Delayed Manifestation and Slow Progression of Cerebral Infarction Caused by Polycythemia Rubra Vera

Kurabayashi, Hitoshi; Hishinuma, Akiko; Uchida, Ryohei; Makita, Shigeru; Majima, Mitsuru

doi:10.1097/maj.0b013e31805370a9

Cited by 6 publications

(3 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…While these vascular problems are systemic and affect many organs, they can have devastating consequences in the brain. In both ET and PV, occlusion of brain blood vessels is a frequent complication that leads to transient ischemic attacks and cerebral infarcts . Obstructions in small brain vessels, which have not been observed directly, could also have a significant impact on neuronal health, even in the absence of acute symptoms.…”

Section: Introductionmentioning

confidence: 99%

Stalled cerebral capillary blood flow in mouse models of essential thrombocythemia and polycythemia vera revealed by in vivo two‐photon imaging

Santisakultarm

Paduano

Stokol

et al. 2014

Journal of Thrombosis and Haemostasis

View full text Add to dashboard Cite

To cite this article: Santisakultarm TP, Paduano CQ, Stokol T, Southard TL, Nishimura N, Skoda RC, Olbricht WL, Schafer AI, Silver RT, Schaffer CB. Stalled cerebral capillary blood flow in mouse models of essential thrombocythemia and polycythemia vera revealed by in vivo two-photon imaging. J Thromb Haemost 2014; 12: 2120-30.Summary. Background: Essential thrombocythemia (ET) and polycythemia vera (PV) are myeloproliferative neoplasms (MPNs) that share the JAK2 V617F mutation in hematopoietic stem cells, leading to excessive production of predominantly platelets in ET, and predominantly red blood cells (RBCs) in PV. The major cause of morbidity and mortality in PV and ET is thrombosis, including cerebrovascular occlusive disease. Objectives: To identify the effect of excessive blood cells on cerebral microcirculation in ET and PV. Methods: We used two-photon excited fluorescence microscopy to examine cerebral blood flow in transgenic mouse models that mimic MPNs. Results and conclusions: We found that flow was 'stalled' in an elevated fraction of brain capillaries in ET (18%), PV (27%), mixed MPN (14%) and secondary (non-MPN) erythrocytosis (27%) mice, as compared with controls (3%). The fraction of capillaries with stalled flow increased when the hematocrit value exceeded 55% in PV mice, and the majority of stalled vessels contained only stationary RBCs. In contrast, the majority of stalls in ET mice were caused by platelet aggregates. Stalls had a median persistence time of 0.5 and 1 h in ET and PV mice, respectively. Our findings shed new light on potential mechanisms of neurological problems in patients with MPNs.

show abstract

Section: Introductionmentioning

confidence: 99%

Stalled cerebral capillary blood flow in mouse models of essential thrombocythemia and polycythemia vera revealed by in vivo two‐photon imaging

Santisakultarm

Paduano

Stokol

et al. 2014

Journal of Thrombosis and Haemostasis

View full text Add to dashboard Cite

show abstract

“…Polycythemia vera has a risk of various ischemic strokes such as watershed infraction, lacunar infraction, trunk artery occlusion, and slow progressive infarction [11]. Previous reports have indicated that the pathogenesis of cerebral infarction secondary to polycythemia vera could be caused by embolic infarct, hemodynamic infarct, and prothrombotic state [12]. …”

Section: Discussionmentioning

confidence: 99%

Malignant Hemispheric Cerebral Infarction Associated with Idiopathic Systemic Capillary Leak Syndrome

Miyata

Mikami²,

Mikuni³

et al. 2013

Case Rep Neurol

View full text Add to dashboard Cite

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare condition that is characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse general swelling, fetal hypovolemic shock, hypoalbuminemia, and hemoconcentration. Although ISCLS rarely induces cerebral infarction, we experienced a patient who deteriorated and was comatose as a result of massive cerebral infarction associated with ISCLS. In this case, severe hypotensive shock, general edema, hemiparesis, and aphasia appeared after serious antecedent gastrointestinal symptoms. Progressive life-threatening ischemic cerebral edema required decompressive hemicraniectomy. The patient experienced another episode of severe hypotension and limb edema that resulted in multiple extremity compartment syndrome. Treatment entailed forearm and calf fasciotomies. Cerebral edema in the ischemic brain progresses rapidly in patients suffering from ISCLS. Strict control of fluid volume resuscitation and aggressive diuretic therapy may be needed during the post-leak phase of fluid remobilization.

show abstract

“…Several biochemical processes related to the hypercoagulable state of malignancy have been suggested. These include change in the homeostatic property of blood and blood vessels that involve activation of cell adhesion molecules by mucin secreted from adenocarcinomas ( 113 , 114 ), release of tissue factors by cancer cells causing activation of factor VII and X ( 115 – 118 ), endothelial cell damage from procoagulant cytokines such as TNF-alpha, IL-1, and IL-6, which cause vWF release ( 117 , 118 ), platelet activation, protein C inhibition ( 119 ), intravascular lymphomatosis ( 120 ), and increased viscosity observed in myeloproliferative disorders ( 121 ).…”

Section: Possible Etiologies Of Esus and Csmentioning

confidence: 99%

Embolic Strokes of Unknown Source and Cryptogenic Stroke: Implications in Clinical Practice

et al. 2016

View full text Add to dashboard Cite

Up to a third of strokes are rendered cryptogenic or of undetermined etiology. This number is specifically higher in younger patients. At times, inadequate diagnostic workups, multiple causes, or an under-recognized etiology contributes to this statistic. Embolic stroke of undetermined source, a new clinical entity particularly refers to patients with embolic stroke for whom the etiology of embolism remains unidentified despite through investigations ruling out established cardiac and vascular sources. In this article, we review current classification and discuss important clinical considerations in these patients; highlighting cardiac arrhythmias and structural abnormalities, patent foramen ovale, paradoxical sources, and potentially under-recognized, vascular, inflammatory, autoimmune, and hematologic sources in relation to clinical practice.

show abstract

Delayed Manifestation and Slow Progression of Cerebral Infarction Caused by Polycythemia Rubra Vera

Cited by 6 publications

References 10 publications

Stalled cerebral capillary blood flow in mouse models of essential thrombocythemia and polycythemia vera revealed by in vivo two‐photon imaging

Stalled cerebral capillary blood flow in mouse models of essential thrombocythemia and polycythemia vera revealed by in vivo two‐photon imaging

Malignant Hemispheric Cerebral Infarction Associated with Idiopathic Systemic Capillary Leak Syndrome

Embolic Strokes of Unknown Source and Cryptogenic Stroke: Implications in Clinical Practice

Contact Info

Product

Resources

About