Retrospective Analysis Of Primary Myelofibrosis In Patients Presenting With Portal Hypertension

BackgroundPrimary myelofibrosis (PMF) is a kind of hematopoietic system disease charactered by hyperplasia of fibrous tissue in bone marrow. As most of these cases have an insidious onset, and the clinical symptoms are often atypical, the diagnosis is difficult. Because some PMF patients have the symptoms of portal hypertension, such as hepatomegaly, splenomegaly and esophageal and gastric varices, they may bemisdiagnosed as other digestive system diseases, delaying the timely diagnosis and treatment. Besides, there are few reports of primary myelofibrosis in patients presenting with portal hypertension in China, and the knowledge about it is limited.ObjectiveTo discuss the disease characteristics and clinical features of primary myelofibrosis in patients presenting with portal hypertension.Methods17cases were identified from the patients of Qilu hospital of Shandong university from January2003to November2012. Clinical data was collected, including:general information (gender, age, hospitalization days, etc.), medical history (history of present illness, past medical history, personal history), physical examination results (jaundice, Spider-burst, hepatomegaly, splenomegaly, ascites, varicose veins, edema, etc.), laboratory test results (blood routine, hepatic function, blood coagulation series, ascite routine, blood biochemical examination, etc.), imaging examination results (B ultrasound, CT, etc.), bone marrow cytological test, bone marrow biopsy, and genetic test results, as well as the main treatment therapies and medicines. Then we extract the relevant data and the statistical analysis was through SPSS.Results14male and3female patients were identified. Their ages ranged from46 to78years (median58±8years). The common symptoms were abdominal distension, abdominal mass and fatigue. Some patients had fever, dizziness, weight loss and coughing. Mo derate-severe splenomegaly was the most common positive sign, and mild-moderate hepatomegaly and shifting dullness could also be found in some cases. In blood routine examination, increased WBC and decreased RBC&hemoglobin are common, while the raised or reduced blood platelet could both be found. As to hepatic function test, all the patients had normal glutamic-pyruvic transaminase(ALT) and glutamic-oxalacetic transaminease(AST), but gamma-glutamyltransferas(GGT), alkaline phosphatase(AKP) and serum bilirubin increased in some cases. Blood coagulation series showed prolonged prothrombin time(PT) and raised fibrinogen(Fib). Hepatomegaly, splenomegaly, broadened portal vein and splenic vein and ascites were identified through imaging examinations, while bone marrow cytological test and bone marrow biopsy showed low bone marrow hyperplasia and teardrop-shaped red blood cells in bone marrow and peripheral blood specimens. Besides, a JAK2V617F positive case was found in genetic tests. Patients usually had long disease courses, and the interval from presentation of portal hypertension features to diagnosis ranged from10days to4years (median6months). Hydroxyurea, thalidomide, interferon-a and erythropoietin were the commonly used medicines.ConclusionPMF can be the cause of patients presenting with portal hypertension. These patients will have the symptoms of portal hypertension, such as hepatomegaly, splenomegaly, broadened portal vein and splenic vein, ascites and esophageal and gastric varices. Abdominal distension, significant splenomegaly, mild-moderate hepatomegaly and mild hepatic injury are the characters. Medical therapy is the common treatment method.