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非肝硬化门静脉高压背景下的脾边缘区淋巴瘤

Splenic Marginal Zone Lymphoma in the Setting of Noncirrhotic Portal Hypertension.

作者信息

Ratnayake Saman, Ammar Ali, Rezvani Rodd, Petersen Greti

机构信息

Kern Medical Center, Bakersfield, CA, USA.

出版信息

J Investig Med High Impact Case Rep. 2015 Sep 30;3(4):2324709615609385. doi: 10.1177/2324709615609385. eCollection 2015 Oct-Dec.

DOI:10.1177/2324709615609385
PMID:26904707
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4748510/
Abstract

We present a case of a 65-year-old Hispanic man with a history of disseminated cutaneous coccidioidomycosis who presented to the emergency room for progressively worsening abdominal pain associated with shortness of breath. The patient was found to have pleural effusion and moderate ascites on physical examination. Abdominal ultrasound and computed tomography scan were consistent with moderate ascites and portal hypertension but negative for both liver cirrhosis and for venous or arterial thrombosis. Cytology of ascitic fluid was suggestive of portal hypertension and was negative for infection. Subsequent, thoracentesis was suggestive of exudative effusion and also negative for infection. Liver biopsy confirmed the absence of cirrhosis. Complete blood count indicated pancytopenia, whereas bone marrow biopsy and flow cytometry were suggestive of marginal zone lymphoma (MZL). Clinically, the patient's shortness of breath was resolved by thoracentesis and paracentesis; however, his abdominal pain persisted. A diagnosis of idiopathic noncirrhotic portal hypertension in the setting of splenic MZL was made. The patient was transferred to a higher level of care for splenectomy; however, he missed multiple appointments. Since discharge, the patient has been seen in the outpatient setting and states that he is controlling his disease with diet and exercise; however, he continues to complain of intermittent shortness of breath with exertion.

摘要

我们报告一例65岁的西班牙裔男性病例,该患者有播散性皮肤球孢子菌病病史,因腹痛进行性加重并伴有呼吸急促而就诊于急诊室。体格检查发现患者有胸腔积液和中度腹水。腹部超声和计算机断层扫描显示中度腹水和门静脉高压,但肝硬化、静脉或动脉血栓形成均为阴性。腹水细胞学检查提示门静脉高压,感染阴性。随后,胸腔穿刺术提示渗出性胸腔积液,感染也为阴性。肝活检证实无肝硬化。全血细胞计数显示全血细胞减少,而骨髓活检和流式细胞术提示边缘区淋巴瘤(MZL)。临床上,患者的呼吸急促通过胸腔穿刺术和腹腔穿刺术得到缓解;然而,他的腹痛持续存在。诊断为脾脏MZL背景下的特发性非肝硬化门静脉高压。患者被转至更高水平的医疗机构进行脾切除术;然而,他多次爽约。自出院以来,患者一直在门诊就诊,并表示通过饮食和运动控制病情;然而,他仍继续抱怨运动时间歇性呼吸急促。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/1e73741893ae/10.1177_2324709615609385-fig7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/44453f71e9e2/10.1177_2324709615609385-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/1071e55aa0d8/10.1177_2324709615609385-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/ce4343dfd4d0/10.1177_2324709615609385-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/73d9ee40785b/10.1177_2324709615609385-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/c4fc947c4cc9/10.1177_2324709615609385-fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/a3da55016a7d/10.1177_2324709615609385-fig6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/1e73741893ae/10.1177_2324709615609385-fig7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/44453f71e9e2/10.1177_2324709615609385-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/1071e55aa0d8/10.1177_2324709615609385-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/ce4343dfd4d0/10.1177_2324709615609385-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/73d9ee40785b/10.1177_2324709615609385-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/c4fc947c4cc9/10.1177_2324709615609385-fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/a3da55016a7d/10.1177_2324709615609385-fig6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5ca/4748510/1e73741893ae/10.1177_2324709615609385-fig7.jpg

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本文引用的文献

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Noncirrhotic portal hypertension.非肝硬化性门静脉高压症
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The current clinical aspects of idiopathic portal hypertension.特发性门静脉高压的当前临床情况。
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Management of marginal zone lymphoma.边缘区淋巴瘤的管理
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Non-cirrhotic portal hypertension - diagnosis and management.非肝硬化性门静脉高压症——诊断与管理。
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Splenic marginal zone lymphoma: a population-based study on the 2001-2008 incidence and survival in the United States.脾脏边缘区淋巴瘤:2001-2008 年美国发病率和生存率的基于人群研究。
Leuk Lymphoma. 2013 Jul;54(7):1380-6. doi: 10.3109/10428194.2012.743655. Epub 2012 Nov 26.
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Assessment of splenic perfusion in patients with malignant hematologic diseases and spleen involvement, liver cirrhosis and controls using volume perfusion CT (VPCT): a pilot study.应用容积灌注 CT(VPCT)评估恶性血液病伴脾脏受累、肝硬化及对照组患者的脾脏灌注:一项初步研究。
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Nodular regenerative hyperplasia associated with idiopathic thrombocytopenic purpura in a young girl: a case report and review of the literature.一名年轻女孩中与特发性血小板减少性紫癜相关的结节性再生性增生:病例报告及文献复习
J Pediatr Gastroenterol Nutr. 2005 Aug;41(2):251-5. doi: 10.1097/01.mpg.0000180029.73272.45.
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Patterns of bone marrow involvement in 58 patients presenting primary splenic marginal zone lymphoma with or without circulating villous lymphocytes.58例原发性脾边缘区淋巴瘤伴或不伴循环绒毛淋巴细胞患者的骨髓受累模式
Br J Haematol. 2003 Aug;122(3):404-12. doi: 10.1046/j.1365-2141.2003.04449.x.
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Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients.西方特发性非肝硬化性肝内门静脉高压症:28例患者的重新评估
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