Rare case of portal vein thrombosis tied to PNH successfully treated

No complications reported in woman, 49, after surgical procedure called TIPS

Patricia Inacio PhD avatar

by Patricia Inacio PhD |

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A rare case of portal vein thrombosis (PVT) — where a clot blocks blood flow in the portal vein, a major liver blood vessel — in a 49-year-old woman with paroxysmal nocturnal hemoglobinuria (PNH) was detailed in a case report.

Transjugular intrahepatic portosystemic shunt (TIPS), a surgical procedure that creates a new connection between the portal vein and surrounding vessels, was shown to be effective at improving the woman’s condition, without complications.

“TIPS may become a new treatment option for PNH combined with PVT,” the researchers wrote in the study “Case report: Paroxysmal nocturnal hemoglobinuria presenting with hemorrhagic esophageal varices,” which was published in the journal Frontiers in Medicine.

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Treating portal vein thrombosis in PNH patients known to be complicated

A rare blood disease, PNH is characterized by the destruction of red blood cells, or hemolysis, and thrombophilia, which refers to blood that is prone to form clots. Thrombophilia can lead to thromboembolic events, which occur when a blood clot travels through the bloodstream to a location in the body, where it can block blood flow.

These disease-related events are “the most vicious acquired thrombophilic state known to medicine,” the researchers wrote.

While blood clots can form anywhere, the hepatic veins that carry blood low in oxygen from the liver back to the heart “are the most commonly involved site in PNH,” the researchers wrote.

“PNH with PVT, on the other hand, is very rare and is easy to mistreat due to complex complications,” they added.

The portal vein is a large blood vessel that carries blood from the intestines, stomach, spleen, and pancreas into the liver. If left untreated, portal vein thrombosis can increase blood pressure in the vein and cause a cavernous transformation of the portal vein (CTPV), or the formation of small dilated blood vessels in and around the portal vein.

Researchers at the First Hospital of Hebei Medical University, in China, described the rare case of a woman who developed PVT, CTPV, and hemorrhagic esophageal varices due to PNH, without hepatic vein thrombosis.

Hemorrhagic esophageal varices, which are often caused by blocked blood flow through the portal vein, are enlarged, tortuous, and bleeding veins in the esophagus, the tube that carries food and liquid from the throat to the stomach.

“The isolated involvement of portal veins with visceral veins without the involvement of the hepatic veins is rare,” the researchers wrote.

The woman had a history of melena, a condition marked by the presence of blood in the stools that cause it to appear black, for the past 1.5 years.

She had been living with a PNH diagnosis for nine years, and she was found to have portal vein thrombosis at a routine examination five years before being admitted to the researchers’ hospital.

She showed no PVT-related symptoms, had no history of thrombosis in the family, and had received treatment with the anti-inflammatory prednisolone and the blood thinner warfarin.

A CT scan five months before her hospital admission showed varices in the esophagus and stomach, along with enlargement of the spleen and a wider portal vein diameter. To relieve high portal vein blood pressure and the enlarged spleen, she underwent a selective splenic artery embolization, a surgery to block a spleen artery.

Five months after the surgery, however, the woman developed a more severe form of melena, without any other gastrointestinal symptoms, and came to the researchers’ hospital.

Physical examination showed normal body temperature and respiratory rate. However, her heart rate was high, her blood pressure was low, and she was pale. She also showed signs of an enlarged spleen and tenderness on the left upper side of the abdomen.

Blood work confirmed anemia, as she had markedly low levels of hemoglobin, the protein in red blood cells that carries oxygen. The woman also had high levels of lactate dehydrogenase, a hemolysis marker, low platelet counts, and high levels of two blood clotting markers. A bone marrow test showed increased numbers of blood cell precursors.

A new CT scan confirmed the previously detected PVT and esophageal and stomach varices, but also showed thrombosis in the splenic vein and the superior mesenteric vein, which drains blood from the small intestine, along with CTPV.

“We believe that SSAE [selective splenic artery embolization] not only has no [therapeutic] value but may also have increased PVT,” the researchers wrote.

“The patient was diagnosed with PNH complicated by varices portal vein thrombosis and superior mesenteric vein thrombosis causing esophageal [and] gastric vein bleeding,” the team added.

She underwent TIPS, a surgical procedure that creates a new channel to connect the portal vein and the hepatic vein. A stent, a short wire mesh tube to help keep a blood vessel open, also was placed in the superior mesenteric vein and splenic vein.

She continued treatment with prednisone and received into-the-vein heparin, a molecule that suppresses blood clotting, followed by warfarin.

The varices in the esophagus and stomach disappeared, her hemoglobin levels rose, and blood was no longer found in her stools.

Examinations up to one year after the surgery confirmed smooth blood flow in the targeted blood vessels. No complications were reported.

“In conclusion, portal vein thrombosis in patients with paroxysmal nocturnal hemoglobinuria is rare and [resistant] to treatment,” the researchers wrote. “The long course of the disease facilitates portal cavernous transformation, and it is difficult to achieve a curative effect via simple spleen embolism.”

This case highlights “the improvement of melena and a steady recovery in hemoglobin after … TIPS surgery,” supporting its use in PNH patients with PVT, the scientists concluded.