Primary splenic hairy cell leukaemia: a case report and review of the literature

Hairy cell leukaemia affecting primarily the spleen is a very rare feature of this disease at presentation. Splenectomy in such cases would seem to provide a cure. We report a case of primary splenic hairy cell leukaemia in which clinical and haematological remission were achieved after splenectomy, and we review the literature.     

The worcester venous thromboembolism study

BACKGROUND: While there have been marked advances in diagnostic and therapeutic strategies for venous thromboembolism, our understanding of its clinical epidemiology is based on studies conducted more than a decade ago. OBJECTIVE: The purpose of this observational study was to describe the incidence and attack rates of venous thromboembolism in residents of the Worcester Statistical Metropolitan Area in 1999. We also describe demographic and clinical characteristics, management strategies, and associated hospital and 30-day outcomes. DESIGN AND MEASUREMENTS: The medical records of all residents from Worcester, MA (2000 census=477,800), diagnosed with International Classification of Diseases, 9th revision (ICD-9) codes consistent with possible venous thromboembolism during 1999 were independently validated, classified, and reviewed by trained abstractors. RESULTS: A total of 587 subjects were enrolled with validated venous thromboembolism. The incidence and attack rates of venous thromboembolism were 104 and 128 per 100,000 population, respectively. Three quarters of patients developed their venous thromboembolism in the outpatient setting - a substantial proportion of these patients had undergone recent surgery or had a recent prior hospitalization. Less than half of the patients received anticoagulant prophylaxis during high-risk periods before their venous thromboembolism. Thirty-day rates of venous thromboembolism recurrence, major bleeding, and mortality were 4.8%, 7.7%, and 6.6%, respectively. CONCLUSION: These data provide insights into recent incidence and attack rates, changing patient profiles, management strategies, and subsequent outcomes in patients with venous thromboembolism. The underutilization of prophylaxis before venous thromboembolism, and relatively high 30-day recurrence rates, suggest a continued need for the improvement of venous thromboembolism prophylaxis and management in the community.     

Eustachian valve thrombus:critical factor in outcome of venous thromboembolism

This case report describes a patient with pulmonary embolism (PE) in whom transesophageal echocardiography showed a thrombus in the right atrium attached to the eustachian valve (EV). The EV is typically absent in the adult, but when present it is considered to be benign. It is an uncommon site for thrombus formation. This patient was treated with systemic anticoagulation.     

A comparison of three rapid D-dimer methods for the diagnosis of venous thromboembolism

We compared three rapid D-dimer methods for the diagnosis of venous thromboembolism. Patients presenting to four teaching hospitals with the possible diagnosis of deep vein thrombosis or pulmonary embolism were investigated with a combination of clinical likelihood, D-dimer (SimpliRED) and initial non-invasive testing. Patients were assigned as being positive or negative for deep vein thrombosis or pulmonary embolism based on their three-month outcome and initial test results. The three D-dimer methods compared were: (a) Accuclot D-dimer (b) IL-Test D-dimer (c) SimpliRED D-dimer. Of 993 patients, 141 had objectively confirmed deep vein thrombosis or pulmonary embolism. The sensitivity of SimpliRED, Accuclot and IL-Test were 79, 90 and 87% respectively. All three D-dimer tests gave similar negative predictive values. The SimpliRED D-dimer was found to be less sensitive than the Accuclot or IL-Test. When combined with pre-test probability all three methods are probably acceptable for use in the diagnosis of venous thromboembolism.     

以肺栓塞为首发表现的高嗜酸粒细胞综合征一例并文献复习

目的 分析高嗜酸粒细胞综合征伴多发血栓形成的临床特征,提高临床对嗜酸粒细胞增多导致血栓性疾病的认识.方法 以“嗜酸粒细胞增高、血小板减少、深静脉血栓形成”为中文关键词,“hypereosinophilic,thrombocytopenia,deep vein thrombosis"为英文关键词检索2002年1月-2012年1月万方数据库和Pebmed数据库的相关文献,共获得9篇,其中中文5篇,英文4篇,排除未明确诊断的2篇文献,共纳入7篇,报道了8例患者,其中男5例,女3例,年龄24-75岁,结合文献报道的病例特点及本病例特征进行文献复习.结果 患者男,27岁,以胸痛气短伴痰中带血起病,经胸部强化CT确诊肺栓塞,同时有下肢深静脉血栓形成,实验室检查提示血嗜酸粒细胞增高和血小板下降.结合文献嗜酸粒细胞增高可损伤血管内皮细胞,导致多发动静脉血栓形成,抗凝同时应用糖皮质激素治疗有效.结论 高嗜酸粒细胞综合征是嗜酸粒细胞增多症的一种,临床以多器官受累为表现,伴多发血栓形成,抗凝治疗同时应用皮质激素可降低嗜酸粒细胞计数,减少其血管内皮损伤对血栓形成的影响.     

Management of venous thromboembolism – controversies and the future

Despite the availability of comprehensive evidence‐based guidelines there are difficult and controversial areas in the management of venous thromboembolism. Institutions and even countries disagree on the importance of calf vein thrombosis, with some rigorously detecting and treating it and others deliberately not looking for it. The need to treat proximal deep vein thrombosis and pulmonary embolism is accepted but which patients with an unprovoked first event should have long‐term anticoagulation has become a difficult clinical decision. We are uncertain how to reduce the incidence of post‐thrombotic syndrome seen in a substantial number of patients. How hard to look for an undiagnosed underlying cancer has become a contentious issue particularly in the United Kingdom following the recent publication of a guideline from the National Institute for Health and Clinical Excellence. Whilst we are wrestling with these dilemmas we are entering an era of new anticoagulants and have to solve the logistical problems of introducing them into clinical practice despite cost pressures. These issues will be explored in this review.     

Duration of anticoagulant therapy for venous thromboembolism: balancing benefits and harms on the long term

Venous thromboembolism (VTE) is effectively treated with anticoagulant therapy. After an initial treatment phase, extended treatment is effective to prevent recurrence after a first event but this is at the expense of a continued risk of bleeding. Ideally, patients at a high risk of recurrence and low risk of bleeding continue anticoagulant therapy, and for those at low risk of recurrence the duration of treatment can be limited. Identifying these patients, however, is difficult. Duration of treatment after a first VTE provoked by a transient risk factor should be limited to 3 months. Although guidelines suggest extended treatment for all patients after unprovoked VTE unless bleeding risk is high, we emphasize that the long-term risks of recurrent VTE off anticoagulation are uncertain whereas the risk of bleeding associated with anticoagulant therapy increases with age. In the absence of evidence of replaced mortality or improved quality of life with extended anticoagulant treatment, we suggest a limited duration for most patients after a first VTE. Extended treatment can be considered, based mainly on patient preference.     

Recent air travel and venous thromboembolism resulting in hospital admission

OBJECTIVE: The objective of the study was to determine the proportion of patients admitted to hospital with a deep vein thrombosis (DVT) or pulmonary embolism (PE) in whom recent air travel was documented in the medical records. METHODS: A retrospective review of the medical records of patients with a primary or secondary discharge diagnosis of DVT or PE in four hospitals in New Zealand was undertaken. From the medical records information was collected on demographic details, documentation of the presence of risk factors and results of radiological investigations. Strict radiological criteria were applied to ensure that the diagnosis of DVT or PE was firmly established. RESULTS: In 60 of 576 (10.4%) patients with a confirmed venous thromboembolism there was documentation of recent air travel; in 31 of these 60 subjects no other risk factors were recorded. In those cases in whom details of the air travel had been recorded, it had been undertaken in the previous 1 week in 65.0%, and in 43.3% the air travel was of at least 10-h duration. CONCLUSION: Long distance air travel is an important risk factor for venous thromboembolism requiring hospital admission and represents a significant public health problem in New Zealand.     

Dural venous sinus thrombosis in a patient with sickle cell disease: case report and literature review

We report a case of dural venous sinus thrombosis (DVST) in a patient who developed seizures following exchange transfusion for treatment of acute chest syndrome associated with sickle cell disease. Evaluation with magnetic resonance imaging and magnetic resonance venography of the brain indicated left sigmoid sinus thrombosis. The history and laboratory evaluation did not reveal any other inherited or acquired hypercoagulable states. This is the fourth case of dural venous sinus thrombosis associated with sickle cell disease reported in literature. The patient had a favorable outcome with early treatment of unfractionated heparin.     

A sickle cell disease patient with dural venous sinus thrombosis: a case report and literature review

Abstract

Dural venous sinus thrombosis (DVST) is a rare disease associated with hypercoagulable states. Patients with sickle cell disease are known to be prothrombotic. We report a case of DVST presenting with anterior neck and facial pain in a 24-year-old female with sickle cell disease, found to have extensive thrombotic disease involving the internal jugular vein. A literature review of DVST in sickle cell disease consisting of 14 case reports was summarized. Headache was a presenting feature in two-thirds of patients. Nine cases were associated with vaso-occlusive crisis (VOC), transfusion, or acute respiratory illness. Most patients were treated with anticoagulation therapy. Over three-quarters either died or suffered from a serious neurological complication, including stroke, seizure, coma, or elevated intracranial pressure. Given its association with life-threatening complications, DVST should be considered when patients with sickle cell disease present with a VOC, especially in the context of headache or neurological deficits.     

CD103-CD23+ classical hair cell leukemia: A case report and review of the literature

Introduction:This case report is presented to improve our understanding of the atypical immunophenotype of hairy cell leukemia.Patient concerns:A 58-year-old woman presented to our department with fatigue for >10 days.Diagnosis:The patient was diagnosed with an increased proportion of abnormal lymphocytes in peripheral blood and bone marrow smear, positive for CD11c, CD19, CD20, CD22, CD25, CD123, CD200, and Kappa, partial expression of CD23, but no expression of CD103, positive for BRAF V600E mutation.Interventions and outcomes:Cladribine combined with rituximab achieved complete remission of minor residual disease negativity.Conclusion:Hairy cell leukemia is rare, and the diagnosis and differential diagnosis should be made by combining the patient''s medical history, clinical manifestations, immunophenotype, gene detection, and other means. Purine nucleoside analogs are the first-line treatments.     

Hairy cell leukaemia, occupation, and smoking

Summary The roles of farm practices, occupational exposures to organic solvents, and ionizing radiation in the risk of hairy cell leukaemia (HCL) were examined in a French hospital-based multicentre case-control study including 291 cases (229 men and 62 women) and 541 controls (425 men and 116 women). No positive association was observed with occupations involving exposure to organic solvents or with self-declared exposures to solvents, but a significant association with self-reported exposure to petrol or diesel was found for men (OR =1–5 CI95% [1–0–2–1]). No association with ionizing radiation was detected. Agriculture employment gave an odds ratio of 1–7 (CI95% [11–2–4]) for men and 2–7 (CI95% [1–1–6–7]) for women. Among men, the association seems to affect farmers rather than agricultural workers. Self-declared exposure to pesticides or bovine cattle breeding was related to HCL risk in both genders. Finally, a significant negative association with smoking was observed in men, with an inverse exposure-risk relationship odds ratios of 0–6, 0–5 and 0–2, respectively, for cumulative consumptions of <10, 10–23 and 24 pack-years), contrasting with an odds ratios clearly <1 in women.     

Update on diagnosis and anticoagulant therapy for venous thromboembolism

Venous thromboembolism (VTE) continues to be a common medical problem requiring the need for rapid treatment with anticoagulant therapy. Until the recent availability of the direct oral anticoagulants for treatment of VTE, the option for oral anticoagulation was limited to warfarin therapy. The addition of these new medications has been welcomed, but has led to added complexities in deciding the most appropriate agent for each patient based on individual risk factors. Furthermore, there are several circumstances where optimal duration of therapy is not well established. This article will focus on the diagnosis of VTE, the choice of anticoagulant and treatment duration.     

Prevalence of APC resistance and its relationship to arterial and venous thromboembolism in a general population sample of elderly Swedish men: The Study of Men Born in 1913

BACKGROUND: Most studies of hereditary resistance to activated protein C (APC resistance) as a risk factor for venous thromboembolism are derived from case-control studies of hospitalized patients, whilst the importance of this condition in the general population has been only sparsely investigated. OBJECTIVE: To study the prevalence of APC resistance and its relationship to morbidity and mortality in a general population sample of elderly men. DESIGN: Cross-sectional and prospective follow-up study. SETTING: General community: The Study of Men Born in 1913. SUBJECTS: A random population sample of 404 men, all 75 years of age. MAIN OUTCOME MEASURES: Four hundred and four men participated in a screening examination in 1988. The APC ratio was analysed in 382 of them. All the men were followed up for 5 years. Medical records were reviewed for all the men with a history of deep vein thrombosis, pulmonary embolism, myocardial infarction or stroke. RESULTS: Twenty-five men (6.5%) were found to have APC resistance. The incidence of venous thromboembolism, myocardial infarction or stroke did not differ between men with or without APC resistance, either retrospectively or during follow-up. Only two men experienced a deep vein thrombosis before the age of 80 and there was no case of pulmonary embolism. Mortality during 5 years of follow-up did not differ between men with and without APC resistance. CONCLUSION: The prevalence of APC resistance was 6.5% in this study of Swedish men. Although the size of the population sample is somewhat small, the study shows that, amongst elderly men, the association between APC resistance and venous thromboembolic disease was weak and men with this hereditary condition did not have any increase in morbidity or mortality compared with men without APC resistance.     

Revised guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant*

The British Committee for Standards in Haematology first produced guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant in 2000. This revision updates those guidelines and covers the areas of diagnosis, treatment and assessment of response to therapy.     

Prolonged seated immobility at work is a common risk factor for venous thromboembolism leading to hospital admission

The role of seated immobility at work in the pathogenesis of venous thromboembolism (VTE) is uncertain. In this case series, 61 patients aged <65 years with a recent admission for deep venous thrombosis and/or pulmonary embolism completed an interviewer-administered questionnaire to obtain information regarding risk factors. Prolonged seated immobility at work in the 4 weeks before the VTE event was defined as being seated at least 8 h in a 24-h period and at least 3 h at a time without getting up, at least 10 h in a 24-h period and at least 2 h at a time without getting up or at least 12 h in a 24-h period and at least 1 h at a time without getting up. The most commonly identified risk factors were family history of VTE (21 of 61, 34%), seated immobility at work (21 of 61, 34%) and a thrombophilic state (19 of 61, 31%). We conclude that prolonged seated immobility at work may represent a common and important risk factor for VTE.