Portal hypertension in systemic mastocytosis

Capron, Jean‐Pierre; Lebrec, Didier; Degott, Claude; Chivrac, D; Coevoet, B; Delobel, J.

doi:10.1016/0016-5085(78)90303-7

Cited by 38 publications

(11 citation statements)

References 15 publications

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“…Bleeding from the gastrointestinal tract is typically due to peptic ulcer disease in approximately 11% of patients with systemic mastocytosis, while liver infiltration with portal hypertension is presumed to be rare. First described by Capron et al in 1978, non-cirrhotic portal hypertension as a result of systemic mastocytosis is thought to be either pre-sinusoidal or sinusoidal 4. While the exact mechanism is unknown, it is postulated that non-cirrhotic portal hypertension may develop as a result of infiltration of inflammatory mast cells within the portal vein and obstruction of the sinusoids 4.…”

Section: Discussionmentioning

confidence: 99%

“…First described by Capron et al in 1978, non-cirrhotic portal hypertension as a result of systemic mastocytosis is thought to be either pre-sinusoidal or sinusoidal 4. While the exact mechanism is unknown, it is postulated that non-cirrhotic portal hypertension may develop as a result of infiltration of inflammatory mast cells within the portal vein and obstruction of the sinusoids 4. This infiltration is thought to result in increased portal pressures and sinusoidal obstruction.…”

Section: Discussionmentioning

confidence: 99%

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Systemic Mastocytosis Complicated by Non-Cirrhotic Portal Hypertension and Variceal Bleeding

McCarty

Hung

Mohanty

et al. 2017

ACG Case Reports Journal

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Systemic mastocytosis is a myeloproliferative disorder characterized by extracutaneous involvement of at least one organ. Although rare, infiltration of inflammatory mast cells within the portal vein may lead to obstruction of the sinusoids resulting in non-cirrhotic portal hypertension. We present a patient with known history of systemic mastocytosis with bone marrow involvement presenting with new-onset esophageal variceal bleeding. Although systemic mastocytosis is uncommon, the subsequent development of hepatic involvement and non-cirrhotic portal hypertension are discussed. Further highlighted is a lack of organization guidelines and the potential for gastrointestinal and hepatic screening of mastocytosis patients with known extracutaneous involvement.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Systemic Mastocytosis Complicated by Non-Cirrhotic Portal Hypertension and Variceal Bleeding

McCarty

Hung

Mohanty

et al. 2017

ACG Case Reports Journal

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“…Systemic mastocytosis has been reported to be complicated with portal hypertension 9,10 . Several mechanisms have been proposed for mastocytosis‐associated portal hypertension, such as portal fibrosis, 9,17–19 subendothelial collagen deposition, 18 increased splenic blood flow, 20 hepatic venous flow block, 9,19 and mast cell infiltration 9,17–22 . Portal fibrosis and subendothelial collagen deposition might be partly explained by the effect of mast cell‐derived factors (e.g.…”

Section: Discussionmentioning

confidence: 99%

“…9,10 Several mechanisms have been proposed for mastocytosis-associated portal hypertension, such as portal fibrosis, 9,[17][18][19] subendothelial collagen deposition, 18 increased splenic blood flow, 20 hepatic venous flow block, 9,19 and mast cell infiltration. 9,[17][18][19][20][21][22] Portal fibrosis and subendothelial collagen deposition might be partly explained by the effect of mast cell-derived factors (e.g. chymase, tryptase, tumor necrosis factor-a, fibroblast growth factor, platelet-derived growth factor, and transforming growth factor-b) on fibroblast proliferation and increased collagen synthesis.…”

Section: Discussionmentioning

confidence: 99%

Mast cell leukemia with rapidly progressing portal hypertension

Yoshida

Nishikawa²,

Yamamoto³

et al. 2009

Pathology International

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Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. A 52-year-old woman presented with urticaria-like skin symptoms, anemia, and thrombocytopenia. Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Chemotherapy with cytosine arabinoside and idarubicin was ineffective and the patient died of multi-organ failure with rapidly progressing hepatosplenomegaly and large-volume ascites 3 months after admission. At autopsy the bone marrow, spleen, liver, and lymph nodes were extensively infiltrated by atypical tumor cells with occasional bi- or multi-lobated nuclei. They were positive for mast cell tryptase and possessed an activating mutation of the c-kitgene (D816V). Ascites (2200 mL) and non-ruptured esophageal varices with submucosal hemorrhage indicated the presence of severe portal hypertension. Although there was no evidence of liver cirrhosis, the hepatic sinusoids were clogged with tumor cells, with a tendency to be more severe in the perivenular areas, and the lumens of central veins were obliterated by tumor cell infiltration. The present case demonstrates that non-cirrhotic portal hypertension due to blocking of sinusoidal and venous flow could be a serious complication in mast cell leukemia.

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“…Physical examination may show splenomegaly, lymphadenopathy, and hepatomegaly. Infiltration of the liver with mast cells results in fibrosis for 20% of patients and causes portal hypertension, noncirrhotic liver disease, and refractory ascites 2–4. SM symptoms reflect mast cell degranulation and include fever, weight loss, fatigue, sweats, pruritus, urticaria, dermatographism, abdominal pain, flushing, headache, tachycardia, hypotension, hypertension, syncope, arthralgia, fractures, and other symptoms 5…”

Section: Discussionmentioning

confidence: 99%

Unexplained and prolonged perioperative hypotension after orthotopic liver transplantation: Undiagnosed systemic mastocytosis

et al. 2009

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Arterial vasodilation is common in end-stage liver disease, and systemic hypotension often may develop, despite an increase in cardiac output. During the preparation for and the performance of orthotopic liver transplantation, expected and transient hypotension may be caused by induction agents, anesthetic agents, liver mobilization, or venous clamping. A mild decrease of the already low systemic vascular resistance is often observed, and intermittent use of short-acting agents for vasopressor support is not uncommon. In this report, we describe a patient with unexpected and prolonged hypotension due to vasodilation during and after orthotopic liver transplantation. The preoperative end-stage liver disease evaluation, intraoperative events, and intensive care unit course were reviewed, and no cause for the vasodilation and prolonged hypotension was evident. The explant pathology report was later available and showed systemic mastocytosis. We hypothesize that the unexpected hypotension and vasodilation were caused by mast cell degranulation and its systemic effects on arterial tone. Liver Transpl 15: [701][702][703][704][705][706][707][708] 2009 A 74-year-old white man presented for evaluation and consideration for orthotopic liver transplantation after receiving the diagnosis of cryptogenic cirrhosis and a referral from another transplant institution. His medical history was remarkable for obesity (body mass index, 36 kg/m 2 ), weight loss of approximately 100 pounds during the past 3 years (attributed to exercise, diet, malaise, fatigue, ascites burden, anorexia, and other reasons), intermittent diarrhea-constipation cycles, and a 14-year history of partial complex seizures that were controlled with medication. Thrombocytopenia was identified during routine laboratory evaluation of an exercise-related injury. Further evaluation included a liver biopsy, which showed fibrosis (stages 3 and 4) and chronic biliary injury. Gastric and colon polyp biopsies performed before liver transplantation did not show any mast cell infiltration. A transjugular intrahepatic portosystemic shunt was placed to manage refractory ascites.Physical examination showed a tall, severely musclewasted man with moderate ascites and palpable splenomegaly. The patient was not febrile, flushed, or diaphoretic. Blood pressure variation was not extreme. He had no skin lesions, dermatographia, or lymphadenopathy. Results of preoperative laboratory tests at our institution are shown in Table 1. The patient had leukopenia, thrombocytopenia, and microcytic, normochromic anemia. A computed tomography scan of the abdomen showed splenomegaly, a large amount of ascites, gallbladder and bowel wall thickening, a liver with

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Portal hypertension in systemic mastocytosis

Cited by 38 publications

References 15 publications

Systemic Mastocytosis Complicated by Non-Cirrhotic Portal Hypertension and Variceal Bleeding

Systemic Mastocytosis Complicated by Non-Cirrhotic Portal Hypertension and Variceal Bleeding

Mast cell leukemia with rapidly progressing portal hypertension

Unexplained and prolonged perioperative hypotension after orthotopic liver transplantation: Undiagnosed systemic mastocytosis

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