Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myelofibrosis with myeloid metaplasia (MMM) is a collective term that describes the related disorders AMM, PPMM, and PTMM. The chronic myeloid disorders include chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, and agnogenic myeloid metaplasia (myelofibrosis). These disorders display varying propensities for pathologic enlargement of the spleen which can lead to mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Splenectomy has been found to be of little benefit in the early stages of chronic myeloid leukemia. Similarly, the benefit of splenectomy in advanced cases is limited to symptomatic palliation and treatment of delayed engraftment after allogeneic bone marrow transplantation. Although polycythemia vera and essential thrombocythemia are also characterized by splenomegaly, splenectomy is not considered a therapeutic option in the absence of transformation of the disease into myelofibrosis with myeloid metaplasia. Splenectomy has been studied most in myelofibrosis with myeloid metaplasia. Although there is no clear survival advantage to splenectomy in this disorder, the surgical procedure can result in substantial palliation of mechanical discomfort, hypercatabolic symptoms, portal hypertension, and anemia. However, the procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern.
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PMID:Splenectomy in chronic myeloid leukemia and myelofibrosis with myeloid metaplasia. 1098 48

Approximately two thirds of cases of hepatic flow obstruction are due to myeloproliferative disorders. Restoration of hepatic blood flow is the essential goal of treatment. Thrombolytic therapy seems to achieve good results at least in selected cases. A 32-year-old woman is presented, with an intermittent increase in platelet count (526-725 x 10(9)/L), two previous spontaneous abortions and acute symptomatic occlusion of hepatic veins, and in whom a diagnosis of essential thrombocythemia was initially carried out in agreement with the polycythemia vera study group criteria. She received recombinant tissue plasminogen activator followed by heparin with restoration of normal hepatic outflow. Asymptomatic re-occlusion of the hepatic veins was observed 1 year later, despite adequate continuous warfarin treatment. Angiography showed marked narrowing of the intrahepatic cava vein due to extrinsic compression by an enlarged liver, not due to a new thrombosis so that no specific intervention could be performed. In the presence of a dearly documented hepatic vein thrombosis, thrombolytic therapy should be considered. The patient was given low-molecular-weight heparin with a dramatic reduction in previously elevated fibrinogen level and a good control of the hepatic function.
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PMID:Thrombocytosis and recurrent hepatic outflow obstruction (Budd-Chiari syndrome) after successful thrombolysis: case report and literature review. 1251 87

Here we describe two women with polycythemia vera presented with fulminant hepatic failure due to acute Budd-Chiari syndrome. Both had a history of severe abdominal pain and distention of short duration. Clinical and laboratory findings showed fulminant hepatic failure due to acute Budd-Chiari syndrome. Diagnosis was confirmed with abdominal ultrasonography and Doppler ultrasonography showing ascites, hepatomegaly, portal hypertension and total occlusion of hepatic veins. Complete blood count and other clinical findings were compatible with polycythemia vera in both patients. Patients were treated successfully with early administration of continuous heparin infusion, repeated phlebotomies and hydroxyurea. We emphasize here early diagnosis and effective treatment in such fulminant cases may be life saving.
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PMID:Early medical treatment is life saving in acute Budd-Chiari due to polycythemia vera. 1274 60

We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.
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PMID:[Budd-Chiari syndrome with fatal outcome in a patient with polycythemia vera and antiphospholipid antibody syndrome]. 1552 39

We report a case of a retroperitoneal hematoma occurring in a patient under anticoagulation therapy for deep-venous thrombosis and presenting as an anuric acute renal failure. A coexisting polycythemia vera led to misdiagnosis that could have been life-threatening. A woman, known for polycythemia vera and a single functioning right kidney, was admitted with mild abdominal pain in a context of recent deep venous thrombosis under low-molecular weight heparin. Clinical examination revealed hepatomegaly associated with polycythemia vera. Biochemical evaluation disclosed an acute renal failure, and renal ultrasonography showed no dilation of the renal pelvis. Retroperitoneal hematoma resulted in shock, progressive anemia and obstructive renal failure, related to renal pelvic compression. A right renal indwelling catheter was introduced to restore urine flow after one hemodialysis session, and an inferior vena cava filter was placed because of anti-coagulation contra-indication. However, pulmonary embolism occurred, so that oral anticoagulants were introduced. The hematoma resorbed spontaneously, and a year after this episode, the patient is still alive and well. Retroperitoneal hematoma is a rare cause of obstructive acute renal failure and a life-threatening complication of anti-coagulation therapy.
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PMID:Retroperitoneal hematoma compressing a single functioning kidney: an unusual cause of obstructive renal failure. 1754 41

Involvement of abdominal organs in Osler's disease may lead to the development of hepatic arteriovenous shunts with a dilatation of the hepatic artery. Right and subsequent global heart failure due to cardiac valvular insufficiency, pulmonary artery hypertension, and hepatomegaly as well as increased cardiac output may result. This hyperdynamic hepatic blood flow can be reduced by ligature or banding of the hepatic artery or by orthotopic liver transplantation. We report on two female patients suffering from Osler's disease (68 and 76 years old) with severe heart insufficiency (NYHA III-IV) caused by the high hepatic shunt volumes. A gradual banding of the hepatic artery directed by intraoperative flow measurement in the hepatic artery and control of the systemic hemodynamics by Swan-Ganz or COLD catheters was performed in these patients. The banding was achieved by encasing the hepatic artery in a PTFE cuff (length, 1.0 cm). The high cardiac output could be reduced from 11.2 to 7.0 l/min and from 10.7 to 6.0 l/min, respectively. The respective hepatic artery flow was reduced from 2.0 to 0.3 l/min and from 4.0 to 0.7 l/min. An improvement of heart insufficiency, a reduction in the severity of the cardiac valvular insufficiency, and a reduction of the pulmonary arterial hypertension could be already observed intraoperatively. One patient died of right cardiac failure after an orthotopic liver transplantation 7 months later. The other one died 3 years after the banding. The banding of the hepatic artery controlled by hepatic arterial flow measurement can be considered as an effective and safe palliative procedure in intrahepatic HHT compared to therapeutic alternatives such as hepatic artery ligation or embolization.
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PMID:Treatment of high output cardiac failure by flow-adapted hepatic artery banding (FHAB) in patients with hereditary hemorrhagic telangiectasia. 1802 57

It has been suggested that polycythemia vera (PV) could be preceded by an "early" phase of the disease (e-PV), in which the increase in the red cell parameters is lower than required for a PV diagnosis. In this study, we compared the clinicopathologic and molecular features of 17 patients with e-PV with those of 14 patients with essential thrombocythemia (ET) and 19 with PV. The results for e-PV were more similar to those for PV than for ET. In fact, patients with e-PV were characterized by an increase in the red cell parameters, splenomegaly (P<.05), and hepatomegaly (P=.038), together with hypercellular bone marrow due to increased erythropoiesis and granulopoiesis, associated with megakaryocytic hyperplasia, with pleomorphic aggregates (P<.001). The frequency of the JAK2V617F mutation was similar in e-PV (16 cases tested [100%]) and PV (18/19 [95%]) but was significantly lower (7/13 [54%]) in ET (P=.0007). We propose a diagnostic algorithm helpful to distinguish ET from the early prepolycythemic phase of PV.
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PMID:The significance of bone marrow biopsy and JAK2V617F mutation in the differential diagnosis between the "early" prepolycythemic phase of polycythemia vera and essential thrombocythemia. 1870 5

Budd-Chiari syndrome (BCS) is a rare disorder characterized by hepatic venous obstruction. A 41-year-old male patient presented with right upper quadrant pain, abdominal distension, and dyspnea. He had a history of BCS that was associated with polycythemia vera. Abdominal computed tomography showed hepatomegaly and a hypodense filling defect suggestive of thrombus formation in the hepatic, splenic, and portal veins, and suprahepatic part of the inferior vena cava. Transthoracic echocardiography performed to assess the extension of this pathological process and its relation with intracardiac structures showed a mass lesion in the right atrium, about 4 x 3 cm in diameter. The lesion manifested as an intracardiac thrombus extending from the inferior vena cava. The patient underwent surgical treatment to remove the atrial mass. At surgery, the lesion turned out to be an atrial tumor, which was diagnosed as atrial myxoma in histopathologic examination. The symptoms of the patient resolved after surgery.
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PMID:Right atrial myxoma associated with portal and splenic vein thrombosis in a patient with Budd-Chiari syndrome. 2164 35

Myeloproliferative diseases (MPD) are clonal stem cell disorders which mainly include polycythemia vera (PV), essential thrombocythemia (ET), and idiopathic myelofibrosis (IMF). They are characterized by leucocytosis, thrombocytosis, erythrocytosis, splenomegaly, and bone marrow hypercellularity. This might also result in extramedullary hematopoiesis. Abdominal manifestation has been recognized as a feature of these disorders. Splenomegaly and hepatomegaly are fairly common as opposed to ascites which is rare. The MPDs mainly affect the hepatic circulatory systems. The common hepatic manifestations are Budd-Chiari syndrome (BCS), portal vein thrombosis (PVT), and nodular regenerative hyperplasia. A few other features seen in MPDs are caused by extramedullary hematopoiesis, increased hepatic blood flow, and secondary hemosiderosis from multiple blood transfusions. Portal hypertension is found in up to 7% of patients. We report a case of portal hypertension with ascites in a patient with extramedullary hematopoiesis treated with transjugular intrahepatic portocaval shunt (TIPS).
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PMID:Portal hypertension and ascites in extramedullary hematopoiesis. 2575 27

We describe a 38 year-old woman with polycythemia vera who presented with fulminant hepatic failure due to acute Budd-Chiari syndrome. She had a history of abdominal pain and distention for 4 days. Laboratory and clinical findings showed fulminant hepatic failure due to acute Budd-Chiari syndrome. Diagnosis was confirmed with abdominal ultrasonography and doppler ultrasonography showing ascites, hepatomegaly, portal hypertension and total occlusion of hepatic veins. Complete blood count and other clinical findings were compatible with polycythemia vera. The patient was successfully treated with urgent administration of continuous heparin infusion, repeated phlebotomies and hydroxyurea. We emphasize that early diagnosis and effective treatment in such fulminant cases can be life saving.
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PMID:Polycythemia Vera Presenting with Fulminant Hepatic Failure Due to Acute Budd-Chiari Syndrome: Case Report. 2726 25


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