Headache in essential thrombocythaemia

Frewin, Rebecca; Dowson, A.

doi:10.1111/j.1742-1241.2012.02986.x

Cited by 13 publications

(19 citation statements)

References 46 publications

(90 reference statements)

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“…Chronic brain ischemia often manifests itself with headache and/or asthenia [ 7 ]. There exists a firm link between hematological characteristics and cephalgia, supported by several studies [ 8 , 9 ] where it has been demonstrated that in PV and ET, headache is often a first symptom of Ph-MPD [ 10 ]. Our findings confirm these data, with as much as 70% of patients referring to their physician with headache as the main complaint (which preceded the diagnosis of Ph-MPD).…”

Section: Discussionmentioning

confidence: 99%

Clinical Characteristics of Cerebrovascular Pathology with Patients Suffering from Ph-Negative Myeloproliferative Disease

Tanashyan

Кузнецова

Шабалина

et al. 2016

Cerebrovasc Dis Extra

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Background: Disturbances of microcirculation play a significant role in the development and progression of both acute and chronic cerebrovascular diseases (CVD) and may be associated with different hemogram abnormalities. One of the reasons of the prothrombogenic state of the endothelium is the increase in the number of blood corpuscles leading to (non-Ph) myeloproliferative disorders (MPD) including essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PM). Materials and Methods: The study included 167 patients: 102 patients with Ph-MPD and the control group comprising 65 patients with CVD. According to MPD subtype, the patients were divided into three groups: patients with ET (37%, n = 38, male/female 7/31, age 52 ± 7 years), those with PV (40%, n = 41, male/female 20/21, age 50 ± 6 years) and those with PM (23%, n = 23, male/female 5/18, age 54 ± 4 years). Results: In 79% (n = 81) of cases in the study group (with Ph-MPD), patients had chronic CVD, with the most frequently identified symptoms being asthenia (92%) and headache (72%). Headache in Ph-MPD patients was more frequently (86%) associated with PM, while in patients with PV and ET it was equally distributed (70%). Neurological symptoms in 53% of cases were associated with focal changes of the brain on MRI localized in the subcortical area of the frontal and parietal lobes. Twenty-one (21%) patients suffered an acute cerebrovascular accident, 8 of them had thrombotic occlusion of one of the internal carotid arteries leading to hemispheric infarcts. Endothelial function (as measured by flow-dependent dilation of the brachial artery) was severely impaired in all study groups (median 5% with normal cut-off at 10%), the lowest degree of vasodilator activity being specific for patients with a history of stroke (p = 0.011). Conclusion: Patients suffering from MPD had asymptomatic focal changes in the brain in the absence of concomitant vascular disease (hypertension, atherosclerotic vascular disease, heart rhythm disorders) in 50% of cases. MPD, while remaining un- or underdiagnosed, presents a major concern in the cerebrovascular setting. A large number of thrombotic strokes occurring in patients with ET underline the necessity of early diagnostics and preventive therapy in these patients.

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Section: Discussionmentioning

confidence: 99%

Clinical Characteristics of Cerebrovascular Pathology with Patients Suffering from Ph-Negative Myeloproliferative Disease

Tanashyan

Кузнецова

Шабалина

et al. 2016

Cerebrovasc Dis Extra

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“…The association of JAK2 V617F -mutated thrombocythemia in ET and PV patients who have a high risk of aspirin-responsive platelet thrombophilia with a broad spectrum of microvascular circulation disturbances including erythromelalgic ischemic complications and MIAs is being increasingly recognized (fig. 4) [23,24,25,26]. Similar to the platelet-mediated arteriolar painful thrombotic and inflammatory processes that occur in the peripheral and cerebral endarterial circulation (erythromelalgia and MIAs), this thrombocythemia also occurs in the coronary (acute coronary syndromes) and liver circulation (Budd-Chiari syndrome), the adrenal microcirculation and the microcirculation of the skin of the legs and arms (superficial thromboangiitis) [1,22,25].…”

Section: Discussionmentioning

confidence: 99%

“…This broad spectrum of microvascular circulation disturbances and the subsequent major thrombotic complications have been observed in JAK2 V617F - and MPL 515 -mutated thrombocythemia and in JAK2 wild type thrombocythemia [23,24]. If the disturbances are not recognized due to unfamiliarity with thrombocythemia-specific manifestations, and when treatment with aspirin is not received, ET and PV patients are at very high risk of major arterial thrombosis including TIAs, stroke, angina pectoris, myocardial infarction and digital gangrene [23,24,25,26,27,28]. …”

Section: Discussionmentioning

confidence: 99%

Aspirin-Responsive, Migraine-Like Transient Cerebral and Ocular Ischemic Attacks and Erythromelalgia in JAK2<sup>V617F</sup>-Positive Essential Thrombocythemia and Polycythemia Vera

et al. 2014

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Migraine-like cerebral transient ischemic attacks (MIAs) and ocular ischemic manifestations were the main presenting features in 10 JAK2^V617F-positive patients studied, with essential thrombocythemia (ET) in 6 and polycythemia vera (PV) in 4. Symptoms varied and included cerebral ischemic attacks, mental concentration disturbances followed by throbbing headaches, nausea, vomiting, syncope or even seizures. MIAs were frequently preceded or followed by ocular ischemic events of blurred vision, scotomas, transient flashing of the eyes, and sudden transient partial blindness preceded or followed erythromelalgia in the toes or fingers. The time lapse between the first symptoms of aspirin-responsive MIAs and the diagnosis of ET in 5 patients ranged from 4 to 12 years. At the time of erythromelalgia and MIAs, shortened platelet survival, an increase in the levels of the platelet activation markers β-thromboglobulin and platelet factor 4 and also in urinary thromboxane B2 were clearly indicative of the spontaneous in vivo platelet activation of constitutively JAK2^V617F-activated thrombocythemic platelets. Aspirin relieves the peripheral, cerebral and ocular ischemic disturbances by irreversible inhibition of platelet cyclo-oxygenase (COX-1) activity and aggregation ex vivo. Vitamin K antagonist, dipyridamole, ticlopidine, sulfinpyrazone and sodium salicylate have no effect on platelet COX-1 activity and are ineffective in the treatment of thrombocythemia-specific manifestations of erythromelalgia and atypical MIAs. If not treated with aspirin, ET and PV patients are at a high risk of major arterial thrombosis including stroke, myocardial infarction and digital gangrene.

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“…Por este motivo se ha propuesto en la práctica clínica, que los pacientes que inician los episodios de cefalea en relación temporal con el diagnóstico de TE y si éstos episodios se resuelven en el espacio de tres meses de tratamiento con dosis bajas de Ácido acetil salicílico (AAS) o terapia citoreductora, entonces puede considerarse la cefalea como manifestación de la TE (15).…”

Section: Introduccionunclassified