scholarly journals Essential thrombocytosis: review of literature with a rare presentation of cerebral thrombosis treated with endovascular therapy

Author(s):  
Aravind V. Datla ◽  
Sibasankar Dalai

Essential thrombocytosis (ET) is a clonal hematopoietic stem cell disorder. Clinically there is an over-production of platelets without a definable cause. ET is a myeloproliferative neoplasm (MPN). It has an incidence of 1 to 2 per 100,000 population and a distinct female predominance. Once considered a disease of the elderly and responsible for significant morbidity due to haemorrhage or thrombosis, it is now clear that ET can occur at any age in adults and often without symptoms or disturbances of hemostasis. ET can cause both thrombosis or haemorrhage and carries a risk of acute leukemic transformation. Being an MPN, in some cases, polycythemia vera (PV) or primary myelo-fibrosis (PMF) can present as ET. Though the average life expectancy only slightly deviates from the standard expected for age-matched individuals, a thorough understanding of its etiopathogenesis, clinical presentation, complication and management can make a world of difference to patients. Thrombosis is one such complication that has a significant impact on the mortality and morbidity of ET. We proposed using endovascular therapy (EVT) as a treatment modality for cerebral venous sinus thrombosis.

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Zhongmou Xu ◽  
Xiang Li ◽  
Dongxia Feng ◽  
Tianyi Wang ◽  
Xiang Xu ◽  
...  

2019 ◽  
Vol 12 (1) ◽  
pp. bcr-2018-225851 ◽  
Author(s):  
Amna Bibi ◽  
Chathuri Liyanapthirana ◽  
Sajjad Khan

This is a case of a female patient presented with a mild headache associated with recurrent episodes of nausea and vomiting, confusion and right-sided hemiparesis, diagnosed of haemorrhagic venous infarction from the thrombosis of left transverse and sigmoid sinus and probably the left vein of Labbe. She had severe microcytic hypochromic anaemia due to iron deficiency, without any other risk factors for cerebral venous thrombosis such as trauma history, infections, coagulation disorders or autoimmune diseases. She had a good prognosis after iron supplementation and anticoagulation therapy.


2021 ◽  
Vol 27 ◽  
pp. 107602962199910
Author(s):  
Huixin Shen ◽  
Xiaoqin Huang ◽  
Chunqiu Fan

Antiphospholipid syndrome (APS) with cerebral venous sinus thrombosis (CVST) is a relatively rare phenomenon, and this observational study aimed to investigate the clinical characteristics of APS patients complicated with CVST. We retrospectively investigated the clinical characteristics of CVST events in APS and compared differential characteristics and associated factors between APS patients with and without CVST. Twenty-one CVST patients with APS were enrolled including 14 females (9.4%) and 7 males (5.8%). The median age and disease duration at onset of CVST was 33 years (IQR 28-48) old and 1.3 months (IQR 0.7-4), respectively. Among APS patients with CVST, 12 (57.1%) cases presented with neurologic symptoms of CVST as the initial manifestation. Onset of CVST was mainly chronic (52.4%). Headache (90.5%) was the most common neurological symptom. The common locations of CVST were transverse sinus (76.2%) and superior sagittal sinus (57.1%), with more frequently (76.2%) dual or multiple sinuses involved. All patients with CVST were treated with anticoagulant, and 5 (23.8%) patients received endovascular therapy. Sixteen (84.2%) patients had good outcomes and 3 (15.8%) patients died at last follow-up. There were no significant differences ( P > 0.05) between two groups in the analysis of related APS indicators. There were no significant differences ( P > 0.05) between two groups in the analysis of related APS indicators. Although APS complicated with CVST is rare and predominately chronic developed. The evaluation of CVST should be performed for APS patients with intracranial hypertension syndrome. The routine screening of antiphospholipid antibodies (aPLs) is highly recommended in unexplained CVST patients. Most CVST patients with APS will have a good prognosis after treatment, and endovascular therapy is an alternative treatment.


2020 ◽  
Vol 26 (3) ◽  
pp. 341-345
Author(s):  
Yoshihisa Fukushima ◽  
Kenji Takahashi ◽  
Ichiro Nakahara

Introduction Heparin-induced thrombocytopenia (HIT) is an immune-mediated complication of heparin exposure. A limited number of studies have reported cerebral venous sinus thrombosis accompanied by heparin-induced thrombocytopenia. Here, we present a case of successful endovascular therapy (EVT) without periprocedural heparinization in this situation. Case presentation A 47-year-old woman taking an oral contraceptive was admitted to our hospital with severe headache to be diagnosed as cerebral venous sinus thrombosis. Initially, she got improved by medical treatment with intravenous unfractionated heparin. However, she rapidly developed disturbance of consciousness and right hemiplegia due to cerebral venous sinus thrombosis accompanied by heparin-induced thrombocytopenia on the 14th hospital day. She underwent emergent EVT by immediate conversion of anticoagulation from heparin to argatroban. Despite a large clot burden, sufficient recanalization and anterograde venous drainage were re-established by combined EVT including aspiration, balloon sinoplasty, and local thrombolysis with urokinase infusion. She got improved immediately after the intervention and discharged home without any neurological sequelae after two months. Conclusion This unique case of cerebral venous sinus thrombosis worsened by occurrence of heparin-induced thrombocytopenia during the treatment finally resulted in excellent outcome highlights effectiveness of emergent endovascular intervention for critical cerebral venous sinus thrombosis resistant to initial medical treatment and of immediate establishment of effective anticoagulant strategy for both of heparin-induced thrombocytopenia and cerebral venous sinus thrombosis. Neuroendovascular therapy for cerebral venous sinus thrombosis using alternative argatroban to heparin for periprocedural anticoagulation might be safe and feasible.


2021 ◽  
Author(s):  
Yanan Ren ◽  
Tao Quan ◽  
Yueyuan Xie ◽  
Qi Ren ◽  
Xiaoyong Zhang ◽  
...  

Abstract Purpose Clinical approach for staging cerebral venous sinus thrombosis (CVST) depends on the duration from symptom onset to clinical diagnosis, which is subjective and may be incorrect. To investigate if T1-weighted signal characteristics of the thrombus can be used for staging the thrombus on segment level and predicting endovascular therapy outcome. Methods Ninety-one CVST patients underwent non-contrast-enhanced T1-weighted 3D variable-flip-angle fast-spin-echo (T1-SPACE) and 29 of them received endovascular therapy. We divided the 91 patients into acute, subacute, and chronic CVST stage according to the clinical approach, and then analyzed the T1 weighted signal characteristics (i.e., thrombus shape and signal intensity) of the thrombus at each stage. To investigate the endovascular therapy outcome associated with the thrombus stage and signal characteristics, we evaluated the thrombolytic ratio on patient level and recanalization rate on segment level, respectively. Results The thrombi at acute or chronic stage were depicted as isointense signals but as hyperintense signals at subacute stage. Compared to the thrombus at acute stage, the thrombus at chronic stage was contractive and can be distinguished. Developing thrombi at transition periods of acute-to-subacute or subacute-to-chronic stage were found appearing mixed iso-/hyper-intense signals. On patient’s level, the patients at earlier stage had higher thrombolytic ratio (acute: 80.7 ± 18.3%, subacute: 62.6 ± 36.3%, chronic: 22.9 ± 24.2%). On segment level, the thromboembolic segments of isointense or mixed iso-/hyper-intense thrombi at non-chronic stage had good thrombolytic outcome. Conclusion T1-weighted signal characteristics of the thrombus can be used for staging CVST and predicting the endovascular therapy outcome.


2016 ◽  
Vol 12 (7) ◽  
pp. 611-621 ◽  
Author(s):  
Nosha Farhadfar ◽  
Sonia Cerquozzi ◽  
Mrinal Patnaik ◽  
Ayalew Tefferi

Myelofibrosis is a myeloproliferative neoplasm with cardinal features of extramedullary hematopoiesis, hepatosplenomegaly, cytopenias, and constitutional symptoms that result in shortened survival and leukemic transformation. It is a disease predominantly of the elderly, and currently available therapies only offer symptom control without curative benefit or ability to alter disease progression. Allogeneic hematopoietic stem-cell transplant (HSCT) is the only potentially curative intervention; however, this is only feasible in younger and medically fit patients and selectively offered to those with high-risk disease. Despite ongoing advancements, HSCT is associated with substantial morbidity and mortality, and the determination of which patients with myelofibrosis are ideal candidates and the selection of the opportune moment to proceed with transplantation remains challenging. This review summarizes our current recommendations for the role of and indications for HSCT in myelofibrosis.


2009 ◽  
Vol 27 (5) ◽  
pp. E3 ◽  
Author(s):  
Aristotelis Filippidis ◽  
Eftychia Kapsalaki ◽  
Gianna Patramani ◽  
Kostas N. Fountas

Cerebral venous sinus thrombosis (CVST) is a rare clinicopathological entity. The incidence of CVST in children and neonates has been reported to be as high as 7 cases per million people, whereas in adults the incidence is 3–4 cases per million. The predisposing factors to this condition are mainly genetic and acquired prothrombotic states and infection. The clinical picture of CVST is nonspecific, highly variable, and can mimic several other clinical conditions. Diagnosis of CVST is established with the implementation of neuroimaging studies, especially MR imaging and venography. Identification and elimination of the underlying cause, anticoagulation, proper management of intracranial hypertension, and anticonvulsant prophylaxis constitute cornerstones of CVST treatment. Newer treatment strategies such as endovascular thrombolysis and decompressive craniectomy have been recently used in the treatment of patients with CVST with variable success rates. Further clinical research must be performed to delineate the exact role of these newer treatments in the management of severe cases of CVST. The recent advances in the diagnosis and treatment of patients with CVST have significantly lowered the associated mortality and morbidity and have improved the outcome of these patients.


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