Dr. Jia Zhong, Hangzhou Huansha Road 261,310006,Department of Hepatobiliary Surgery,10th Floor of No.1 building of Hangzhou First People’s Hospital, Zhejiang Province, China. Fax:+86-0571-87914773;Mobile Phone:+86-13958114181; E-mail:
Meanwhile, we don’t find any sign of swollen skin and other complications and she denied the feeling of itch. She repeatedly complained that her health went worse gradually since she began to work on spray paint, when compared to her previous medical health record. Hence, she highly suspected her symptoms might be associated with her work environment or some paint solvents. She and her family hoped clinicians could confirm or exclude her suspicion during therapeutic course in this admission. Her words arouse our great interest, and we decided to decode the conundrum. Amazingly, no specific clues were obtained from her family history, appetites, general laboratory tests. Further enhanced contrast computed tomography of abdomen showed special findings ,including isolated gastric varices, major portal vein thrombosis(red arrowhead), focal splenic vein stenosis (blue arrowhead), and splenomegalia complied with focal splenic, but myelofibrosis was confirmed by bone marrow biopsy, and the expression of JAK2-V617F was detected in her peripheral blood sample (Figure A).
Patients with positive JAk2-V617F can produce some cytokines, which may lead to vessel and epithelia endothelial cells proliferation and its fibrosis formation. This may be a definitive reason why splenic vein stenosis disease in this case was ultimately formed. And then, both thrombocytosis and splenic vein stenosis resulted in portal vein thrombosis, while these changes may account for congestive splenomegaly and gastric varices. The differential diagnosis includes blood diseases, postcirrhotic portal hypertension and Budd-Chiari syndrome, etc.
Most of symptomatic patients with left-sided portal hypertension can be treated with splenectomy alone or plus devascularization operation, the minority of patients may choose conservative therapy. In this case, we have to face another layer of complex with two different risks. At the first layer, conservative therapy may be the priority of choice, and endoscopic technique can be applied to stop bleeding due to occasional gastric varices rupture. But on second look, how to resolve early satiety and abdominal distention triggered by gradually enlarged spleen?
As a matter of fact, sometimes it’s necessary to take adventure if benefits from splenectomy beyond the potential risks of myelofibrosis. For example, we have ever encountered a gastric body cancer patient with myelofibrosis, who must receive total gastroectomy plus splenectomy. Based on our previous similar experience, the crucial key to success is better administration of myelofibrosis after surgery than fear.
Up to date, the overwhelming majority of the patients with positive. The incidence of secondary hematologic and non hematologic malignancies was increased in patients with chronic myeloid proliferative tumors. Compared with the incidence rate of the general population, the risk of having two tumors in patients with primary thrombocytosis is 1.2 times; the patients with polycythemia vera are 1.6 times; the patients with chronic myelogenous leukemia are 1.6 times. terestingly, hypersplenism counteracted effects of thrombosis before surgery, as a result, the platelet count seemed to be within normal range before surgery. But elevation of platelet may be temporarily a natural response to splenectomy. On the other hand, the elevation of platelet after splenectomy also may be another positive echo to curb the progress of myelofibrosis, but interconnected ties between them still remains unclear and waits for further study.
Although antiplatelet therapy (aspirin intake add venous heparin) was conducted to treat portal vein thrombosis, the platelet count seemed to be out of control, with sharply rising and up to its peak [1,620,000/uL (125000/ul-350000/ul)] at postoperative day 21.In fact, the backlash of platelet number was just a normal response to splenectomy, while impact of myelofibrosis was minor. Whatever it moved, enhancing antiplatelet therapy /hydroxyurea intake adding interferon) was administrated. As usual, heparin and war far in underwent overlapping use for 5 days before discharge. Fortunately, thanks to positive response to therapies, the platelet count began to decline at postoperative day 22 without any signs of rebound to rising. After discharge, the patient was administrated by oral war far in intake alone. In review, 3 months after surgery, she recovered with normal platelet count and without evidence of rebleeding. But a few months later, we received the call that when the patients stop taking antiplatelet medicine the platelet count will fluctuate may be a result of the splenectomy or the withdrawal symptoms.
Note: The case report has obtained informed consent from the patient and her family. Also, we has got the approval of ethic committee in our institution.
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