secondary polycythemia

  • 文章类型: Case Reports
    肌瘤样红细胞增多综合征是由子宫平滑肌瘤演变而来的继发性红细胞增多症的罕见现象。尽管潜在的病理学仍然未知,患者静脉血栓形成的风险增加.44岁的GO(gravida零)偶然发现继发性红细胞增多症,由于她的子宫肌瘤很大,因此诊断为肌瘤样红细胞增多症综合征。发生肺栓塞和硬脑膜窦静脉血栓形成后,她接受了治疗性抗凝治疗。随后,她做了子宫动脉栓塞术,这导致她的促红细胞生成素(8.1mU/mL)以及血红蛋白(15.1g/dL)和血细胞比容(45g/dL)大幅下降。肌瘤样红细胞增多综合征可引起静脉血栓形成,导致神经系统并发症.在手术风险增加的患者中,子宫动脉栓塞是一种有效的治疗选择。
    Myomatous erythrocytosis syndrome is a rare phenomenon of secondary polycythemia evolving from uterine leiomyoma. Although the underlying pathology is still unknown, patients have an increased risk of venous thrombosis. A 44-year-old GO (gravida zero) presented with an incidental finding of secondary polycythemia, and a diagnosis of myomatous erythrocytosis syndrome was made because of her large uterine fibroids. She was placed on therapeutic anticoagulation after developing pulmonary embolisms and a dural sinus venous thrombosis. Subsequently, she underwent uterine artery embolization, which resulted in a substantial decrease in her erythropoietin (8.1 mU/mL) along with hemoglobin (15.1 g/dL) and hematocrit (4 5g/dL). Myomatous erythrocytosis syndrome can cause venous thrombosis, leading to neurologic complications. In patients with increased risk for surgery, uterine artery embolization is an effective option for treatment.
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    文章类型: Case Reports
    继续保持其作为第六大最常见的癌症原因和第三大癌症死亡原因的地位,全球,肝细胞癌(HCC)仍然是医疗保健的优先事项。各种物质的产生可能导致全身或代谢并发症,通常被称为肝癌的副肿瘤现象。一名56岁的男性,有未经治疗的慢性乙型肝炎病史,在入院前的最后两天出现全身无力和间歇性头痛。实验室结果显示血红蛋白升高(20.5g/dl),甲胎蛋白(29,845ng/dl),和d-二聚体(2,120ng/ml)水平。低血糖症(44mg/dl)记录与正常的基础胰岛素水平,确认非胰岛细胞瘤低血糖。腹部多相CT扫描显示大面积实性病变累及整个右肝叶,在动脉阶段过度增强,在静脉和延迟阶段冲洗模式,门静脉血栓形成;因此,确认HCCBCLCC。进一步检查显示骨髓活检细胞过多,没有JAK2突变。他接受了连续放血,口服80毫克乙酰水杨酸,以及降低血栓形成风险的细胞还原剂。尽管应用了不同的干预措施,如果不进行高葡萄糖负荷的肠胃外给药,则无法实现低血糖的控制。他计划接受口服多激酶抑制剂,然而,他因严重的医院获得性肺炎而去世。副肿瘤现象在HCC中很常见。红细胞增多症引起的血液高粘度和血栓形成的风险增加,以及由低血糖引起的医疗紧急情况表明,这两种情况不应被忽视,因为它们可能会恶化患者的预后。
    Continuously holding its position as the sixth most common cause of cancer and the third leading cause of cancer death, globally, Hepatocellular Carcinoma (HCC) remains as a healthcare priority. Production of various substances may result into systemic or metabolic complications, often known as paraneoplastic phenomena of HCC. A 56-year-old male with history of untreated chronic hepatitis B arrived with generalized weakness and intermittent headache in the last two days prior to admission. Laboratory findings demonstrated elevated hemoglobin (20.5 g/dl), alpha-fetoprotein (29,845 ng/dl), and d-Dimer (2,120 ng/ml) levels. Hypoglycemia (44 mg/dl) was documented with normal basal insulin level, confirming non-islet cell tumor hypoglycemia. Abdominal multiphasic CT-scan demonstrated a large solid lesion involving the whole right liver lobe, hyper-enhanced at arterial phase and wash-out pattern at venous and delayed phases, with portal vein thrombosis; thus, confirming HCC BCLC C. Further examinations revealed hypercellularity from bone marrow biopsy with the absence of JAK2 mutation. He underwent serial phlebotomy and received 80 mg acetylsalicylic acid orally, as well as cytoreductive agent to reduce the risk of thrombosis. Despite applications of different interventions, control of hypoglycemia could not be achieved without parenteral administration of high dextrose load. He was planned to receive oral multikinase inhibitor, however, he passed away due to severe hospital-acquired pneumonia. Paraneoplastic phenomena are common in HCC. Increased risk of blood hyper-viscosity and thrombosis attributed to polycythemia, as well as medical emergency resulting from hypoglycemia showed that both conditions should not be overlooked since they may worsen the patient\'s prognosis.
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  • 文章类型: Case Reports
    真性红细胞增多症(PV)是由世界卫生组织(WHO)标准2016诊断的骨髓增殖性肿瘤(MPN)之一,需要存在3个主要标准:高血红蛋白/血细胞比容,骨髓检查结果,和Janus激酶2(JAK2)突变或两个主要和一个次要标准,包括促红细胞生成素(EPO)水平。然而,在临床实践中,由于可能被吸烟或阻塞性睡眠呼吸暂停(OSA)等红细胞增多的继发原因所掩盖,因此可能出现诊断困难.
    这里,我们报道了一位55岁的绅士,在家庭持续气道正压通气(CPAP)机上患有OSA的病态肥胖,偶然发现患有红细胞增多症。进一步的评价证实了PV的诊断。
    PV可以通过基于历史的继发性红细胞增多症的假设来掩盖。这强调了通过JAK2和EPO测试筛选此类队列以避免遗漏PV的重要性。
    UNASSIGNED: Polycythemia vera (PV) is one of the myeloproliferative neoplasms (MPN) diagnosed by World Health Organization (WHO) criteria 2016, which requires the presence of 3 major criteria: high hemoglobin/hematocrit, bone marrow findings, and Janus Kinase 2 (JAK2) mutation or two major and one minor criteria, including erythropoietin (EPO) level. However, in clinical practice, difficulties in diagnosis can arise as it may be masked by secondary causes for erythrocytosis such as smoking or obstructive sleep apnea (OSA).
    UNASSIGNED: Here, we report a 55-year-old gentleman, morbidly obese with OSA on home continuous positive airway pressure (CPAP) machine, who was incidentally found to have polycythemia. Further evaluation confirmed the diagnosis of PV.
    UNASSIGNED: PV can be masked by the assumption of secondary polycythemia based on history. This underscores the importance of screening such cohort through JAK2 and EPO testing to avoid missing PV.
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  • 文章类型: Case Reports
    法洛氏四联症是一种紫红色先天性心脏病,如果不及时治疗会导致成年后死亡。在极少数情况下,如果存活下来,这可能会由于慢性缺氧而导致继发性红细胞增多症,并可能出现多种不明显的症状,从而导致诊断困境。
    作者介绍了一例罕见的继发性红细胞增多症病例,在50年代早期被诊断为具有独特的非对比计算机断层扫描头颅表现,表现出一系列长期症状和神经系统表现史。
    在成年期被诊断为红细胞增多症的患者可能具有未经治疗的先天性心脏病的背景,可以通过在资源贫乏的环境中进行适当的放射学调查来进一步补充。
    年龄超过50岁的先天性心脏病继发红细胞增多症是罕见的。这些患者的表现可能会给临床医生带来诊断挑战,可以通过适当了解特殊的非对比计算机断层扫描头部发现来缓解这种挑战。
    UNASSIGNED: Tetralogy of Fallot is a cyanotic congenital heart disease which if untreated leads to death by adulthood. In rare cases who survive this can lead to secondary polycythemia due to chronic hypoxia who can present with multiple indistinct symptoms leading to diagnostic dilemma.
    UNASSIGNED: The authors present a rare case of secondary polycythemia diagnosed in early fifties with the unique non-contrast computerized tomography head findings who presented with a spectrum of long standing symptoms and a history of neurological manifestations.
    UNASSIGNED: Patients diagnosed with polycythemia in adulthood can have a background of an untreated congenital heart disease, which can be complemented further by appropriate radiological investigations in a resource poor setting.
    UNASSIGNED: Polycythemia secondary to an untreated congenital heart disease in the age beyond 50 is a rare occurrence. The presentation of these patients might present clinicians with a diagnostic challenge which can be mitigated with an appropriate knowledge of the peculiar non-contrast computerized tomography head findings.
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  • 文章类型: Case Reports
    背景:继发性红细胞增多症被认为是慢性缺氧的常见并发症。理论上可以提高载氧能力,但是这种适应性特征有有害影响,因为血液粘度增加,会导致显著的发病率和死亡率,如中风和心肌梗塞。
    方法:一名55岁的男性,有先天性小主肺动脉病史,出现在急诊科,行走不稳定,头晕和眩晕。评估显示血红蛋白升高和上后循环脑动脉血栓形成。患者接受高通量氧气吸入和抗血小板聚集治疗。
    结论:在慢性缺氧病例中很少报道脑血管受累。本病例是首例先天性小主肺动脉患者由于慢性缺氧而导致的上后循环脑动脉血栓形成。该病例表明认识到一些可导致缺氧和继发性红细胞增多症从而导致高凝状态和随后的血栓形成的慢性疾病的重要性。
    BACKGROUND: Secondary polycythemia is considered the usual complication of chronic hypoxia. It can theoretically increase the oxygen-carrying capacity, but this adaptive trait has a deleterious effect because the blood viscosity increases, which can induce significant morbidity and mortality, such as stroke and myocardial infarction.
    METHODS: A 55-year-old man with a history of a congenitally small main pulmonary artery presented to the emergency department with sustained unsteady walking, dizziness and vertigo. Evaluation revealed elevated hemoglobin and superior posterior circulation cerebral artery thrombosis. The patient was treated with high flux inhalation of oxygen and anti-platelet aggregation.
    CONCLUSIONS: The involvement of cerebral vessels has rarely been reported in chronic hypoxia cases. The present case is the first case of superior posterior circulation cerebral artery thrombosis due to chronic hypoxia in a patient with a congenitally small main pulmonary artery. This case demonstrates the importance of recognizing some chronic diseases that can lead to hypoxia and secondary polycythemia thereby leading to hypercoagulable state and subsequent thrombosis.
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  • 文章类型: Case Reports
    此案是一名53岁的妇女。出生时,她被诊断为假Taussig-Bing异常,伴有肺动脉狭窄和单心室。然而,没有进行心脏手术,即使成年后,心血管外科医生仍继续保守治疗。由于继发性红细胞增多症和多发性脑梗死的病史,她服用了抗血小板药物和抗凝剂。然而,她第四次被诊断为脑梗塞。据认为,该患者因心脏畸形伴发先天性心脏病伴凝血异常而处于矛盾脑栓塞的高风险中。考虑到病理生理学,我们决定联合使用阿司匹林和华法林。
    The case was a 53-year-old woman. At birth, she was diagnosed with a false Taussig-Bing anomaly with pulmonary artery stenosis and a single ventricle. However, no cardiac surgery was performed, and conservative treatment was continued by a cardiovascular surgeon even after adulthood. Because of secondary polycythemia and a history of multiple cerebral infarctions, she took anti-platelet drugs and anti-coagulants. However, she was admitted with the diagnosis of cerebral infarction for the fourth time. It was considered that the patient was at high risk of paradoxical cerebral embolism due to cardiac malformation with cyanotic congenital heart disease accompanied by coagulation abnormalities. Considering the pathophysiology, we decided to use aspirin in combination with warfarin.
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  • 文章类型: Case Reports
    儿童肥胖症流行涉及与该临床和公共卫生问题相关的不寻常且未被认可的并发症。肥胖低通气综合征(OHS)被定义为肥胖的三联症,白天通气不足,在没有替代神经肌肉的情况下,睡眠呼吸紊乱,通气不足的机械或代谢解释。我们在此报告一名12岁男孩,他被诊断患有OHS。患者通过静脉切开术和双水平气道正压通气得到改善。据我们所知,这是报告的首例因OHS引起的继发性红细胞增多症,需要进行治疗性静脉切开术.
    The childhood obesity epidemic involves unusual and underrecognized complications associated with this clinical and public health problem. Obesity hypoventilation syndrome (OHS) is defined as the triad of obesity, daytime hypoventilation, and sleep-disordered breathing in the absence of an alternative neuromuscular, mechanical or metabolic explanation for hypoventilation. We herewith report a 12-year-old boy who was diagnosed with OHS. The patient improved with phlebotomy and bi-level positive airway pressure. To the best of our knowledge, this is the first reported case of secondary polycythemia due to OHS requiring therapeutic phlebotomy.
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