{"title":"Nonsteroidal Anti-inflammatory Drug Induced Thrombotic Thrombocytopenic Purpura.","authors":"Karlos Z Oregel, Jeremy Ramdial, Stefan Glück","doi":"10.4137/CMBD.S12843","DOIUrl":"https://doi.org/10.4137/CMBD.S12843","url":null,"abstract":"<p><p>A 21-year-old male presented to the emergency department after a 5-day history of recurrent vomiting and decreased urine output. History revealed ingestion of ibuprofen. During the diagnostic workup, the following was identified: white blood cell count 13.4 (×10(3)/mcL), hemoglobin 11.9 (×10(6)/mcL) with an MCV of 73 fL, hematocrit 34% and platelets were 31,000/mcL, sodium of 130 mmol/L, potassium of 5.1 mmol/L, chloride of 83 mmol/L, bicarbonate of 21 mmol/L, blood urea nitrogen of 184 mg/dL and creatinine of 19.1 mg/dL. He was later diagnosed with thrombotic thrombocytopenic purpura (TTP) based on the fact that he presented with most components of the TTP pentad (except for fever), which included altered mental status, acute kidney injury, thrombocytopenia, and evidence of red cell fragmentation and his ADAMTS13 level was found to be less than 10% prior to therapy. The patient then received plasma exchange, oral corticosteroids, and hemodialysis, which led to a full recovery of platelet count and renal function. </p>","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2013-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.4137/CMBD.S12843","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32910858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hemoglobinuria misidentified as hematuria: review of discolored urine and paroxysmal nocturnal hemoglobinuria.","authors":"Prashant Veerreddy","doi":"10.4137/CMBD.S11517","DOIUrl":"https://doi.org/10.4137/CMBD.S11517","url":null,"abstract":"<p><p>Discolored urine is a common reason for office visits to a primary care physician and urology referral. Early differentiation of the type or cause of discolored urine is necessary for accurate diagnosis and prompt management. Paroxysmal nocturnal hemoglobinuria is a clonal disorder caused by acquired somatic mutations in the PIG-A gene on the X- chromosome of hemopoietic stem cells and leads to deficiency of surface membrane anchor proteins. The deficiency of these proteins leads to an increased risk of hemolysis of erythrocytes and structural damage of platelets, resulting in a clinical syndrome characterized by complement-mediated intravascular hemolytic anemia, bone marrow failure, and venous thrombosis. Patients with this clinical syndrome present with paroxysms of hemolysis, causing hemoglobinuria manifesting as discolored urine. This can be easily confused with other common causes of discolored urine and result in extensive urologic work-up. Three commonly confused entities of discolored urine include hematuria, hemoglobinuria, and myoglobinuria. Specific characteristics in a dipstick test or urinalysis can guide differentiation of these three causes of discolored urine. This article begins with a case summary of a woman presenting with cranberry-colored urine and a final delayed diagnosis of paryxysmal nocturnal hemoglobinuria. Her hemoglobinuria was misdiagnosed as hematuria, leading to extensive urologic work-up. The article also gives an overview of the approach to diagnosing and treating discolored urine. </p>","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2013-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.4137/CMBD.S11517","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32910857","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ramy Ibrahim, Jaffar Ahmad Alhilli, Tyler T Cooper, Irina Dashkova, Judah Guy, Anjula Gandhi, Mohammad Zaman
{"title":"Idiopathic thrombocytopenia with iron deficiency anemia.","authors":"Ramy Ibrahim, Jaffar Ahmad Alhilli, Tyler T Cooper, Irina Dashkova, Judah Guy, Anjula Gandhi, Mohammad Zaman","doi":"10.4137/CMBD.S11371","DOIUrl":"https://doi.org/10.4137/CMBD.S11371","url":null,"abstract":"<p><p>We report a rare case of anemia and thrombocytopenia as a result of uterine fibroid and adenomyosis, complicated by immune thrombocytopenic purpura (ITP). Symptoms were presented as menorrhagia and metrorrhagia in a 34-year-old African American woman, who was later treated with blood and platelet transfusion and iron therapy with steroids. Uterine fibroids are commonly found to cause hematologic disturbances such as anemia and reactive thrombocytosis and, less commonly, thrombocytopenia. Moreover, such hematologic disturbances are secondary to heavy and irregular uterine bleeding, which is typically presented. A previous uterine fibroid diagnosis was made and reconfirmed by pelvic and transvaginal ultrasound to exclude other locoregional pathologies. ITP was suggested by Coombs test and several other serologies, leading to confirmation via bone marrow biopsy. In a previous case study, we reported positive responses in hemotecrit and platelet count after the introduction of iron therapy to an iron-depleted middle-aged female presenting severe anemia and thrombocytopenia.1. </p>","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2013-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.4137/CMBD.S11371","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32910856","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical and Hematological Features of 335 Patients of Chronic Myelogenous Leukemia Diagnosed at Single Centre in Northern Pakistan","authors":"F. A. Bhatti, Suhaib Ahmed, N. Ali","doi":"10.4137/CMBD.S10578","DOIUrl":"https://doi.org/10.4137/CMBD.S10578","url":null,"abstract":"There are no studies regarding analysis of clinical and haematological features of chronic myelogenous leukemia (CML) from Pakistan. This study analyzes the data of patients suffering from CML, reporting to a major referral Institute in Northern Pakistan in the past 6 years and 3 months. CML constitutes approximately 80% of all myeloproliferative disorders, with a peak incidence between 21-50 years of age, and a male:female ratio of 2:1. Anaemia and massive splenomegaly were the main clinical features found in 92% and 47% patients respectively. There was significant correlation between anaemia and WBC counts with degree of splenomegaly. Three percent of all CML patients presented as de novo accelerated phase, and another 3% presented as blast crises without any previous history of chronic phase. The ratio of myeloid and lymphoid blast crisis was 2:1. Median duration of chronic phase in patients on hydroxyurea treatment was 6 years. Thirty six percent of patients in chronic phase of CML belonged to intermediate and high risk according to Sokal and Hasford scoring systems. In contrast to the Caucasian populations where the peak incidence of the disease is in 6th to 7th decade, CML occurs in Pakistan in a much younger population, with a broad peak between 21-50 years of age. Patients present in fairly advanced disease because of poor access to health care facilities, due to non-affordability and lack of health insurance coverage.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2012-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85199473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Y. Vinogradova, I. Kaplanskaya, R. S. Samoilova, I. Vorobiev, B. Zingerman, Y. Sidorova, N. Shklovskiy-Kordi, L. G. Aitova, D. C. Maryin, J. Morris, L. Varticovski, A. Vorobiev
{"title":"Clinicopathological Features and Outcomes of T- and NK-Cell Lymphomas in European Russia","authors":"Y. Vinogradova, I. Kaplanskaya, R. S. Samoilova, I. Vorobiev, B. Zingerman, Y. Sidorova, N. Shklovskiy-Kordi, L. G. Aitova, D. C. Maryin, J. Morris, L. Varticovski, A. Vorobiev","doi":"10.4137/CMBD.S7804","DOIUrl":"https://doi.org/10.4137/CMBD.S7804","url":null,"abstract":"With the introduction of the revised World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues in 2001, many patients had to be re-evaluated for the correct diagnosis of T- and NK-cell lymphomas. Because some T-cell malignancies are associated with poor outcome, it is important to identify subsets of patients that may benefit from novel or more intensive therapies. The purpose of this study was to determine, for the first time, the relative frequencies, pathological features and outcomes of patients with T- and NK-cell lymphomas in a predominantly Russian Slavic population. We identified 291 patients with a diagnosis of T- and NK-cell malignancies treated at our Center between 2000-2008. In applying the revised WHO classification, we confirmed the diagnosis and had complete clinical follow up and pathological information on 264 cases that were included in the analysis. We found some differences in frequency of several subsets as compared with previously published reports, including younger age of onset and relatively higher incidence of T-LGL in our patients. We also confirm that intensive treatment regimens of advanced stage PTCL and ALK—ALCL led to considerable improvement in response rates, but not in the overall survival.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2012-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85282190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
K. Nsiah, V. Dzogbefia, Daniel Ansong, A. Akoto, H. Boateng, D. Ocloo
{"title":"Pattern of AST and ALT changes in Relation to Hemolysis in sickle cell Disease","authors":"K. Nsiah, V. Dzogbefia, Daniel Ansong, A. Akoto, H. Boateng, D. Ocloo","doi":"10.4137/CMBD.S3969","DOIUrl":"https://doi.org/10.4137/CMBD.S3969","url":null,"abstract":"Background Elevated aminotransferase levels are commonly associated with compromised hepatic integrity from various insults. In sickle cell disease, aspartate transaminase (AST) is also released via intravascular hemolysis. This study was done to determine the pattern of changes in AST and alanine transaminase (ALT), in particular the AST:ALT ratio, and to relate these to the hemolytic state, which we consider to be more important than hepatic and cardiac dysfunction in some individuals with sickle cell disease. Methods Serum aminotransferase levels were measured in 330 subjects with sickle cell disease, as well as hemoglobin, reticulocytes, and lactate dehydrogenase. The AST:ALT ratio was designated as a hemolytic marker, and simple and multivariate regression analyses were carried out between this ratio and other hemolytic markers. Results Mean AST and ALT levels were 48.24 % 27.78 and 26.48 % 22.73 U/L, respectively. However, for 49 subjects without sickle cell disease, mean AST and ALT levels were the same, ie, 23.0 U/L. In the subjects with sickle cell disease, the increases in AST levels were far higher than for ALT, supporting its release via intravascular hemolysis. In 95.8% of the subjects with sickle cell disease, the AST:ALT ratio was > 1, but our results did not suggest overt malfunctioning of the liver and heart in the majority of subjects. Conclusion Regression analyses support the use of the AST:ALT ratio as a hemolytic marker, because it has an inverse association with the hemoglobin level. Whether in steady state or in crisis, provided hepatic and cardiac integrity has not been compromised, subjects with sickle cell disease would have higher AST levels due to the hemolytic nature of the condition. This is the first report highlighting the AST:ALT ratio in sickle cell disease.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2011-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83482793","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema","authors":"S. Valle, A. T. França, R. Campos, A. Grumach","doi":"10.4137/CMBD.S4090","DOIUrl":"https://doi.org/10.4137/CMBD.S4090","url":null,"abstract":"Hereditary angioedema (HAE) is a relatively rare disease characterized by acute episodes of swelling. These swellings can be disfiguring, painful and life-threatening. Since the symptoms occur in different areas and most patients experience a delay in their diagnosis, resulting in unnecessary suffering and dangerous situations. HAE can have a tremendous impact on the quality of life. The major genetic deficiency in this disorder is either an absent or nonfunctional C1INH which regulates the complement, fibrinolitic, kalikrein and plasmin pathways.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2011-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90271498","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Safety and Efficacy of Argatroban in the Management of Heparin-Induced Thrombocytopenia","authors":"B. Saugel, R. Schmid, W. Huber","doi":"10.4137/CMBD.S5118","DOIUrl":"https://doi.org/10.4137/CMBD.S5118","url":null,"abstract":"Heparin-induced thrombocytopenia (HIT) is a life-threatening adverse reaction to heparin therapy that is characterized by thrombocytopenia and an increased risk of venous and arterial thrombosis. According to guidelines, in patients with strongly suspected or confirmed HIT all sources of heparin have to be discontinued and an alternative, nonheparin anticoagulant for HIT treatment must immediately be started. For both the prophylaxis of thrombembolic events in HIT and the treatment of HIT with thrombosis the direct thrombin inhibitor argatroban is approved in the United States. The objective of this review is to describe the mechanism of action and the pharmacokinetic profile of argatroban, to characterize argatroban regarding its safety and therapeutic efficacy and to discuss its place in therapy in HIT.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2011-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86433325","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
D. Calverley, I. Casserly, Qamrul G. Choudhury, T. Phang, B. Gao, J. Messenger, M. Geraci
{"title":"Platelet Gene Expression as a Biomarker Risk Stratification Tool in Acute Myocardial Infarction: A Pilot Investigation","authors":"D. Calverley, I. Casserly, Qamrul G. Choudhury, T. Phang, B. Gao, J. Messenger, M. Geraci","doi":"10.4137/CMBD.S5005","DOIUrl":"https://doi.org/10.4137/CMBD.S5005","url":null,"abstract":"Platelets play a major role in the pathophysiology of acute myocardial infarction (AMI). Recent evidence reveals megakaryocyte-derived platelet pre-mRNA is spliced to mRNA and then translated into functional proteins in response to external stimulation. An exon microarray analyzes pre-mRNA alternative splicing and is thus applicable for studying gene expression in the anucleate platelet. We hypothesized a subset of megakaryocyte/platelet genes exists that are significantly over or underexpressed in AMI compared with stable coronary artery disease (CAD), yielding a gene expression profile for further study. Microarray analysis employing platelet mRNA was used to generate gene expression data in the above two patient groups. Unsupervised hierarchical clustering has revealed an expression profile that includes 95 over- or under-expressed genes depicted in a heat map where separation of both sets takes place. This preliminary study reveals a platelet-based gene expression signature that differentiates between AMI and stable CAD, and further study may yield a prognostic tool for a future AMI event in atherosclerosis risk factor-based subsets of CAD patients.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2010-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79416898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Taysir Garadah, S. Kassab, Najat Mahdi, Ahmed A. Abu-Taleb, A. Jamsheer
{"title":"Pulsed and Tissue Doppler Echocardiographic Changes in Patients with Thalassemia Major","authors":"Taysir Garadah, S. Kassab, Najat Mahdi, Ahmed A. Abu-Taleb, A. Jamsheer","doi":"10.4137/CMBD.S4377","DOIUrl":"https://doi.org/10.4137/CMBD.S4377","url":null,"abstract":"Background Doppler echocardiographic studies of left ventricle (LV) systolic and diastolic function in patients with β-Thalassemia Major (β-TM) had shown different patterns of systolic and diastolic dysfunction. Aim This cross-sectional study was designed to study the LV systolic and diastolic function in patients with β-TM using Pulsed Doppler (PD) and Tissue Doppler (TD) echocardiography. Methods All patients were evaluated clinically and by echocardiography, The study included patients with β-TM (n = 38, age 15.7 ± 8.9 years) compared with an age-matched control group (n = 38, age 15.9 ± 8.9 years). The pulse Doppler indices were normalized for age and heart rate. Results Compared with control patients, M-Mode showed that patients with β-TM have thicker LV septal wall index (0.659 ± 0.23 vs. 0.446 ± 0.219 cm, P ≤ 0.001), posterior wall index (0.659 ± 0.235 vs. 0.437 ± 0.214 cm, P ≤ 0.01), and larger LVEDD index is (3.99 ± 0.48 vs. 2.170 ± 0.57 mm. P = 0.035). Pulsed Doppler showed high LV trans-mitral E wave velocity (70.818 ± 10.139 vs. 57.532 ± 10.139, p = 0.027) and E/A ratio (1.54 vs. 1.23, P ≤ 0.01). The duration of Deceleration time (DT) and isovolumic relaxation time (IVRT) were significantly shorter in patients with β-TM (150.234 ± 20.0.23 vs. 167.123 ± 19.143 msec, P ≤ 0.01) and (60.647 ± 6.77 vs. 75.474 ± 5.83 msec, P ≤ 0.001), respectively. The ratio of transmitral E wave velocity to the tissue Doppler E wave at the basal septal mitral annulus E/Em– was significantly higher in β-TM group (14.024 ± 2.29 vs. 12.132 ± 1.82, P ≤ 0.01). The Tissue Doppler systolic velocity (Sm) and the early diastolic velocity (Em) were significantly lower in β-TM group compared to control (4.31 ± 1.2 cm/s vs. 6.95 ± 2.1, P ≤ 0.01 and 4.31 ± 2.7 cm/s vs. 5.82 ± 2.5, P ≤ 0.01) respectively. The tricuspid valve velocity was significantly higher than controls (2.993 ± 0.569 vs. 1.93 ± 0.471 m/sec, respectively, P ≤ 0.01). However, the LVEF% and fractional shortening were normal with no significant difference in both groups. Conclusion In this study, patients with β-thalassemia major compared with controls, have significantly thicker LV wall, and larger LV cavity and LV diastolic filling indices suggestive of restrictive pattern with a higher tricuspid valve velocity. These data showed that left ventricle diastolic indices are compromised initially in patients with β-thalassemia major.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":null,"pages":null},"PeriodicalIF":3.0,"publicationDate":"2010-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80405971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}