Nodular Regenerative Hyperplasia, Blood Disorders and Other Uncommon Diseases Associated with Intrahepatic Portal Hypertension

BACKGROUND/AIMSNodular regenerative hyperplasia of the liver is a histological lesion usually associated with systemic diseases, haematological malignancies, or drugs. Its prognosis depends on portal hypertension, which usually is well tolerated and requires medical management only.PATIENTSThree unrelated families, in which two sibling adult male patients presented with nodular regenerative hyperplasia of the liver, were studied.METHODSComplete clinical charts and liver biopsy specimens were available for all patients. In addition, explanted livers were available for examination for the two transplanted patients.RESULTSThere was no evidence of any of the various clinical situations known to be associated with nodular regenerative hyperplasia of the liver. Portal hypertension was severe, requiring surgical treatment in two cases. Renal lesions were present in three patients. In two patients, progressive evolution to liver atrophy and hepatic failure, associated with renal failure, led to combined liver and renal transplantation.CONCLUSIONSThis report describes the existence of familial cases of nodular regenerative hyperplasia of the liver, occurring without underlying or associated systemic disease, characterised by a poor clinical course and often associated with progressive renal failure.

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Portal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer

Case Reports in Pathology ◽

10.1155/2013/826284 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Andrew T. Turk ◽

Matthias J. Szabolcs ◽

Jay H. Lefkowitch

Keyword(s):

Breast Cancer ◽

Portal Hypertension ◽

Breast Carcinoma ◽

Portal Vein ◽

Metastatic Breast ◽

Nodular Regenerative Hyperplasia ◽

Metastatic Breast Carcinoma ◽

Noncirrhotic Portal Hypertension ◽

Diagnostic Difficulties ◽

Recurrent Breast

Nodular regenerative hyperplasia (NRH) of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. This report describes a 64-year-old man with clinically presumed cirrhosis who presented to our institution with coffee-ground emesis, esophageal varices, ascites, and encephalopathy. Eleven years earlier he had been treated for breast cancer with mastectomy and chemo-radiotherapy. He died suddenly, and the autopsy showed no evidence of cirrhosis but instead demonstrated NRH with extensive emboli of recurrent breast carcinoma within the portal vein and its intrahepatic branches. Neoplastic occlusion of the portal vein as a cause of presinusoidal noncirrhotic portal hypertension has not previously been reported for metastatic breast carcinoma. This case highlights the importance of obstructive portal venopathy in the pathogenesis of NRH as well as the diagnostic difficulties that may be encountered in determining the cause of portal hypertension.

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6-Thioguanine Associated Nodular Regenerative Hyperplasia in Patients With Inflammatory Bowel Disease May Induce Portal Hypertension

The American Journal of Gastroenterology ◽

10.1111/j.1572-0241.2007.01530.x ◽

2007 ◽

Vol 102 (11) ◽

pp. 2495-2503 ◽

Cited By ~ 60

Author(s):

Arnulf Ferlitsch ◽

Alexander Teml ◽

Walter Reinisch ◽

Gregor Ulbrich ◽

Fritz Wrba ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Portal Hypertension ◽

Bowel Disease ◽

Nodular Regenerative Hyperplasia ◽

Inflammatory Bowel

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Nodular Regenerative Hyperplasia of the Liver: An Unusual Cause of Portal Hypertension in Childhood

Archives of Pediatrics and Adolescent Medicine ◽

10.1001/archpedi.1981.02130300070026 ◽

1981 ◽

Vol 135 (6) ◽

pp. 572 ◽

Cited By ~ 1

Author(s):

GARTH ALPERSTEIN

Keyword(s):

Portal Hypertension ◽

Nodular Regenerative Hyperplasia

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Portal hypertension after prolonged use of trastuzumab emtansine: nodular regenerative hyperplasia with autoimmune features

Research Society and Development ◽

10.33448/rsd-v10i8.17005 ◽

2021 ◽

Vol 10 (8) ◽

pp. e10810817005

Author(s):

Gustavo Vargas Borgongino Monteiro ◽

Marcela Chagas Lima Mussi ◽

Mateus Jorge Nardelli ◽

Bruno Campos Santos ◽

Cláudia Alves Couto

Keyword(s):

Portal Hypertension ◽

Esophageal Varices ◽

Ductal Carcinoma ◽

Rare Complication ◽

Nodular Regenerative Hyperplasia ◽

Trastuzumab Emtansine ◽

Her2 Breast Cancer ◽

History Of ◽

The Right

We report a rare complication associated with prolonged use of trastuzumab emtansine (T-DM1) – a composed therapy for HER2+ breast cancer – presenting with laboratorial autoimmune features that could have delayed the diagnosis or led to misdiagnosis. A 55-year-old female was referred to the hepatologist with a computed tomography suggestive of portal hypertension for etiological investigation. History of invasive ductal carcinoma in the right breast undergoing treatment for 5 years. She had already undergone neoadjuvant chemotherapy, mastectomy, radiotherapy and adjuvant chemotherapy. By the time of metastatic diagnosis, she was in monotherapy with T-DM1 for 2.5 years. Upper endoscopy showed esophageal varices and portal hypertension gastropathy. Laboratorial tests revealed increased transaminases, hypergammaglobulinemia and positive antinuclear antibody. Liver biopsy was performed for autoimmune hepatitis differential diagnosis but revealed nodular regenerative hyperplasia. T-DM1 was discontinued. After a 2-year follow-up, the patient did not present any complications of portal hypertension, although persisted with esophageal varices.

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