Intrapetrous internal carotid artery dissection and essential thrombocythemia: what relationship? A case report
- Daniele D'Ambrosio†1,
- David Della-Morte†1, 2Email author,
- Gaetano Gargiulo1,
- Marianna Rossetti1,
- Tatjana Rundek2,
- Franco Rengo1 and
- Pasquale Abete1
© D'Ambrosio et al; licensee BioMed Central Ltd. 2008
Received: 07 August 2008
Accepted: 27 November 2008
Published: 27 November 2008
Internal carotid artery (ICA) dissection is responsible for 10–20% of strokes in young and middle-aged patients. Isolated ICA dissection involving the intrapetrous carotid canal is particularly rare, and no case has been reported to describe an association between intrapetrous ICA dissection and essential thrombocythemia. We report a case of ischemic stroke in the presence of intrapetrous right ICA dissection and essential thrombocythemia. The diagnosis of essential thrombocythemia was performed by bone marrow biopsy. The essential thrombocythemia may cause endothelial dysfunction and predispose to vascular damage such as carotid artery dissection.
Internal carotid artery (ICA) dissection is responsible for less than 2% of all ischemic strokes, and for 10–20% of strokes in young and middle-aged patients, with a peak incidence in the fifth decade . Isolated ICA dissection involving the intrapetrous carotid canal is particurarly rare . Although not completely known, the main risk factors related to carotid artery dissection are genetic and environmental factors, traumatic events, cervical manipulations, migraine, recent infections, hyperhomocysteinemia, and hereditary connective-tissue disorders . We report a case of ischemic stroke of a patient with intrapetrous ICA dissection and essential thrombocythemia.
Spontaneous dissections of the ICA have often a good prognosis, but ischemic cerebrovascular events occur frequently: these lesions remain a major cause of stroke in young and middle-aged patients . The immobility of the petrous portion fixed in the carotid canal makes the intrapetrous internal carotid artery dissection a rare event. Although thromboembolism is supposed to be the main stroke mechanism in ICA dissection , haemodynamic change can also play an important role, especially in cases of intracranial dissection . Ischemic stroke attributed to essential thrombocythemia was found in 0.4% of cases, as reported in the Lausanne Stroke Registry . Haematological disorders were identified as the definitive cause of cerebrovascular disease in 0.1% – 0.8% of cases in general stroke registries [5, 6]. In our patient, the diagnosis of essential thrombocythemia was established after occurring episodes of stroke, right ICA dissection, coronary heart disease and portal vein thrombosis, all described as complications of this disease, except for dissection .
Essential thrombocythemia is a characterized by a high platelet count, originating from a pluripotent stem cell and it is diagnosed at a rate of about 2–3/100.000 individuals annually, with a slight female preponderance (1.5–2.1). The disease usually affects middle aged to elderly individuals, with an average age at diagnosis of 50–60 years. Approximately half of the patients are asymptomatic while the other half have vasomotor, thrombotic or hemorrhagic disturbances. Typical essential thrombocythemia is a Philadelphia bcr-abl negative (Ph1-) CMPD with a good prognosis and overall survival. For a long time, the Polycythemia Vera Study Group (PVSG) criteria for the diagnosis of ET have not included histopathological data. Recently, a new World Health Organization (WHO) classification for CMPD has been developed, incorporating clinical, laboratory and morphologic data along with fresh knowledge and techniques . The clinical picture of essential thrombocythemia is dominated by a predisposition to vascular occlusive events and haemorrhages .
In our patient we observed a progressive increase of platelet count and various complications, typical of essential thrombocythemia. We performed a bone marrow biopsy and the study of BCR-ABL gene rearrangement and we excluded causes of reactive thrombocytosis. It is unclear whether there is a relationship between intrapetrous internal carotid artery dissection and essential thrombocythemia. An endothelial damage, due to the activation of leukocytes and the consequent release of elastase and alkaline phosphatase, is observed in essential thrombocythemia and it seems to play a major role about the pathogenesis of the thrombophilic state, including elevated levels of platelet-specific proteins, increased tromboxane generation and expression of activation-dependent epitopes on platelet surface . The association between vasculitis disorders determining stroke, such as moyamoya disease, that affects the cerebral blood vessels mimicking sometimes a spontaneous internal carotid artery dissection on Doppler ultrasound, and essential thrombocythemia is described .
It is much more likely that thrombocytosis and elevated homocysteine, because they both increase thrombosis, make dissections more likely to be symptomatic, than that they actually predispose to dissection. Probably there are many patients with dissection that is asymptomatic; those with increased thrombosis are more likely to have symptoms and thereby be diagnosed.
In summary, this case shows that essential thrombocythemia might predispose to vascular dysfunction and damage such as carotid artery dissection. Whether this association is casual or causal remains a matter of speculation. However, essential thrombocythemia should be excluded in the presence of carotid artery lesions and thrombocytosis.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Philadelphia Chronic myeloproliferative disease
internal carotid artery
National Institute of Health Stroke Scale
Philadelphia bcr-abl negative
percutaneous transluminal coronary angioplasty
Polycythemia Vera Study Group
World Health Organization.
- Schievink WI: Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med. 2001, 344: 898-906. 10.1056/NEJM200103223441206.View ArticlePubMedGoogle Scholar
- Huang YC, Chen CM, Lai SL, Lee TH, Chen ST, Chin SC, Chen YC: Spontaneous intrapetrous internal carotid artery dissection: a case report and literature review. J Neurol Sci. 2007, 253: 90-3. 10.1016/j.jns.2006.11.019.View ArticlePubMedGoogle Scholar
- Rubinstein SM, Peerdeman SM, van Tulder MW, Riphagen I, Haldeman S: A systematic review of the risk factors for cervical artery dissection. Stroke. 2005, 36: 1575-80. 10.1161/01.STR.0000169919.73219.30.View ArticlePubMedGoogle Scholar
- Benninger DH, Georgiadis D, Kremer C, Studer A, Nedeltchev K, Baumgartner RW: Mechanism of ischemic infarct in spontaneous carotid dissection. Stroke. 2004, 35: 482-5. 10.1161/01.STR.0000109766.27393.52.View ArticlePubMedGoogle Scholar
- Bogousslavsky J, Van Melle G, Regli F: The Lausanne Stroke Registry: analysis of 1,000 consecutive patients with first stroke. Stroke. 1988, 19 (9): 1083-1092.View ArticlePubMedGoogle Scholar
- Hart RG, Kanter MC: Hematologic disorders and ischemic stroke. A selective review. Stroke. 1990, 21 (8): 1111-1121.View ArticlePubMedGoogle Scholar
- Michiels JJ, Berneman Z, Bockstaele DV, Planken van der M, De Raeve H, Schroyens W: Clinical and laboratory features, pathology of platelet-mediated thrombosis and bleeding complications and the molecular etiology of essential thrombocythemia and polycythemia vera: therapeutic implications. Semin Thromb Hemost. 2006, 32: 174-207. 10.1055/s-2006-939431.View ArticlePubMedGoogle Scholar
- Thiele J, Kvasnicka HM: Chronic myeloproliferative disorders. The new WHO classification. Pathologe. 2001, 22: 429-43. 10.1007/s002920100492.View ArticlePubMedGoogle Scholar
- Falanga A, Marchetti M, Evangelista V, Vignoli A, Licini M, Balicco M, Manarini , Finazzi G, Cerletti C, Barbui T: Polymorphonuclear leukocyte activation and hemostasis in patients with essential thrombocythemia and polycythemia vera. Blood. 2000, 96: 4261-6.PubMedGoogle Scholar
- Kornblihtt LI, Cocorullo S, Miranda C, Lylyk P, Heller PG, Molinas FC: Moyamoya syndrome in an adolescent with essential thrombocythemia: successful intracranial carotid stent placement. Stroke. 2005, 36 (Suppl 8): 71-3. 10.1161/01.STR.0000174193.89864.55.View ArticleGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.