic and clinical features, children with TTP were also identified from a systematic review of published reports and from samples sent to a reference laboratory for analysis of ADAMTS13. RESULTS: The standardized annual incidence rate of TTP in children was 0.09 × 10(6) children per year, 3% of the incidence rate among adults (2.88 × 10(6) adults per year). Among the 79 children who were identified (one from the Oklahoma Registry, 55 from published reports, 23 from the reference laboratory), TTP appeared to be more common among females, similar to the relative increased frequency of women among adults with TTP, and more common in older children. Clinical data were available on 52 children; the frequency of severe renal failure, relapse, treatment with rituximab, and systemic lupus erythematosus in these children was similar to adults with TTP. CONCLUSIONS: TTP associated with severe, acquired ADAMTS13 deficiency is uncommon in children. The demographic and clinical features of these children are similar to the features of adults with TTP.

Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura-hemolytic uremic syndrome, 1996 to 2011.

BACKGROUND: Plasma exchange (PEX) treatment for patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) has risk for major complications. STUDY DESIGN AND METHODS: Data for PEX-related complications have been prospectively collected on all patients enrolled in the Oklahoma TTP-HUS Registry, 1996 to 2011. PEX-related complications have been defined as major or minor and as central venous catheter related or plasma related. RESULTS: During 15 years, 1996 to 2011, 72 (24%) of 302 consecutive patients had major PEX-related complications. Analysis of five consecutive 3-year cohorts demonstrated that there has been a significant trend for decreasing frequency of all PEX-related major complications (p = 0.014) and central venous catheter-related major complications (p = 0.021) but not for the less common plasma-related major complications (p = 0.380). ADAMTS13 activity was measured in 288 (95%) of the 302 patients. Analysis of the 66 patients with ADAMTS13 activity of less than 10% demonstrated a significant trend for decreasing frequency of PEX-related major complications (p = 0.036); the trend for the 222 patients with ADAMTS13 activity of at least 10% was not significant (p = 0.118). The decreased frequency of PEX-related major complications among patients with ADAMTS13 activity of less than 10% may be related to a significant trend for decreasing duration of PEX treatment (p = 0.040) and decreasing frequency of requirement for more than one central venous catheter (p = 0.044). The decreased duration of PEX treatment may be related to increased use of adjunctive treatments: corticosteroids (p < 0.001) and rituximab (p < 0.001). CONCLUSIONS: The frequency of PEX-related major complications has decreased from 1996 to 2011, possibly related to increased use of corticosteroids and rituximab and the decreased duration of PEX required to achieve remission.

Thrombotic microangiopathic syndromes associated with drugs, HIV infection, hematopoietic stem cell transplantation and cancer.

Thrombotic microangiopathy (TMA) has multiple etiologies. In the four disorders described in this review, the primary organ involved is the kidney. Drug-associated TMA can be an acute, immune-mediated disorder or the result of gradual, dose-dependent toxicity. TMA may occur in patients with advanced HIV infection, possibly mediated by angio-invasive infections. TMA following allogeneic hematopoietic stem cell transplantation may also be caused by drug toxicity; the pathogenesis may involve inhibition of vascular endothelial cell growth factor in renal podocytes. Malignancies of many types with systemic microvascular involvement may cause TMA. Recognition that these syndromes may mimic TTP is important to provide appropriate management and to avoid the inappropriate use of plasma exchange treatment.

Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes.

Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) represent multiple disorders with diverse etiologies. We compared the gender and race of 335 patients enrolled in the Oklahoma TTP-HUS Registry across 21 years for their first episode of TTP or HUS to appropriate control groups. The relative frequency of women and white race among patients with TTP-HUS-associated with a bloody diarrhea prodrome and the relative frequency of women with quinine-associated TTP-HUS were significantly greater than their control populations. The relative frequency of women and black race among patients with idiopathic TTP and TTP-associated with severe ADAMTS13 deficiency was significantly greater than their control populations. The relative frequency of black race among patients who had systemic lupus erythematosus (SLE) preceding TTP was significantly greater than among a population of patients with SLE, and the relative frequency of black race among patients with other autoimmune disorders preceding TTP was significantly greater than their control population. No significant gender or race disparities were present among patients with hematopoietic stem cell transplantation-associated thrombotic microangiopathy, TTP associated with pregnancy, or TTP associated with drugs other than quinine. The validity of these observations is supported by the enrollment of all consecutive patients across 21 years from a defined geographic region, without selection or referral bias. These observations of different gender and race disparities among the TTP-HUS syndromes suggest the presence of different risk factors and may serve as starting points for novel investigations of pathogenesis.

Frequency and significance of HIV infection among patients diagnosed with thrombotic thrombocytopenic purpura.

BACKGROUND: Case series of patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) have reported different frequencies of human immunodeficiency virus (HIV) infection; some series suggest that HIV infection may cause TTP. METHODS: We systematically reviewed all reports of HIV infection in case series of patients with TTP. We analyzed data from the Oklahoma TTP-HUS (hemolytic uremic syndrome) Registry, an inception cohort of 362 consecutive patients, for 1989-2007. RESULTS: Nineteen case series reported the occurrence of HIV infection at the time of diagnosis of TTP in 0%-83% of patients; individual patient data were rarely described. The Oklahoma TTP-HUS Registry determined the HIV status at the time of diagnosis of TTP in 351 (97%) of 362 patients. HIV infection was documented in 6 (1.84%; 95% CI, 0.68%-4.01%) of 326 adult patients (age, 26-51 years); follow-up data were complete for all 6 patients. The period prevalence of HIV infection among all adults in the Oklahoma TTP-HUS Registry region for 1989-2007 was 0.30%. One patient had typical features of TTP with 5 relapses. Five patients had single episodes; in 4, the clinical features that had initially suggested the diagnosis of TTP were subsequently attributed to malignant hypertension (in 3 patients) and disseminated Kaposi sarcoma (in 1 patient). CONCLUSIONS: HIV infection, similar to other inflammatory conditions, may trigger acute episodes of TTP in susceptible patients. More commonly, acquired immunodeficiency syndrome-related disorders may mimic the clinical features of TTP. If the diagnosis of TTP is suggested in a patient with HIV infection, there should be careful evaluation for alternative diagnoses and cautious consideration of plasma exchange, the required treatment for TTP.

Disseminated malignancy misdiagnosed as thrombotic thrombocytopenic purpura: A report of 10 patients and a systematic review of published cases.

BACKGROUND: Patients with disseminated malignancy who present with microangiopathic hemolytic anemia and thrombocytopenia may be misdiagnosed as thrombotic thrombocytopenic purpura (TTP), resulting in inappropriate plasma exchange treatment, a procedure with major risk, and delay of appropriate chemotherapy. PURPOSE: To assess clinical features that may distinguish occult disseminated malignancy from TTP. PATIENTS AND METHODS: We report the 17-year experience of The Oklahoma TTP-Hemolytic-Uremic Syndrome (HUS) Registry (1989-2005) and a systematic review of previously published case reports. RESULTS: Ten of 351 patients in the Oklahoma Registry who were initially diagnosed with TTP and treated with plasma exchange were subsequently discovered to have disseminated malignancy. Only one patient had a history of cancer. In these 10 patients, neurologic abnormalities, hematocrit, platelet count, and serum creatinine were not different from the 133 concurrent patients with idiopathic TTP. Patients with disseminated malignancy had a longer duration of symptoms, more frequent presence of respiratory symptoms, higher lactate dehydrogenase levels, and more often failed to respond to plasma exchange treatment. Diagnosis of malignancy was made by bone marrow biopsy in six patients but not until autopsy in two patients. A systematic literature review identified 19 additional patients, reported from 1965 to 2005, in whom TTP or HUS was initially suspected and systemic malignancy was subsequently discovered. Fourteen different malignant disorders were diagnosed in these 29 patients. CONCLUSIONS: Occult disseminated malignancy may mimic TTP. A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange.

Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications.

Splenectomy has been a standard treatment for adult patients with idiopathic thrombocytopenic purpura (ITP) for more than 50 years. However, the durability of responses, the ability to predict who will respond, and the frequency of surgical complications with splenectomy all remain uncertain. To better interpret current knowledge we systematically identified and reviewed all 135 case series, 1966 to 2004, that described 15 or more consecutive patients who had splenectomy for ITP and that had data for 1 of these 3 outcomes. Complete response was defined as a normal platelet count following splenectomy and for the duration of follow-up with no additional treatment. Forty-seven case series reported complete response in 1731 (66%) of 2623 adult patients with follow-up for 1 to 153 months; complete response rates did not correlate with duration of follow-up (r = -0.103, P = .49). None of 12 preoperative characteristics that have been reported consistently predicted response to splenectomy. Mortality was 1.0% (48 of 4955 patients) with laparotomy and 0.2% (3 of 1301 patients) with laparoscopy. Complication rates were 12.9% (318 of 2465) with laparotomy and 9.6% (88 of 921 patients) with laparoscopic splenectomy. Although the risk of surgery is an important consideration, splenectomy provides a high frequency of durable responses for adult patients with ITP.

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic HPC transplantation: a diagnostic dilemma.

BACKGROUND: Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) has been described as a specific sequela of allogeneic HPC transplantation (HPCT). Nevertheless, because multiple transplant-related sequela can cause the characteristic clinical features of TTP-HUS, the diagnosis is difficult. STUDY DESIGN AND METHODS: All English-language articles describing patients with TTP-HUS following HPCT were identified. Articles reporting five or more total patients, including at least one patient diagnosed with TTP-HUS following allogeneic HPCT, were reviewed. All articles describing autopsies of patients diagnosed with TTP-HUS following allogeneic HPCT were also reviewed. RESULTS: Thirty-five articles reporting 5 or more total patients described 447 patients diagnosed with TTP-HUS following allogeneic HPCT. The frequency of diagnosis of TTP-HUS following allogeneic HPCT varied by 125-fold (0.5%-63.6%). Twenty-eight different sets of diagnostic criteria were described in the 35 articles; 25 articles included both RBC fragmentation and increased serum LDH. Many risk factors described as correlating with the diagnosis of TTP-HUS also predict greater risk for multiple transplant-related complications. Benefit of plasma exchange treatment could not be documented. Survival information was reported for 379 patients, 232 (61%) died, and reported mortality rates varied from 0 to 100 percent. Autopsies have been reported for 35 patients who were diagnosed with TTP-HUS following allogeneic HPCT; none had systemic thrombotic microangiopathy, the diagnostic abnormality of TTP-HUS; and infection (19 patients) was the most commonly reported cause of death. CONCLUSIONS: The clinical features of TTP-HUS following allogeneic HPCT may be caused by common transplant-related complications; the benefit from plasma exchange treatment is uncertain.

The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry: a community perspective of patients with clinically diagnosed TTP-HUS.

The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry enrolls all consecutive patients for whom plasma exchange treatment is requested for clinically diagnosed TTP-HUS within a defined geographic region. During 14.5 years, from January 1, 1989 until June 30, 2003, 301 patients have been enrolled; follow-up is complete on 300 patients. Clinical categories have been designated based on associated conditions and potential etiologies; presenting features and clinical outcomes have been defined to allow comparisons between groups. ADAMTS-13 activity was measured on 142 (88%) of 161 consecutive patients enrolled from 1995 to 2001. Only 13% of all patients, and 33% of patients with idiopathic TTP-HUS, had severe ADAMTS-13 deficiency (<5% activity). The presenting features and clinical outcomes of patients with severe ADAMTS-13 deficiency were heterogeneous and not distinct from patients without severe ADAMTS-13 deficiency. These data suggest that severe ADAMTS-13 deficiency does not detect all patients who may be appropriately diagnosed with TTP-HUS and who may respond to plasma exchange treatment. Prospective data from consecutive patients are essential to translate new observations on pathogenesis into improved patient care.