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OBJECTIVES: Polycythaemia vera (PV) is a condition that may potentially put patients undergoing cardiac surgery at an increased risk of bleeding and thrombosis; however, there is currently a paucity of literature regarding the management of these patients. We aim to examine the literature in this systematic review to indicate the interventions that may be considered to minimise complications. METHODS: We conducted a literature search using keywords and MeSH terms to identify articles discussing PV and cardiac surgery. The studies were identified and qualitatively analysed using the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) protocol. RESULTS: In total, 10 case reports representing 11 patients were identified for this systematic review and were included in qualitative analysis. 63.6% of patients had preoperative intermittent phlebotomy, and the majority of patients received postoperative therapy that involved one antiplatelet agent and one anticoagulant. Generous perioperative fluid management, phlebotomy, preservation of core body temperature, early extubation, monitoring of myocardial ischaemia, infarction and vascular events, intense chest physiotherapy and patient mobilisation are important to consider to reduce the risk of complications arising from surgery. CONCLUSION: These considerations should be systematically discussed in a multidisciplinary team, where the acute surgical need can be balanced appropriately against the risk of haemorrhage and thrombosis.

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Cefazolin is an antibiotic that is commonly administered perioperatively to reduce the risk of surgical site infections. Cephalosporins have a well-established safety profile, but have been associated with thrombocytopenia and neutropenia due to their myelosuppressive effects. While this effect may be benign in healthy patients undergoing minor surgery, it can be detrimental in patients with underlying hematologic disorders presenting for open-heart surgery. Herein, we discuss the first case in the literature of cefazolin-induced thrombocytopenia and severe coagulopathy in a patient with polycythemia vera (PCV) during a coronary artery bypass-grafting surgery.

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BACKGROUND: Sodium-glucose co-transporter-2 inhibitors are novel antidiabetes drugs that act via inhibition of renal glucose reabsorption. This action causes osmotic diuresis, reduces intravascular volume and is associated with various adverse effects. In the present paper, we describe the first report on the unmasking of underlying polycythemia vera by canagliflozin in a person with Type 2 diabetes mellitus, which was temporally related to the use of the drug. CASE REPORT: A 51-year-old obese man with Type 2 diabetes was prescribed canagliflozin 100 mg for control of his glycaemia. He presented 6 months later with asymptomatic elevation of his haemogram measurements (haemoglobin: 16.9 g/dl; haematocrit: 55%; red cell number: 8.1 million/mm3 ; total leukocytes: 23010/mm3 ; platelet count: 9.7 *106 /mm3 ). He had no history of smoking, exposure to high altitude or other drugs. Subsequent investigations revealed myeloproliferative neoplasm (polycythemia vera) on trephine biopsy of bone marrow, normal erythropoietin level and JAK2V617F positivity. Because of the possibility that the underlying condition had been unmasked by canagliflozin, the latter was stopped. This led to a remarkable improvement in the man's haematological profile, with no other significant intervention. The man subsequently restarted the drug of his own accord, causing his haematological profile to worsen again and thereby posing a challenge in monitoring of both polycythemia vera as well as diabetes mellitus. CONCLUSION: This report brings to light unmasking of a new adverse effect of sodium-glucose co-transporter-2 inhibitors in clinical practice caused by volume loss, apart from hypotension and falls.

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The coexistence or the development of Philadelphia chromosome-negative myeloproliferative neoplasms after a lymphoproliferative disease in the same patient is an extremely rare event. We report the case of a 72-year-old man who developed JAK2V617F polycythemia vera 3 years after the diagnosis and treatment of primary diffuse large B cell non-Hodgkin's lymphoma of the central nervous system. We also review the literature regarding the pathogenesis underlying the association of myeloproliferative and lymphoproliferative chronic disorders.

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Polycythemia vera (PV), a hematologic stem cell disorder characterized by predominant erythroid proliferation, is extremely rare in childhood. Some PV patients develop acute leukemia, especially acute myelogenous leukemia, but cases of PV occurring after treatment of acute leukemia are rare. The authors describe a girl with an atrioventricular canal who was diagnosed with acute lymphoblastic leukemia (ALL) at 23 months of age, was cured with chemotherapy, and developed PV 7 years later. She went on to develop hepatic complications of PV that culminated in death from liver disease at 20 years of age, without recurrence of ALL.

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To date, the diagnosis of polycythemia vera (PV) relies on clinical criteria. We have recently described the overexpression of a hematopoietic receptor, polycythemia rubra vera-1 (PRV-1), in patients with PV. Here, we report a quantitative reverse transcriptase-polymerase chain reaction (RT-PCR) assay for the measurement of PRV-1 mRNA levels. We have determined PRV-1 expression in 71 patients with PV, 11 patients with secondary erythrocytosis (SE), as well as in 80 healthy controls. PV patients express significantly higher amounts of PRV-1 than healthy controls or patients with SE (P <.0001). Because there is no overlap between the PRV-1 expression in PV patients versus healthy controls or SE patients, the assay has a very high sensitivity and specificity for the diagnosis of PV in our population. In patients with erythrocytosis, the quantitative RT-PCR assay described here therefore provides a rapid, highly specific and sensitive tool for the diagnosis of PV.

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In November 1981, a 77-yr-old woman consulted for myeloid metaplasia with fibrosis. A persistent hyperleucocytosis was treated with hydroxyurea from March, 1985 to March, 1989. At that time facial dyskinesia and polycythaemia developed. Investigations revealed a regression of myelofibrosis and a predominance of myeloid metaplasia in the liver. The mechanism of this event, rarely observed in myeloproliferative syndromes, is discussed in this new case.

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A case of typical idiopathic myelofibrosis turning into polycythaemia vera 6 years after diagnosis is reported. This transformation occurred after treatment with busulphan. The present case is added to similar previous observations to give further support to the contention that the transition of chronic idiopathic myelofibrosis to polycythaemia vera may be a consequence, though rare, of therapy rather than a spontaneous event occurring during the course of the disease.

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Among 246 patients (49 with Hodgkin's disease, 29 with multiple myeloma, 75 with other lympho- and immunoproliferative syndromes, 70 with carcinomas and 23 with non-malignant affections) treated by cytostatic or immunosuppressive chemotherapy, 6 developed malignant hemopathy (acute myeloblastic leukemia, erythroleukemia and erythremia) apparently induced during the last 7 1/2 years. In addition, 2 carcinomas have been noted. All have received melphalan or chlorambucil, alone or associated with other cytostatic drugs. 5 out of these 6 patients also underwent radiotherapy. The lenght of chemotherapy ranged between 7 and 110 months and the latency between 45 and 110 months. A "preleukemic" cytopenia phase was observed in 4 out of 6 cases. An exceptional 45-month survival was secured in case 1 (acute myeloblastic leukemia in a patient probably cured of Hodgkin's disease IIIB). Observation 2 is the 3rd case ever published of induced acute leukemia in disseminated lupus erythematosus. All these observations are compared with the latest findings in the literature. To the very extent that the utilization of cytostatic drugs produces improvement in the prognosis of very serious diseases, their leukemogenic potential becomes more dangerous and demands limitation of their use.

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