Splenic–Portal– Mesenteric Thrombosis With Splenic Infarction as First Presentation of Essential Thrombocytosis

Jm, Chebli; Pd, Gaburri; Af, Meirelles de Souza; Ge, Gonçalves Felga; Cg, Forn; Pinto, FJ

doi:10.1097/01.mcg.0000123226.15379.38

Cited by 4 publications

(5 citation statements)

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“…Another retrospective study involving 84 patients demonstrated that MPN-associated SVT involved the portal vein in 69% of the cases, and that the thrombus extended to the mesenteric and/or splenic vein in < 20% of these patients [5]. Although the thrombus extension is usually limited to one of these venous systems, SVT involving more than one of the main splanchnic veins has rarely been described as the onset manifestation of MPN, including ET [6][7][8][9][10], PV [10,11], and PMF [12] cases (summarized in Table 1). In some of these patients, the thrombus extended throughout the portal, splenic, and superior mesenteric venous systems [6][7][8]10], similar to in our patient.…”

Section: Discussionmentioning

confidence: 99%

“…Although the thrombus extension is usually limited to one of these venous systems, SVT involving more than one of the main splanchnic veins has rarely been described as the onset manifestation of MPN, including ET [6][7][8][9][10], PV [10,11], and PMF [12] cases (summarized in Table 1). In some of these patients, the thrombus extended throughout the portal, splenic, and superior mesenteric venous systems [6][7][8]10], similar to in our patient. This is a critical clinical situation that may result in severe complications.…”

Section: Discussionmentioning

confidence: 99%

“…Abdominal pain resulting from increased venous pressure is almost universal and can be incapacitating. Other serious potential complications include variceal bleeding, ascites or hepatic encephalopathy resulting from portal hypertension [9,13], splenic infarction secondary to splenic vein obstruction [6], and intestinal ischemia secondary to mesenteric vein obstruction [14].…”

Section: Discussionmentioning

confidence: 99%

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Primary Myelofibrosis in the Prefibrotic Stage Presenting as Portal, Splenic, and Superior Mesenteric Vein Thrombosis: A Case Report and Review of the Literature

Dias

Liberal

Costa-Moreira

et al. 2021

GE Port J Gastroenterol

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Introduction: Myeloproliferative neoplasms are the most common cause of splanchnic vein thrombosis in the absence of cirrhosis or nearby malignancy. Case Presentation: A 31-year-old male presented to the emergency department with epigastric pain associated with mild thrombocytosis and elevated levels of aminotransferases, lactate dehydrogenase, and C-reactive protein. Contrast-enhanced abdominal computed tomography revealed splanchnic venous thrombosis that involved the portal, splenic, and superior mesenteric veins, without signs of chronic liver disease. Anticoagulation with warfarin was immediately started. Diagnostic work-up was remarkable for the presence of the JAK2 V617T mutation and hypercellular bone marrow, with increased myeloid cells and atypical megakaryocytes, consistent with primary myelofibrosis in a prefibrotic stage. No other hypercoagulable conditions were identified. Discussion: We present a rare case of primary myelofibrosis in the prefibrotic stage presenting as portal-splenic-superior mesenteric vein thrombosis. This demonstrates that extensive splanchnic vein thrombosis may be the onset manifestation of myeloproliferative neoplasms, even in early stages and in the absence of concomitant hypercoagulable conditions. The presence of the JAK2 mutation is an important prothrombotic risk factor that can, per se, contribute to large venous thrombosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Primary Myelofibrosis in the Prefibrotic Stage Presenting as Portal, Splenic, and Superior Mesenteric Vein Thrombosis: A Case Report and Review of the Literature

Dias

Liberal

Costa-Moreira

et al. 2021

GE Port J Gastroenterol

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“…Many patients with ET are asymptomatic, but many others have manifest symptoms or signs of bleeding or microvascular thrombosis [2][3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

Acute lower limb ischemia in a patient with aortic thrombus and essential thrombocytosis

et al. 2009

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Aortic thrombus is rare in patients with essential thrombocytosis (ET), so the optimal treatment remains undefined. A 45-year-old man with history of ET, under chronic treatment with aspirin, presented to the emergency department complaining of acute onset in both the legs and abdominal pain. Physical examination revealed that both dorsalis pedis pulses were not palpable with cold and pale feet. His abdomen was soft and nondistended. The platelet count was 436 x 10(9)/L. The thoraco-abdominal computerized tomographic scanning revealed normal aortic diameter with supraceliac and infrarenal nonoccluding thrombus and infarction areas in spleen and left kidney. At the emergency department he presented with recurrent symptoms, losing bilateral posterior tibial pulses. A decision was made to perform a thoracoretroperitoneal incision. A longitudinal sequential aortotomy was performed in the distal thoracic and infrarenal aorta, and the thrombus was easily removed. Following this, he underwent bilateral crural thrombectomy and local intra-arterial thrombolytic therapy. The postoperative course was uneventful. The left toes were amputated because of necrosis. He was discharged and put on antiaggregants, anticoagulants and hydroxyurea. Aortic thrombus in patients with ET is unusual, but potentially lethal. There is complete relief from symptoms in recurrent cases following surgery. An appropriate medical treatment after intervention must be supported.

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“…We present a case of a patient with essential thrombocytosis and obscure-overt GI bleeding, who was diagnosed with gastric varices only after multiple presentations. Essential thrombocytosis is associated with gastro-intestinal vascular thrombosis [4]. Multiple Doppler studies of the splenic vein also failed to reveal any splenic vein thrombosis to explain the etiology of the isolated gastric varices.…”

Section: Introductionmentioning

confidence: 99%

Isolated Gastric Varices due to Essential Thrombocytosis Related to Splenic Vein Thrombosis: A Challenge to Uncover the Concealed Diagnosis

Patel

Bhandari

Kumar³

et al. 2019

Cureus

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Essential thrombocytosis is associated with gastrointestinal (GI) vascular thrombosis. Sinistral portal hypertension (left-sided portal hypertension) due to splenic vein thrombosis can lead to isolated gastric varices and should be suspected in a patient with obscure GI bleeding with normal liver function. This case reviews the challenges presented in diagnosing isolated gastric varices due to splenic vein thrombosis through radiologic or endoscopic studies. Unrevealing radiologic or endoscopic studies does not rule out splenic vein thrombosis, which should be suspected in a patient with essential thrombocytosis and obscure GI bleeding.

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Splenic–Portal– Mesenteric Thrombosis With Splenic Infarction as First Presentation of Essential Thrombocytosis

Cited by 4 publications

References 1 publication

Primary Myelofibrosis in the Prefibrotic Stage Presenting as Portal, Splenic, and Superior Mesenteric Vein Thrombosis: A Case Report and Review of the Literature

Primary Myelofibrosis in the Prefibrotic Stage Presenting as Portal, Splenic, and Superior Mesenteric Vein Thrombosis: A Case Report and Review of the Literature

Acute lower limb ischemia in a patient with aortic thrombus and essential thrombocytosis

Isolated Gastric Varices due to Essential Thrombocytosis Related to Splenic Vein Thrombosis: A Challenge to Uncover the Concealed Diagnosis

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