Introduction

Polycythemia vera (PV) is a rare hematologic disorder characterized by clonal proliferation of bone marrow progenitors.4) The clinical features of PV are highly variable. Patients may present with the classical findings of increased red blood cells, white blood cells, and platelet counts and splenomegaly, or with any combination of these or even myelofibrosis.11) Thrombotic and hemorrhagic complications are the major causes of the morbidity and mortality in PV.8) We report a case of PV initially presenting with intraventricular hemorrhage (IVH) and ischemic heart disease.

Case Report

A 43-year-old man presented to the emergency department with severe headache, left hemiparesis; Grade III, chest discomfort and dyspnea. The patient had no medical history. The initial brain computed tomography (CT) scan and CT angiography revealed an IVH in all ventricles (Fig. 1) but no evidence of cerebral vascular malformations. The patient slipped into a stupor after undergoing the brain CT scan, necessitating an emergency external ventricular drainage.

On initial laboratory testing, erythrocytosis (hematocrit: 57.7%) and leukocytosis (12,580 /mm3) were noted. There was no cause of secondary erythrocytosis. These hematologic evaluation confirmed PV.

The patient had a sinus tachycardia and ST elevation on electrocardiography (ECG) and cardiac enzymes were elevated (Troponin-T: 2.85 ng/mL, CKMB: 62.03 ng/mL). Due to the patient? severe hypotension, vasopressor therapy was initiated. An echocardiography for evaluation of heart function revealed a left ventricular ejection fraction of 20% and global hypokinesia of the left ventricle, indicative of ischemic heart disease as a complication of PV.

The patient underwent repeated phlebotomy for 3 days. The hematocrit decreased from 57.5% to 43.7%. On day 7 after admission, the patient? condition improved with no focal neurologic sign. A follow-up brain CT showed totally resolved IVH. But, a follow-up echocardiography after 6 days showed residual decreased left ventricular ejection fraction of 39% and global hypokinesia

Discussion

PV was first described in 189212) and further elaborated as a disease entity in 1903.6) In 1951, the clinical and bone marrow histologic similarities among PV, myeloid metaplasia, essential thrombocythemia, chronic myeloid leukemia, and erythroleukemia were recognized, resulting in the grouping of these diseases together as myeloproliferative disorders.3) Patients present most commonly with thrombosis or hemorrhage.9) Quantitative platelet derangements may play a major role in the development of thrombosis and ischemia and thus precipitate a stroke, peripheral arterial or venous thrombosis, or myocardial infarction. The hemorrhagic tendency in PV may be related to decreased platelet adhesion, defective platelet aggregation, chronic disseminated intravascular coagulation with excess production of hyperaggregated platelets, or the release of ineffective platelets. These platelet abnormalities may also predispose the patient to bleeding at virtually any site.7)

Primary in IVH, bleeding in the ventricular system without a discernable parenchymal fragment is a rare neurological disorder. The incidence of primary IVH among all patients with intracranial hemorrhage is 3.1%.10) Primary IVH has been described with vascular malformations such as arteriovenous malformations, aneurysms, arteriovenous fistulae, and Moyamoya disease.10) Hypertension is one of the most commonly associated risk factors for primary IVH.2)

 The present case indicates that PV is another cause for primary IVH. The patient showed cardiac dysfunction. Some reports have described neurogenic stunned myocardium, which is defined as myocardial injury and dysfunction occurring after diverse types of acute brain injury as a result of imbalance of the autonomic nervous system. The spectrum of observed cardiac abnormalities includes ECG changes, arrhythmia, myocardial necrosis, release of B-type natriuretic peptide, and both systolic and diastolic dysfunction of the left ventricle.5) Two features are key to the identification of neurogenic stunned myocardium. First, the post-ischemic dysfunction is fully reversible. Second, the dysfunction is not caused by a primary defect in myocardial perfusion. Most of the situations in which neurogenic stunned myocardium occurs involve brief occlusion of the coronary arteries.1) However, the present patient displayed continuous cardiac dysfunction, which is indicative of ischemic heart disease.

Conclusion

We report a rare case of primary IVH with ischemic heart disease as the initial presentation of PV. When intracranial hemorrhage with ischemic disease occurs, it may indicate PV. PV should be considered based on laboratory findings and treated with proper management.

REFERENCES

  1)    Bolli R, Marban E. Molecular and cellular mechanisms of myocardial stunning. Phygiol Rev 79:609-34, 1999

  2)    Caplan LR. Primary intraventricular haemorrhage, in Kase CS, Caplan LR: Intracerebral hemorrhage. London, Butterworth-Heinemann, 1994, pp 383-401

  3)    Dameshek W. Some speculations on the myeloproliferative syndromes. Blood 6:372-375, 1951

  4)    Gruppo Italiano Studio Policitemia. Polycythemia vera: The natural history of 1213 patients followed for 20 years. Ann Intern Med 123:656-664, 1995

  5)    Nguyen H, Zaroff JG. Neurogenic stunned myocardium. Curr Neurol Neruosci Rep 9(6):486-91, 2009

  6)    Osler W. Chronic cyanosis, with polycythemia and enlarged spleen: A new clinical entity. Am J Med Sci 126: 187-201, 1903

  7)    Paolo V, Gerald C. Polycythemia and the heart. Texas Heart Institute Journal 21:198-201, 1994

  8)    Pearson TC. Hemorheologic considerations in the pathogenesis of vascular occlusive events in polycythemia vera. Semin Thromb Hemost 23:433-9, 1997

  9)    Randi ML, Stocco F, Rossi C, Tison T, Girolami A. Thrombosis and hemorrhage in thrombocytosis: Evaluation of a large cohort of patient. J Med 22:213-23, 1991

10)    Semih G, Orhan S, Fevzi BS, Kadir T, Mehmet K, Basak KG, et al. Spontaneous primary intaventricular hemorrhage in adults: Clinical data, etiology and outcome. Turkish Neurosurgery 4:338-44, 2009

11)    Tefferi A, Spivak JL. Polycythemia vera: Scientific advances and current practice. Semin hematol 42(4):206-20, 2005

12)    Vaquez MH. Sur une forme speciale de cyanose s’accompagnant d’hyperglobulie excessive et persistante. CR Soc Biol(Paris) 44:384-388, 1892