Primary myelofibrosis

A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.

Portal hypertension

Increased pressure in the portal vein.


Total: 11

                      


(per page)
PMID (PMCID)
28011890
MALE Adult
Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal and pulmonary extramedullary haematopoeisis, portal hypertension and tuberculosis: initial presentation and 2 years follow-up.
Panda A, Chandrashekhara SH, Nambirajan A, Mishra P.
BMJ Case Rep. 2016;2016:.
Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal and pulmonary extramedullary haematopoeisis, portal hypertension and tuberculosis: initial presentation and 28197years follow-up.
22722458
MALE
Transjugular intrahepatic porto-systemic stent-shunt for therapy of bleeding esophageal varices due to extramedullary hematopoiesis in primary myelofibrosis: a case report.
Phillip V, Berger H, Straub M, Saugel B, Treiber M, Einwachter H, Schmid RM, Huber W.
Onkologie. 2012;35(6):368-71.
This report illustrates that TIPS placement can also be a promising treatment option in patients with primary myelofibrosis and portal hypertension due to extramedullary hematopoiesis.
20181038
(2830976)
OTHER
Acute variceal bleeding in a patient with idiopathic myelofibrosis successfully treated with endoscopic variceal band ligation and chemotherapy: a case report.
Tamaki K, Otaka M, Sakamoto N, Matsumoto K, Yamashina S, Watanabe S.
J Med Case Rep. 2010;4:25.
Portal hypertension is observed in some patients with idiopathic myelofibrosis.
22958626
(3436631)
OTHER
Pulmonary hypertension diagnosed by echocardiography during idiopathic myelofibrosis. A case report and a brief review of the literature.
Perrone C, Cartolari R, Lupi B, Morelli S.
Multidiscip Respir Med. 2010;5(4):267-70.
Pulmonary hypertension (PH) is only occasionally reported, although recent studies have suggested an association between PH and myeloproliferative disorders.We present a case of PH diagnosis by echocardiography in a patient affected by idiopathic myelofibrosis with portal hypertension.
20886427
FEMALE Middle Aged
[A rare cause of variceal bleeding].
Naderi J, Lohse AW, Galle PR, Schramm C.
Z Gastroenterol. 2010;48(10):1215-8.
Agnogenic myeloid metaplasia is a rare cause of portal hypertension and may present with variceal bleeding.
17301514
MALE Middle Aged
Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension due to idiopathic myelofibrosis.
Doki N, Irisawa H, Takada S, Sakura T, Miyawaki S.
Intern Med. 2007;46(4):187-90.
Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension due to idiopathic myelofibrosis.
16181389
MALE Middle Aged
Portal hypertension secondary to myelofibrosis: a study of three cases.
Alvarez-Larran A, Abraldes JG, Cervantes F, Hernandez-Guerra M, Vizzutti F, Miquel R, Gilabert R, Giusti M, Garcia-Pagan JC, Bosch J.
Am J Gastroenterol. 2005;100(10):2355-8.
In patients with idiopathic myelofibrosis (IM), portal hypertension (PHT) without thrombosis of the hepatic or splenoportal veins is infrequent.
15648462
MALE Middle Aged
Prevention of hepatic infarction as acute-phase complication of TIPS by temporary balloon occlusion in a patient with primary myelofibrosis.
Kobayashi S, Matsui O, Yoshikawa J, Gabata T, Kawamori Y, Sanada J, Terayama N, Kadoya M.
Radiat Med. 2004;22(6):432-6.
Severe acute liver dysfunction occurred following transjugular intrahepatic portosystemic shunt (TIPS) creation in a patient with massive ascites due to portal hypertension associated with primary myelofibrosis.
11293952
MALE Middle Aged
Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS).
Belohlavek J, Schwarz J, Jirasek A, Krajina A, Polak F, Hruby M.
Wien Klin Wochenschr. 2001;113(5-6):208-11.
The authors report a 56-year-old man with idiopathic myelofibrosis and splenomegaly complicated by hepatopathy, severe portal hypertension and recurrent variceal bleeding.
11293952
MALE Middle Aged
Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS).
Belohlavek J, Schwarz J, Jirasek A, Krajina A, Polak F, Hruby M.
Wien Klin Wochenschr. 2001;113(5-6):208-11.
Idiopathic myelofibrosis may be accompanied by portal hypertension.
2973304
MALE Adult
[The chronic form of the Budd-Chiari syndrome].
Kharchenko NM, Al'pidovskii VK, Sultanov IIa.
Arkh Patol. 1988;50(7):79-83.
The diagnosis on discharge was idiopathic myelofibrosis with portal hypertension.