Acute upper gastrointestinal bleeding due to portal hypertension in a patient with primary myelofibrosis: A case report

被引:1
作者
Chen, Yu [1 ]
Kong, Bing-Bing [1 ]
Yin, He [1 ]
Liu, Hao [2 ]
Wu, Sheng [1 ]
Xu, Ting [1 ]
机构
[1] Tsinghua Univ, Beijing Tsinghua Changgung Hosp, Sch Clin Med, Dept Emergency, 168 Litang Rd, Beijing 102218, Peoples R China
[2] Tsinghua Univ, Beijing Tsinghua Changgung Hosp, Sch Clin Med, Dept Pathol, Beijing 102218, Peoples R China
关键词
Acute esophageal variceal bleeding; Portal hypertension; Myelofibrosis; JAK2 V617F mutation; Case report; VEIN THROMBOSIS; RUXOLITINIB; DISORDERS;
D O I
10.12998/wjcc.v12.i15.2621
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BACKGROUND Acute upper gastrointestinal bleeding is a common medical emergency that has a 10% hospital mortality rate. According to the etiology, this disease can be divided into acute varicose veins and nonvaricose veins. Bleeding from esophageal varices is a life-threatening complication of portal hypertension. Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10 mmHg. Cirrhosis is the most common cause of portal hypertension, and thrombosis of the portal system not associated with liver cirrhosis is the second most common cause of portal hypertension in the Western world. Primary myeloproliferative disorders are the main cause of portal venous thrombosis, and somatic mutations in the Janus kinase 2 gene (JAK2 V617F) can be found in approximately 90% of polycythemia vera, 50% of essential thrombocyrosis and 50% of primary myelofibrosis. CASE SUMMARY We present a rare case of primary myelofibrosis with gastrointestinal bleeding as the primary manifestation that presented as portal-superior-splenic mesenteric vein thrombosis. Peripheral blood tests revealed the presence of the JAK2 V617F mutation. Bone marrow biopsy ultimately confirmed the diagnosis of myelofibrosis (MF-2 grade). CONCLUSION In patients with acute esophageal variceal bleeding due to portal hypertension and vein thrombosis without cirrhosis, the possibility of myeloproliferative neoplasms should be considered, and the JAK2 mutation test should be performed.
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相关论文
共 20 条
[1]  
[Anonymous], 2016, Acute Upper Gastrointestinal Bleeding in Over 16 s: Management
[2]   Increased risk of thrombosis in JAK2 V617F-positive patients with primary myelofibrosis and interaction of the mutation with the IPSS score [J].
Barbui, Tiziano ;
Ghirardi, Arianna ;
Carobbio, Alessandra ;
Masciulli, Arianna ;
Carioli, Greta ;
Rambaldi, Alessandro ;
Finazzi, Maria Chiara ;
Bellini, Marta ;
Rumi, Elisa ;
Vanni, Daniele ;
Borsani, Oscar ;
Passamonti, Francesco ;
Mora, Barbara ;
Brociner, Marco ;
Guglielmelli, Paola ;
Paoli, Chiara ;
Alvarez-Larran, Alberto ;
Triguero, Ana ;
Garrote, Marta ;
Pettersson, Helna ;
Andreasson, Bjorn ;
Barosi, Giovanni ;
Vannucchi, Alessandro Maria .
BLOOD CANCER JOURNAL, 2022, 12 (11)
[3]   Portal Vein Thrombosis [J].
Basit, Syed Abdul ;
Stone, Christian D. ;
Gish, Robert .
CLINICS IN LIVER DISEASE, 2015, 19 (01) :199-221
[4]   Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients [J].
De Stefano, V. ;
Vannucchi, A. M. ;
Ruggeri, M. ;
Cervantes, F. ;
Alvarez-Larran, A. ;
Iurlo, A. ;
Randi, M. L. ;
Pieri, L. ;
Rossi, E. ;
Guglielmelli, P. ;
Betti, S. ;
Elli, E. ;
Finazzi, M. C. ;
Finazzi, G. ;
Zetterberg, E. ;
Vianelli, N. ;
Gaidano, G. ;
Nichele, I. ;
Cattaneo, D. ;
Palova, M. ;
Ellis, M. H. ;
Cacciola, E. ;
Tieghi, A. ;
Hernandez-Boluda, J. C. ;
Pungolino, E. ;
Specchia, G. ;
Rapezzi, D. ;
Forcina, A. ;
Musolino, C. ;
Carobbio, A. ;
Griesshammer, M. ;
Barbui, T. .
BLOOD CANCER JOURNAL, 2016, 6 :e493-e493
[5]   Vascular Disorders of the Liver [J].
DeLeve, Laurie D. ;
Valla, Dominique-Charles ;
Garcia-Tsao, Guadalupe .
HEPATOLOGY, 2009, 49 (05) :1729-1764
[6]   Causal role for JAK2 V617F in thrombosis [J].
Fleischman, Angela G. ;
Tyner, Jeffrey W. .
BLOOD, 2013, 122 (23) :3705-3706
[7]   JAK Inhibition with Ruxolitinib versus Best Available Therapy for Myelofibrosis [J].
Harrison, Claire ;
Kiladjian, Jean-Jacques ;
Al-Ali, Haifa Kathrin ;
Gisslinger, Heinz ;
Waltzman, Roger ;
Stalbovskaya, Viktoriya ;
McQuitty, Mari ;
Hunter, Deborah S. ;
Levy, Richard ;
Knoops, Laurent ;
Cervantes, Francisco ;
Vannucchi, Alessandro M. ;
Barbui, Tiziano ;
Barosi, Giovanni .
NEW ENGLAND JOURNAL OF MEDICINE, 2012, 366 (09) :787-798
[8]   How I Manage Thrombotic/Thromboembolic Complications in Myeloproliferative Neoplasms [J].
Koschmieder, Steffen .
HAMOSTASEOLOGIE, 2020, 40 (01) :47-53
[9]  
Leebeek FWG, 2012, NETH J MED, V70, P400
[10]   Portal vein thrombosis: The role of imaging in the clinical setting [J].
Margini, Cristina ;
Berzigotti, Annalisa .
DIGESTIVE AND LIVER DISEASE, 2017, 49 (02) :113-120