骨髓 障碍
Overview
骨髓增殖性肿瘤, 或mpn，也称为骨髓增生性疾病, or MPDs — are a collection of blood disorders that are believed to be caused by mutations in bone marrow stem cells. 这些干细胞通常会产生血液中的成熟细胞, 比如红细胞, 它们携带氧气, 白细胞, 可以对抗感染, 和血小板, 哪些能促进血液凝固. 在MPN中，突变导致受影响的干细胞产生异常数量的这些细胞. 在美国有超过20万人.S. 和MPN一起生活的人.
一般来说，mpn与某些成熟血细胞类型的过量产生有关. Polycythemia vera is an MPN associated with too many red blood cells; essential thrombocythemia refers to the abnormal overproduction of platelets, 和 慢性髓性白血病 是否与白细胞过多有关.
Many people with these conditions have few, if any, 症状, but complications can occur, including 中风 疾病控制不佳的患者. mpn有时可以转换为 白血病 or to myelofibrosis, an MPN that is characterized by excessive scar-type tissue in the bone marrow.
The molecular causes of the majority of MPN cases have been identified — mutations in the genes JAK2 和 ABL are found in nearly all patients with polycythemia vera 和 慢性髓性白血病, 分别. This knowledge has led to the development of targeted therapies that shut off the activity of these overactive mutant proteins. 在慢性髓性白血病的情况下, 结果，病人的前景发生了巨大的变化.
我们治疗骨髓增生性疾病的方法
UCSF is dedicated to delivering the most advanced treatments for myeloproliferative disorders with care 和 compassion. 骨髓增生性疾病有几种类型, 最好的治疗取决于类型和病人的症状. 一般来说，治疗的目标是使血细胞恢复到正常水平. 病人 who aren't experiencing 症状 may not need immediate treatment but require careful monitoring.
We are also dedicated to discovering better treatments for myeloproliferative disorders through research. Interested patients may have the option to participate in clinical trials of potential new therapies.
奖 & 识别
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是全国顶尖的十大赌博平台排行榜之一
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北加州最好的，排名第一. 全国癌症治疗排名第七
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每年进行血液和骨髓移植
迹象 & 症状
病人 with MPN may have many or few 症状 depending on how abnormal their blood counts become.
Those with essential thrombocythemia 和 polycythemia vera have "thick" blood that is more prone to clotting 和 is associated with heart attacks, 中风 还有腿部静脉血栓. 病人 with chronic myelogenous 白血病 和 myelofibrosis often feel tired 和 report pain or fullness in their belly from an enlarged spleen.
MPN患者可能有中度或重度贫血, 没有足够的红细胞携带氧气的情况. 其他体征和症状可能包括:
- 呼吸急促用力时呼吸急促
- 虚弱和疲劳
- 苍白的皮肤
- 食欲不振
- 由于血小板计数低，小伤口持续出血
- Purpura, a condition in which the skin bleeds, causing black 和 blue or pin-sized spots on the skin
- 由于白细胞计数低而引起的鼻窦、皮肤或泌尿系统感染
治疗
尽管骨髓增生性肿瘤通常无法治愈, 所有患有这种疾病的患者都有治疗方法. mpn的治疗取决于类型和症状的存在. 一般来说，治疗的目的是纠正异常的血细胞计数.
有些人没有任何症状，因此不需要治疗.
低剂量化疗药片, 比如水螅, can be given to patients with essential thrombocythemia 和 polycythemia vera to lower their platelet 和 red blood cell counts. Phlebotomy (the removal of blood intravenously) is also an effective strategy for lowering red blood cell counts 和 controlling 症状.
慢性粒细胞白血病的治疗选择是一种新的抗白血病药物, 叫做格列卫. 睾酮治疗有时可以改善骨髓纤维化患者的贫血. 骨髓纤维化的其他潜在治疗方法包括化疗, 放射治疗, 沙利度胺和干扰素. 此外，骨髓纤维化患者经常需要输注红细胞.
化疗
化疗使用药物杀死体内多余的血细胞. 化疗 may be taken orally in a pill format or may be administered intravenously (through an IV). 化疗被称为全身治疗，因为药物进入血液, 在体内传播，可以杀死全身的细胞.
放射治疗
Radiation therapy uses high-energy X-rays to kill cells 和 is sometimes used to relieve 症状 of MPN. 外束放射治疗, 它使用体外的机器来控制辐射, 通常是哪种类型的放射治疗用于治疗MPN. 除了, a radioactive drug called P32 can be given intravenously to lower the number of red blood cells made by the bone marrow.
手术
切除脾脏的手术, 叫做脾切除术, 如果病人的脾脏肿胀，可以进行手术吗.
生物治疗
生物疗法作为一种可能的治疗MPN的方法正在试验中. 这种疗法使用身体或实验室制造的材料来促进, 指导或恢复身体的免疫系统. Biological therapy sometimes is called biological response modifier (BRM) therapy or immunotherapy.
激素
在某些情况下，激素可用于治疗MPN的副作用. 在不明原因的骨髓化生患者中, 可以使用一种叫做糖皮质激素的激素来延长红细胞的寿命. 除了, hormones called 和rogens sometimes are used to stimulate the bone marrow to produce more blood cells.
A person's recovery or response to therapy depends on their type of MPN, age 和 general health. Most people with essential thrombocythemia 和 polycythemia vera live more than 10 to 15 years with few complications. 骨髓纤维化患者的寿命约为5年，在某些情况下, 这种病可能发展成急性白血病.
干细胞移植
治疗MPN(不包括慢性骨髓性白血病)的唯一有效方法是 异体干细胞移植即将干细胞从一个人转移到另一个人.
Whether stem cell transplantation is an appropriate therapy for an individual patient depends on a number of factors including age, 或然数子类型, 目前的症状或疾病的严重程度以及能否获得供体. We encourage patients to consult with a stem cell transplantation doctor to learn more about MPN, 可能的治疗和干细胞移植.
加州大学旧金山分校健康医学专家已经审查了这些信息. It is for educational purposes only 和 is not intended to replace the advice of your doctor or other health care provider. 我们鼓励您与您的供应商讨论您可能遇到的任何问题或疑虑.
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