Honeycomb-like structure (HLS) in the left anterior-descending coronary artery-recanalized thrombus demonstrated by intravascular ultrasound (IVUS) in a patient with polycythemia vera and thrombocythemia.

A 71 year old male patient who experienced acute myocardial infarction (AMI) 4 years ago and had a history of polycythemia vera and thrombocythemia was admitted because acute attack of chronic heart failure. Coronary angiography revealed an unusual filling defect in the middle segment of the left anterior descending (LAD) coronary artery and IVUS showed it is a HLS which is different from dissection or woven coronary artery. We review the recent literature of HLS in this article and further investigations are warranted for the optimal management of HLS.

Neuroimaging findings of hypercoagulable states: A case report on polycythemia vera (PV).

Vaso-occlusive events leading to neurological complications are a common cause of morbidity and mortality in Polycythemia Vera (PV). Low prevalence and unfamiliarity of clinicians and radiologists to the neurological manifestations and radiological findings often result in delayed diagnosis of this potentially fatal disease. Awareness of a radiologist to the imaging findings of PV and other hypercoagulable states and their full spectrum of presentation is essential for the prevention of neurological complications. There are not many examples of neurological complication of PV. Here, we report a case of a 58-year-old male who presented with stroke. Initial neuroimaging revealed hyperdense dural venous sinuses, multifocal and multi-territorial synchronous, and metasynchromous ischemic infarctions. Prompt laboratory work-up revealed high hematocrit and low serum erythropoietin levels suggesting PV, which was confirmed on bone marrow biopsy.

Polycythemia vera presenting with pulmonary embolism and splenic infarction: a case report.

Pulmonary embolism and splenic infarction are rare in patients with polycythemia vera. We herein describe a man in his early 60s whose main symptoms were chest tightness, cough, and sputum expectoration. Antibiotics, bronchodilators, and mucoactive agents did not improve his symptoms. Pulmonary artery computed tomography angiography showed pulmonary embolism, and abdominal computed tomography showed multiple hypodense foci in the spleen. Bone marrow aspiration cytology, biopsy, and genetic testing confirmed polycythemia vera. The patient's symptoms were relieved after treatment with hydroxyurea and rivaroxaban. This case emphasizes that although pulmonary embolism and splenic infarction are relatively rare in patients with polycythemia vera, the possibility of polycythemia vera should be considered in clinical practice.

Clinical characteristics and brain MRI findings in myeloproliferative neoplasms.

BACKGROUND: Myeloproliferative neoplasms (MPNs) including polycythemia vera (PV) and essential thrombocythemia (ET) have an increased risk of ischemic stroke. However, little is known about brain morphological changes and the cerebral vasculature in MPNs. The aim of the present study is to clarify the prevalence rates of brain infarcts (BIs) on magnetic resonance imaging (MRI) and to assess the detailed clinical and MRI characteristics in those patients. METHODS: We prospectively enrolled patients with MPNs who underwent brain MRI between September 2017 and June 2019. BI patterns were characterized by the numbers and locations of BIs on MRI. RESULTS: A total of 101 patients were included in the present study. BIs were observed in 23 patients (23%). Multiple logistic regression analysis showed that age > 60 years (odds ratio (OR) 7.34, 95% confidence interval (CI) 1.08-49.7, p = .041) and history of thrombosis (OR 40.6, 95% CI 7.97-207, p < .0001) were independently associated with BIs, but not the JAK2V617F mutation. Of the 23 patients with BIs, eight patients (35%) had multiple territorial infarcts, and large vessel involvement was identified in five patients (22%). Two patients had thrombus formation in large vessels. CONCLUSIONS: Among patients with MPNs who underwent MRI, BIs were observed in 23% of patients followed up in our center. Older age and thrombosis history were independently associated with BIs. Some patients with MPNs may present with distinctive MRI findings including multiple territorial infarcts and thrombus formation in large vessels.

Marcaje de leucocitos con 99mTc-exametazima y policitemia vera: un hallazgo casual / Leucocyte labelling with 99mTc-exametazime and polycythemia vera: a casual finding

La clasificación oficial de la Organización Mundial de la Salud de los tumores de tejidos hematopoyéticos y linfoides de 2016 introduce el concepto de policitemia vera (PV) enmascarada y revela que esta entidad ha sido infradiagnosticada en el pasado. Se presenta el caso de un varón de 49 años, fumador, intervenido de fractura de tobillo hace más de 15 años, remitido para valorar un posible proceso infeccioso asociado. Al no producirse separación previa de los hematíes por sedimentación durante el procedimiento de marcaje de leucocitos con 99mTc-exametazima se revisaron las causas de disminución de la velocidad de sedimentación globular. Entre ellas destacan la poliglobulia y el hábito tabáquico, ambas presentes en el paciente. Se recomendó realización de estudio hematológico que concluyó con el diagnóstico de PV. Las indicaciones del especialista en Radiofarmacia permitieron diagnosticar un caso no identificado hasta entonces, pese a que el paciente presentaba síntomas desde hacía años

The official World Health Organization classification of hematopoietic and lymphoid tissue tumors introduces in 2016 the concept of masked polycythemia vera (PV) and reveals that this entity has been underdiagnosed in the past. We present the case of a 49-year-old man, smoker, operated on for ankle fracture more than 15 years ago, referred to evaluate a possible associated infectious process. As there was no previous separation of the red blood cells by sedimentation during the leukocyte labelling procedure with 99mTc-exametazima, the causes of decreased erythrocyte sedimentation rate were reviewed. These include polyglobulia and smoking, both present in the patient. A haematological study was advised, which concluded with the diagnosis of PV. The indications of the specialist in Radiopharmacy allowed diagnosing a case not identified until then, although the patient had had symptoms for years

Non-Enhanced CT Mimicking Contrast Enhanced CT - A Case Report on Polycythemia.

Diffuse hyperdense cerebral vasculature is sometimes encountered on nonenhanced computed tomography, and polycythemia is one of the conditions which appears the same. The current case report is of a case of 37-year-old female patient arrived with a complaint of feeling severe headache for the past 3 days which was insidious in onset, diffuse in nature and it did not respond to any medication. NECT brain study showed the hyperdense circle of Willis and cerebral venous sinuses. Contrast was not administered for computed tomography study of the brain. On blood investigations, the patient had raised hematocrit level (74%). The hyperdense vessel was due to raised hematocrit (Polycythemia Vera).

Partial empty sella in a woman with cerebral venous sinus thrombosis: A rare presentation of polycythaemia rubra vera.

We report the case of a 59 year old woman who presented with a six week history of worsening bifrontal headache. On CT brain the only abnormal finding was a partially empty sella potentially indicative of increased intracranial pressure. MRI found a large cerebral venous sinus thrombosis in the superior sagittal sinus. Blood tests and a bone marrow biopsy revealed a diagnosis of JAK2 positive primary polycythaemia rubra vera. The lack of sensitivity and specificity of CT in the diagnosis of CVST should engender a low threshold for MRI in patients with risk factors and/or non-diagnostic abnormalities on initial CT. Management of this dual pathology involves both the immediate treatment of the thrombus with heparin bridging to warfarin and the long treatment for polycythaemia involving repeat venesections and cytoreductive therapy.

Cladribine treatment for Erdheim-Chester disease involving the central nervous system and concomitant polycythemia vera: A case report.

Erdheim-Chester disease (ECD), a rare form of non-Langerhans cell histiocytosis, is characterized by the infiltration of foamy CD68+ and CD1a- histiocytes into multiple organ systems. Central nervous system (CNS) involvement has recently been reported to be a poor prognostic factor when treating ECD with interferon alpha. We report the case of a 66-year-old Japanese patient with ECD involving the CNS who harbored the BRAF V600E mutation and also concomitantly developed polycythemia vera with the JAK2 V617F mutation. We confirmed 2-chlorodeoxyadenosine (cladribine) therapy to be effective for the patient in this case.

Thoracic Extramedullary Hematopoiesis Mimicking Metastatic Cancer.

Thoracic extramedullary hematopoiesis (EMH) is a rare manifestation in patients with myeloproliferative neoplasm. A 76-year-old woman with a long-standing history of polycythemia vera presented with a 2-month history of worsening dyspnea and left-sided wheezing. A chest computed tomography showed an ill-defined soft tissue mass encasing the left mainstem bronchus causing airway obstruction, associated with paratracheal and paraesophageal lymphadenopathy. Endobronchial ultrasound-guided fine needle aspiration of the soft tissue mass and mediastinoscopy with excisional biopsy of a paratracheal lymph node demonstrated EMH with increased myeloid blasts. A bone marrow biopsy confirmed postpolycythemic myelofibrosis consistent with progression of polycythemia vera to myelofibrosis. We describe the bronchoscopic management of a case of EMH presenting as a mediastinal mass, mimicking malignancy.

Erdheim-Chester Disease With Multiorgan Involvement, Following Polycythemia Vera: A Case Report.

Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis characterized by the migration and infiltration of lipid-laden CD68, CD1a and S100 histiocytes to various target organs, which leads to the disruption of physiological tissue architecture and reactive fibrosis, and thus impairs organ function.We describe the first case of a patient with Erdheim-Chester disease with multiorgan involvement developed after 6 years from polycythemia vera diagnosis. During the follow-up, an abdominal ultrasound scan revealed the presence of dense, bilateral perinephric infiltration. A computed tomographic guided core biopsy was performed in order to identify the histological nature of this lesion, and a morphological analysis demonstrated the accumulation of foamy histiocytes surrounded by fibrosis. The BRAFV600E mutation was detected, and a diagnosis of Erdheim-Chester disease was made.The extreme rarity of Erdheim-Chester disease strongly suggests the existence of potentially common element(s) that may have contributed to the pathogenesis of both disorders. Obviously, further studies are needed to clarify the mutual roles and effects of JAK2 and BRAF mutations in this patient, as well as their possible therapeutic implications.

Recurrent Stroke with Rapid Development of Intracranial Stenoses in Polycythemia Vera.

Polycythemia vera (PV) is a blood disorder in which the first expression may be an ischemic stroke. Stroke mechanism in PV is usually attributed to a hypercoagulability state and blood stasis. We report a case of a patient with PV presenting with recurrent ischemic stroke associated with the development of large intracranial stenosis in a period of 1 month. Stenosis was associated with microembolic signals detected by transcranial Doppler. One year later and after hematocrit control, stenosis persisted but microembolic signals disappeared. We discuss similar reports in the literature and the possible pathophysiological mechanism of large-vessel damage in these patients.

Coronary microvascular dysfunction due to essential thrombocythemia and policythemia vera: the missing piece in the puzzle of their increased cardiovascular risk?

Myeloproliferative neoplasms are most commonly associated with venous thrombosis. Up to 60% of patients experience a thrombotic event in their lifetimes, including stroke or myocardial infarction. It is unclear whether pathogenetic factors linking essential thrombocythemia (ET) and polycythemia vera (PV) to thrombotic complications do play a role in the risk of coronary artery disease (CAD). We aimed to assess coronary flow reserve (CFR) as a marker of coronary microvascular function in asymptomatic patients with ET and PV. Fifty-two patients with ET (M/F 13/39, age 61 ± 7 years) and 22 patients with PV (M/F 13/9, age 60.4 ± 13 years) without clinical evidence of heart disease, and 50 controls matched for age and gender were studied. None had CAD. All control subjects were asymptomatic with no history of heart disease. CFR in the left anterior descending coronary artery was detected by transthoracic Doppler echocardiography, at rest, and during adenosine infusion. In patients with ET and PV, CFR was lower than in controls (2.9 ± 0.94 and 2.2 ± 0.7 vs. 3.8 ± 0.7, P < 0.004 and P < 0.0001 respectively). The prevalence of CFR ≤ 2.5 was higher in patients with ET (20 cases, 38.5%) and PV (15 cases, 68.2%) compared with controls (4.1%) (P < 0.0001). Severe CFR (CFR < 2) impairment was found in eight patients with ET (15.4%), in nine patients with PV (40.9%), and in none of control subjects. The mutation of JAK2 gene was associated with abnormal CFR. Asymptomatic patients with ET and PV have coronary microvascular dysfunction in the absence of clinical conditions suggesting CAD.

Clinics in diagnostic imaging (146). Polycythaemia vera (PV).

A 42-year-old Chinese man presented with left-sided chest pain and splenomegaly. Full blood count revealed erythrocytosis, while plain radiograph and computed tomography of the abdomen and pelvis revealed hepatosplenomegaly with splenic infarction. Further workup confirmed the diagnosis of polycythaemia vera. Clinical and imaging features of polycythaemia vera, as well as the potential pitfalls in image interpretation, are discussed in this article.

An unknown cause of aortic valve stenosis: polycythemia vera.

Polycythemia vera (PV) is a chronic myeloproliferative disorder characterized by an abnormal increase in red blood cells. The involvement of the heart during the course of the illness represents a common cause of morbidity and it is linked to an increased thrombogenic risk subsequent to higher blood viscosity. In our study we evaluated by echocardiography a PV patient population. Our study enrolled 44 patients affected by PV. 17 of them were women and 27 were men. Mean patient age was 66.7. The average follow-up period was 5 years and the average duration of the illness was 5.7 years, since the time of diagnosis. All patients were evaluated quarterly by a cardiovascular objective examination and an ultrasound of the heart, with regard to platelet count and hematocrit (Ht) variations during the follow-up period, according to the therapy administered. Patients were treated with hydrossiurea and pipobroman and they underwent an eritrocitoapheresis in emergency conditions in which Ht levels rose too much, in spite of the myelosuppressive therapy. The echocardiographic assessment of the heart structure and function by the B mode technique revealed the presence of a sclerocalcific degeneration of the aortic valve in 58% of patients, involving the aortic root more then the valve. An average trans-aortic flow velocity of 1.92 m/s was detected by Doppler technique; a stenosis was demonstrated in 11 patients (25.5% of the entire population). After diagnosing the presence of a stenosis, we researched a possible cause of it. PV is a systemic disease well-known causing coronary thrombosis in a more or less high percentage of patients according to the record of cases taken into account. In our experience, more then thrombotic disease, found only in 13.4% of patients, we detected a high prevalence and incidence of mild to severe aortic stenosis, found in 25.5% of the sample studied. About all possible causes of stenosis, nowadays this results dependent of Ht values at moment of diagnosis, in the light of these results, it is reasonable to infer that aortic valve stenosis could depend by high haemodynamic stress on valve that is characteristic of polycythemic patients without chemotherapy.