Pancytopenia

全血细胞减少症
  • 文章类型: Case Reports
    伊马替尼是酪氨酸激酶抑制剂(TKI),并且是用于治疗慢性粒细胞性白血病(CML)的常用药物。再生障碍性贫血是格列卫非常罕见的并发症,文献中只有少数病例报道。我们介绍了一例63岁的亚洲女性,她开始使用伊马替尼治疗CML,细胞计数反应良好。格列卫开始四个月后,患者因极度疲劳入院,并出现严重的全血细胞减少症。患者接受多次输血。最后,患者接受了骨髓活检,这表明对骨髓细胞明显减少的再生障碍性贫血的关注。格列卫举行了会议,监测血细胞计数,并给予了支持性护理。患者的血细胞计数恢复缓慢。关于这一主题的数据仍然很少,并且没有预测TKI治疗骨髓抑制的标准。我们的病例报告旨在再次强调需要增加对TKI治疗骨髓抑制的研究。
    Imatinib is a tyrosine kinase inhibitor (TKI) and is a commonly used medication for treatment of chronic myelogenous leukemia (CML). Aplastic anemia is a very uncommon complication of Gleevec, and only a few cases are reported in the literature. We present a case of a 63-year-old Asian female who was initiated on imatinib for treatment of CML with good response in cell counts. Four months after Gleevec initiation, the patient was admitted to the hospital with extreme fatigue and noted to have severe pancytopenia. Patient received multiple blood transfusions. Finally, the patient underwent bone marrow biopsy, which showed concern for aplastic anemia with marked hypocellular bone marrow. Gleevec was held, blood counts were monitored, and supportive care was given. Patient had slow recovery of her blood counts. There remains scarcity of data on this topic and no criteria exist to predict the myelosuppression with TKI therapy. Our case report aims to reemphasize the need for increased research on myelosuppression with TKI therapy.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    视神经脊髓炎谱系障碍(NMOSD)合并干燥综合征(SS)和全血细胞减少症的患者极为罕见。目前尚无关于此类患者治疗的研究。我们介绍了一例AQP4-IgG血清阳性的难治性NMOSD患者合并SS和全血细胞减少症,对inebilizumab有显着反应。2017年,这名49岁的女性患者在没有任何治疗的情况下被诊断为SS和全血细胞减少症。2022年8月,她突然出现下肢无力,表现为无法行走,伴有尿失禁。接受甲基强的松龙和环磷酰胺后,她恢复了走路的能力。2023年2月,她再次下肢无力,瘫痪在床,伴有尿液和粪便滞留,和双眼视力丧失。在接受甲基强的松龙和三次血浆置换后,情况没有进一步恶化,但没有缓解.2023年3月,患者入院,正式诊断为AQP4-IgG血清阳性NMOSD合并SS和全血细胞减少症。在接受两次300mg的钠珠单抗注射后,不仅NMOSD症状明显改善,同时伴有SS和全血细胞减少的症状。在AQP4-IgG血清阳性NMOSD反复发作并与其他自身免疫性疾病并存的情况下,inebilizumab可能是一个不错的选择。
    Patients with neuromyelitis optica spectrum disorder (NMOSD) coexisting with both Sjögren\'s syndrome (SS) and pancytopenia are exceptionally rare. There is no study on the treatment of such patients. We presented a case of AQP4-IgG seropositive refractory NMOSD patient combined with SS and pancytopenia with significant response to inebilizumab. In 2017 the 49-year-old female patient was diagnosed with SS and pancytopenia without any treatment. In August 2022, she had a sudden onset of lower limbs weakness, manifested as inability to walk, accompanied by urinary incontinence. After receiving methylprednisolone and cyclophosphamide, she regained the ability to walk. In February 2023, she suffered from weakness of both lower limbs again and paralyzed in bed, accompanied by retention of urine and stool, and loss of vision in both eyes. After receiving methylprednisolone and three plasmapheresis, the condition did not further worsen, but there was no remission. In March 2023, the patient was admitted to our hospital and was formally diagnosed with AQP4-IgG seropositive NMOSD combined with SS and pancytopenia. After receiving two 300 mg injections of inebilizumab, not only the symptoms of NMOSD improved significantly, but also the symptoms of concurrent SS and pancytopenia. In the cases of AQP4-IgG seropositive NMOSD who have recurrent episodes and are comorbid with other autoimmune disorders, inebilizumab may be a good choice.
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  • 文章类型: Case Reports
    方法:一名21岁女性患者出现严重的下腰痛4个月。在检查中,病人发热,脸色苍白,和两个骶髂(SI)关节的压痛。病人正在入院和评估,在评估过程中,出现严重头痛,强度严重,并伴有恶心和抛射性呕吐。初步调查:双侧SI关节的X线检查显示炎症,抗核抗体(ANA)为4+,全血细胞减少和乳酸脱氢酶(LDH)升高,但肝功能检查正常.其余的风湿病特征不明显。在评估过程中,她出现了严重的头痛,which,关于成像,显示存在脑水肿伴慢性硬膜下血肿,同时进行的凝血功能障碍检查显示有弥散性血管内凝血(DIC)的证据。
    结论:考虑到整个情况,怀疑是体内的恶性过程,和血清肿瘤标志物癌胚抗原(CEA),糖类抗原19-9(CA19-9),和癌症抗原125(CA-125)被发送,所有这些都被提高了。验证临床线索是对全血细胞减少症进行的骨髓活检,显示恶性上皮浸润。做了对比增强计算机断层扫描(CECT)胸部和整个腹部,以找出主要的,显示胃食管交界处有肿瘤肿块,椎骨和左肾上腺骨转移。通过上消化道内窥镜检查从原发病灶取组织进行组织病理学检查(HPE)。尽管HPE显示III级低分化胃腺癌,当诊断出来时,患者已经屈服于疾病过程。
    结论:简而言之,这个案例完美地说明了实体器官恶性肿瘤可能是多系统疾病的模仿者,从而延迟诊断并进一步恶化预后。
    METHODS: A 21-year-old female patient presented to us with severe low back pain for 4 months. On examination, patient was afebrile, with severe pallor, and tenderness in both sacroiliac (SI) joints. Patient was being admitted and evaluated, and during the course of evaluation, developed severe headache, which was severe in intensity and associated with nausea and projectile vomiting. Initial investigations: An X-ray of the bilateral SI joints revealed inflammation, and the antinuclear antibody (ANA) turned out to be 4+ with pancytopenia and raised lactate dehydrogenase (LDH), but the liver function tests were normal. Rest of the rheumatological profile was unremarkable. During the course of the evaluation, she developed a severe headache, which, on imaging, showed presence of cerebral edema with chronic subdural hematoma, and a concomitant coagulopathy workup revealed evidence of disseminated intravascular coagulation (DIC).
    CONCLUSIONS: Taking the whole picture into consideration, a malignant process in the body was suspected, and serum tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA-125) were sent, all of which were raised. Validating the clinical clue was the bone marrow biopsy done for pancytopenia, which revealed malignant epithelial infiltration. A contrast-enhanced computed tomography (CECT) thorax and whole abdomen were done to find out the primary, which showed a neoplastic mass at the gastroesophageal junction along with bony metastases in the vertebrae and left adrenal. Tissue from the primary lesion was taken for histopathological examination (HPE) through upper gastrointestinal endoscopy. Although HPE revealed grade III poorly differentiated stomach adenocarcinoma, the patient had succumbed to the disease process by the time the diagnosis came to light.
    CONCLUSIONS: In short, this case perfectly illustrates how solid organ malignancies might be a mimicker of multisystem disorders, thereby delaying diagnosis and worsening the prognosis even further.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    甲氨蝶呤是一种抗炎和免疫调节药物,广泛用于中度至重度银屑病和其他风湿病,如类风湿性关节炎,除了某些类型的恶性肿瘤.副作用在高急性剂量中更为普遍,但在低剂量慢性使用中也可以看到,尤其是在药物剂量错误的情况下。可能的毒性症状包括胃肠道,肝,血液和肾功能障碍,但也可能包括粘膜炎和银屑病病变的恶化。这里,我们描述了一例涉及甲氨蝶呤毒性的老年银屑病患者,详细的管理。
    Methotrexate is an anti-inflammatory and immunomodulatory drug, widely used for moderate to severe psoriasis and other rheumatological conditions such as rheumatoid arthritis, besides some types of malignancies. Side effects are more prevalent in high acute doses but can also be seen in low-dose chronic use, especially in cases of drug-dosing errors. Possible symptoms of toxicity include gastrointestinal, hepatic, hematologic and renal dysfunctions, but may also include mucositis and worsening of the psoriatic lesions. Here, we describe a case involving methotrexate toxicity in an elderly patient with psoriasis, detailing the management.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    骨髓凝胶状转化(GTBM)是一种罕见的血液学疾病,其中骨髓中的造血细胞被细胞外凝胶状物质取代,经常导致血细胞减少。这种情况的真实发生率目前尚不清楚,因为目前的文献主要由病例报告组成。然而,对大型骨髓登记的分析表明,即使在需要骨髓活检的人群中,这也是一种非常罕见的实体。我们介绍了一个24岁的男性,有弥漫性大B细胞淋巴瘤和45公斤体重减轻的病史,后来被发现患有GTBM。他的血细胞减少程度导致住院时间延长,并伴有许多并发症,最终导致异基因干细胞移植(ASCT)的实验治疗。据我们所知,这是首例GTBM病例,其中ASCT被用作潜在治疗方式.虽然我们的患者在ASCT后确实有临床改善,这些结果的持久性目前尚不清楚。此外,不确定ASCT是否真的是患者稳定的原因.鉴于此,我们目前无法主张ASCT作为GTBM的治疗方法.我们报告此病例是为了在难治性血细胞减少症的背景下提高对这种罕见实体的认识。
    Gelatinous transformation of bone marrow (GTBM) is a rare hematologic condition in which hematopoietic cells in the bone marrow are replaced by extracellular gelatinous substances, often resulting in cytopenias. The true incidence of this condition is presently unknown, as the current body of literature primarily consists of case reports. However, an analysis of a large bone marrow registry suggests that this is a highly rare entity even among a population requiring bone marrow biopsy. We present a case of a 24-year-old man with a history of diffuse large B cell lymphoma and an associated 45-kilogram weight loss, who was later found to have GTBM. The extent of his cytopenias resulted in a prolonged hospitalization with numerous complications, eventually leading to experimental treatment with allogeneic stem cell transplantation (ASCT). To our knowledge, this is the first reported case of GTBM in which ASCT was employed as a potential treatment modality. While our patient did have clinical improvement following ASCT, the permanence of these results is presently unclear. Furthermore, it is uncertain if the ASCT was truly causative of the stabilization of the patient. Given this, we are currently unable to advocate for ASCT as a treatment for GTBM. We report this case to raise awareness of this rare entity in the context of refractory cytopenias.
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