Hepatic Portal Vein Obstruction ( Ehpvo )

HISTORY OF PRESENT ILLNESS: This 46-year old gentleman with past medical history significant only for degenerative disease of the bilateral hips, secondary to arthritis presents to the emergency room after having had 3 days of abdominal pain. It initially started 3 days ago and was a generalized vague abdominal complaint. Earlier this morning the pain localized and radiated to the right lower quadrant. He had some nausea without emesis. He was able to tolerate p.o earlier around 6am, but he now

Portal hypertension is a condition in which there is a dramatic increase of blood flow thru the hepatic portal vein. The portal vein is responsible for feeding blood to various parts of the gastrointestinal tract along with the spleen and liver. This condition develops due to an increase resistance of blood flow usually within the liver but can be also caused by pre and posthepatic conditions. However, the end result of this condition can lead to the development of variceal bleeding and ascites. Cirrhosis, scarring of the liver tissue, is the most common cause of portal hypertension due to damage liver cells and their inability to function properly, which causes a dramatic slow down of blood flow thru the organ. Unfortunately, portal hypertension

It is important to rule out common causes of liver injury, such as alcohol, drug use, and viral hepatitis as well as other co-existing etiologies for chronic liver disease including alpha-1 antitrypsin deficiency, hemochromatosis, autoimmune liver disease (types 1 and 2), chronic viral hepatitis, and Wilson’s disease

    Factor V Leiden is a hereditary gene mutation that leads to the disorder in the clotting process of blood. It causes the diseases known as Factor V Leiden Thrombophilia or APC resistance. Factor V functions normally in the coagulation pathway of the blood. But a mutation occurs and its cleavage by the body’s natural inhibitor Protein C is slowed. This leads to excessive clot formation in the blood vessels. This mutation is the most common cause for a Deep vein thrombosis(DVT).1 DVT happens most frequently in the legs but can also happen in other parts of the body.3 About 5% of caucasians are heterozygous or homozygous carriers of this factor mutation.2 Heterozygous carriers are associated with a three to sevenfold increase

Disseminated Intervascular Coagulation (DIC) is a clinicopathologic syndrome characterized by widespread intravascular fibrin formation in response to excessive blood protease activity that overcomes the natural anticoagulant mechanisms (Harrison, T. 1974).The mechanism of DIC is a generation of thrombin that is uncontrolled from exposure of blood to levels of tissue factors pathologically. Two factors that add to the deposition of fibrin in small and midsize vessels and also speed up the process are, simultaneous suppression of physiologic anticoagulant mechanisms and abnormal fibrinolysis (Harrison, T. 1974). Fibrin deposition can interfere with the blood supply of many major organs such as the lung, kidney, liver, and brain, all causing

Hemophilia is the name of a group of hereditary blood disorders that doesn’t allow the blood to clot normally (Frey 2009). More specifically hemophilia A, also known as factor VIII, is caused by a missing clotting protein coagulation factor VIII. It is often times referred to as the “Royal Disease” because of how it ran rampant through the royal families of England, Spain , Germany, and other European countries during the 19th and 20th century. Hemophilia A is inherited in X-linked recessive pattern. In males (who have only one X chromosome), one copy of the affected gene is enough to cause the condition. In females (who have two X chromosomes), a mutation on both of the genes would have to be present to cause the disorder. Because it is very unlikely that a female would have both altered copies on both of the genes females generally don’t get hemophilia. Since fathers can only pass on the Y chromosomes to their sons they can’t pass X-linked traits along (Hemophilia 2015). Hemophilia is linked to the mutation of the F8, on the X chromosome, as seen in Figure 1. Mutations in the F8 gene can cause faulty versions of the coagulation protein, therefore this protein cannot participate in the clotting process (Gersten 2014).

The pathological characteristics of cirrhosis is the development of scar tissue that swaps normal parenchyma. This scar tissue barricades the blood flow through the organ thus disturbing normal function. Fibrosis changes the structure and vasculature, worsening blood and lymph flow and leading to hepatic insufficiency and hypertension in the portal vein. Cirrhosis is often caused by hepatitis and fatty liver, if the cause is removed at this stage then the changes can by reversible. In order for them to be irreversible, the patient must alter their modifiable risk

This type differs from haemophilia A and B as there is no bleeding into joints and muscles. Another difference is that unlike factor VIII and IX deficiencies in the other Hemophilia types, factor XI Ievels n the blood don’t predominately correlate with its bleeding severity in the patient. “What is characteristic of factor XI deficiency is that you can have a severe factor XI deficiency with a level of less than 1% and be asymptomatic,” says Ann Hurlet-Jensen, MD, associate professor, pediatric hematology-oncology, Mount Sinai School of Medicine in New York. “On the other hand, you can have a level in the 30% to 40% range and have bleeding.” Although it is most often that those with the Haemophilia disorder display little and varied visible symptoms making early diagnosis and treatment difficult. It is also of autosomal recessive inheritance in which two recessive afflicted alleles are needed to produce afflicted offspring, thus affecting males and females with equal

Portal hypertension is an increase in blood pressure in a system of veins called the portal venous system or an increase in hepatic venous gradient. An increase in portal venous pressure above 10 mmHg or hepatic venous gradient more than 5 mmHg is diagnosed as portal hypertension (Hagen, 239). Hepatocellular disease is an example that causes portal hypertension. The disease causes a blockage of blood flow throughout the liver, which causes the blood to back up into the hepatic portal circulation. The backing up of blood causes the blood pressure in the hepatic circulation to increase, therefor it results in portal hypertension.

PVT is a partial or complete thrombosis within the portal vein or its immediate downstream tributaries. Light symptoms such as fatigue, mild swelling, and abdominal discomfort are the first signs of PVT and are easily overlooked, or attributed to symptoms of cancer or cancer treatment. It can also be nearly asymptomatic until the disease has progressed for a long time. Eventually, symptoms such as ascites, hepatomegaly, and varices present. Varices can rupture and bleed, resulting in potentially life-threatening complications. Additionally, due to a lack of nutrients being delivered to the liver, PVT can cause liver failure. If the disease progresses further, death can result7.

Multiple paraneoplastic syndromes have been introduced for HCC. Thrombocytosis is one of these rare paraneoplastic findings in cases of liver cirrhosis with HCC. Degree of thrombocytosis correlates with the size of the HCC and the level of Alpfa FetoProtein (AFP).

Factor V is a protein made in your liver that helps convert prothrombin into thrombin. If your body doesn’t produce enough

Cancer patients with Factor V Leiden (rs6025), Factor VHR2 haplotype (rs1800595) prothrombin G20210A (rs1799963), PAI-1 4G/5G (rs1799889), MTHFR 677C>T (rs1801133), and factor XIII Val34Leu (rs5985) have a many fold increased risk for developing VTE compared to cancer patients without these hereditary risk factors [15,16]. A potential effect of polymorphisms in clotting factors on cancer risk would be relevant scientifically and clinically and have determining role in management and treatment of such patients. Studies have been undertaken in a number of populations for such risk assessments but no such study has been undertaken in Saudi population till date. With increased burden of Cancer in Saudi Arabia study of risk factors is highly warranted to decrease cancer associated mortality and morbidity and improve the quality of life of such patients. We shall genotype factor V Leiden (FVL), Factor VHR2 haplotype (FVHR2), prothrombin G20210A (PT G20210A), PAI-1 4G/5G, MTHFR 677C>T, and factor XIII Val34Leu (FXIII Val34Leu) in cancer patients and controls to identify these as risk

Manuscript History: Received: 14 October 2015 Final Accepted: 22 November 2015 Published Online: December 2015 Key words: Portal vein, Thrombosis, risk factors, cirrhosis. *Corresponding Author

Coagulation tests measure functions that are related with clotting, coagulation consists of drawing blood and observing it clot. Chelating out the calcium and beginning the clot in a controlled environment. Coagulation tests used to find out if an individual has a clotting disorders and to measure the various proteins and how they function. There are many conditions that can causes problems with coagulation for example thrombophilia and haemophilia. (TRANSFUSION MEDICINE AND HEMOSTASIS BOOK)