Filter by keywords:



Filtering documents. Please wait...

1/21. budd-chiari syndrome associated with factor v leiden mutation: a report of 6 patients.

    budd-chiari syndrome is characterized by hepatic venous outflow obstruction. Although myeloproliferative disorders are usually responsible for this severe thrombotic disorder, deficiency or dysfunction of the natural anticoagulants can be involved. Resistance to activated protein c caused by factor v Leiden mutation has been recently identified as a major cause of thrombophilia. We report 6 patients with budd-chiari syndrome associated with factor v Leiden mutation combined with another acquired thrombophilic state (myeloproliferative disorder and lupus anticoagulant in 3 cases) and without another thrombophilic disorder in the other 3 cases. We conclude that factor v Leiden mutation should be evaluated in any case of hepatic vein occlusion because the prevalence of this mutation in the general population is high.
- - - - - - - - - -
ranking = 1
keywords = vein
(Clic here for more details about this article)

2/21. Erythematous plaques due to platelet plugging: a clue to underlying myeloproliferative disorder.

    We report a syndrome in a middle-aged woman characterized by tender erythematous plaques with histologic evidence of dramatic dermal vessel occlusion. These cutaneous findings occurred in association with progressive inferior vena cava and portal vein thrombosis while on coumarin anticoagulation, following hepatic transplantation for budd-chiari syndrome. The material occluding dermal vessels was proven by immunohistochemical staining to be platelet plugs. These findings led to the diagnosis of an underlying myeloproliferative disorder explaining both her cutaneous and liver abnormalities and institution of appropriate platelet directed anticoagulation with aspirin.
- - - - - - - - - -
ranking = 138.85708011064
keywords = thrombosis, vein thrombosis, vein
(Clic here for more details about this article)

3/21. Fetal hydrops and hepatosplenomegaly in the second half of pregnancy: a sign of myeloproliferative disorder in fetuses with trisomy 21.

    OBJECTIVE: To demonstrate the relationship between fetal hydrops and/or hepatosplenomegaly in the second half of pregnancy with a myeloproliferative disorder in fetuses with trisomy 21 or mosaic trisomy 21. DESIGN: A retrospective case series. SUBJECTS: Cases were selected from 79 cases of trisomy 21 diagnosed in our prenatal unit between 1993 and 1999. methods: All fetuses had a detailed sonographic anatomic survey and biometry. Doppler of the umbilical and middle cerebral arteries, ductus venosus, inferior vena cava and umbilical vein was performed whenever possible. Two-dimensional echocardiography supplemented by color Doppler flow mapping and spectral pulsed wave Doppler was performed in all cases of fetal hydrops. Fetal karyotyping was obtained by amniocentesis, chorionic villus sampling or fetal blood sampling. In the presence of fetal hydrops a cordocentesis was performed for fetal hematology, biochemistry and TORCH serology. In cases with diagnosis of myeloproliferative disorder, peripheral blast cells were characterized by microscopy, cytochemistry and determination of surface markers. All cases with myeloproliferative disorder were stillborn and subsequently had a postmortem examination performed. RESULTS: During the study period 79 cases of trisomy 21 were diagnosed. Eleven of these had fetal hydrops. Three of these fetuses presented with hepatosplenomegaly and myeloproliferative disorder in the second and third trimesters. In addition, one fetus with sonographic markers of trisomy 21, where karyotyping was unfortunately unsuccessful, presented with hepatosplenomegaly, hydrops and myeloproliferative disorder. In the four fetuses with hepatosplenomegaly and hydrops, serology was negative for congenital infection. The characteristics of blast cells in the peripheral blood smear revealed a myeloproliferative disorder. CONCLUSION: Fetal hydrops and/or hepatosplenomegaly in the second half of pregnancy, although suggestive of infectious etiology, may be a sign of myeloproliferative disorder in fetuses with trisomy 21 or mosaic trisomy 21. There is a possibility that a transient myeloproliferative disorder is a more common cause of mid or late-trimester hydrops in cases of trisomy 21 than previously thought. In these hydropic fetuses the prognosis seems to be poor. On the other hand we can speculate that a myeloproliferative disorder and the associated hepatosplenomegaly and/or hydrops may show spontaneous remission or that the transient myeloproliferative disorder may be without any detectable ultrasonographic signs and therefore may be more frequent in utero than realized.
- - - - - - - - - -
ranking = 1
keywords = vein
(Clic here for more details about this article)

4/21. Portal or hepatic vein thrombosis as the first presentation of a myeloproliferative disorder in patients with normal peripheral blood counts.

    myeloproliferative disorders (MPD) are associated with an increased risk of thrombotic complications. We describe three patients with portal or hepatic vein thrombosis and normal peripheral blood counts who had MPD on bone marrow morphology and growth factor-independent megakaryocyte or erythroid colony growth in vitro. The peripheral blood counts have become abnormal subsequently in two patients. patients presenting with unexplained portal or hepatic vein thrombosis should be investigated systematically for the presence of a MPD, which may not be apparent using conventional diagnostic criteria.
- - - - - - - - - -
ranking = 794.20926498208
keywords = thrombosis, vein thrombosis, vein
(Clic here for more details about this article)

5/21. A 25-year clinical history of portopulmonary hypertension associated with latent myeloproliferative disorder.

    Pulmonary hypertension associated with increased pulmonary vascular resistance occurring in the setting of portal hypertension, referred to as "portopulmonary hypertension", is a complication of chronic liver disease, and occurs in 2% to 3% of patients with portal hypertension. Portal hypertension is a relatively common finding in patients with chronic myeloproliferative disorder (CMPD). Pulmonary hypertension is also an occasional finding in CMPD patients. Latent myeloproliferative disorder, on the other hand does not fulfill the diagnostic criteria of classical CMPD and is characterized by younger age of onset, slow disease progression, a high risk of thrombosis, platelet dysfunction, and normal or increased platelet count in spite of the presence of splenomegaly. We report findings in a 50-year-old woman with portal hypertension for which there were three major etiological findings-increased splenic blood flow, infiltration of hematopoietic cells in the liver, and thrombosis in the portal or hepatic vein-over a 25-year clinical course, during which there was also reversible stenosis of the portal vein. Twenty-three years after her first admission, her condition was diagnosed as latent myeloproliferative disorder, and she developed pulmonary hypertension. Her clinical history and data indicated that the portopulmonary hypertension was due to the latent myeloproliferative disorder.
- - - - - - - - - -
ranking = 199.84772885775
keywords = thrombosis, vein
(Clic here for more details about this article)

6/21. erythromelalgia in thrombocythemia of various myeloproliferative disorders.

    erythromelalgia is caused by platelet-mediated acral inflammation and arteriolar thrombosis in thrombocythemia in its primary form or associated with polycythemia vera. The prompt and lasting relief of burning pain by low-dose aspirin is a prerequisite for the diagnosis of thrombocythemic erythromelalgia. Here we extend observations on the occurrence of erythromelalgia in thrombocythemia associated with primary myelofibrosis, philadelphia-chromosome positive micromegakaryocytic myelofibrosis, and myelodysplastic syndrome type II. It is concluded that erythromelalgia may occur in thrombocythemia of all variants of chronic myeloproliferative disease as well as myelodysplastic syndrome if platelet counts are sufficiently high.
- - - - - - - - - -
ranking = 98.923864428873
keywords = thrombosis
(Clic here for more details about this article)

7/21. leg ulcers in elderly on hydroxyurea: a single center experience in Ph- myeloproliferative disorders and review of literature.

    hydroxyurea (HU) is effective in controlling thrombocytosis while reducing the risk of thrombosis in essential thrombocythemia (ET), polycythemia vera (PV) and myelofibrosis (MF). However, HU may carry more or less severe side-effects. Rare cases of patients with painful leg ulcers have been published. We report our experience on such a side-effect in a large cohort of patients with ET and PV treated with HU and review the literature on the topic. Five (4%) out of our 124 patients (69 ET, 51 PV, 4 MF; 49 males, 75 females; mean age at diagnosis 59.1 /-11.8 years) treated with HU developed painful leg ulcers. Sixty-one other patients affected with Phmyeloproliferative disorders (Ph- MPD) developing HU-related painful leg ulcers are described in the English literature. All our five patients were women and developed leg ulcers over the age of 75. Sixty-five percent of all described cases are women; 59% were over 65 years of age and 45% over 70. Most cases received over 1 gr HU per day for at least 1 year. The pathogenesis of HU-induced skin ulcers remains elusive. Treatment is difficult and requires prompt cessation of HU therapy.
- - - - - - - - - -
ranking = 98.923864428873
keywords = thrombosis
(Clic here for more details about this article)

8/21. Hepatic vein thrombosis in Behcet's disease.

    We describe four patients with hepatic vein thrombosis caused by Behcet's disease and review the 17 previously published cases. In addition, we compared these 21 cases of hepatic vein thrombosis to our 24 cases of hepatic vein thrombosis caused by primary myeloproliferative disorders. In patients with Behcet's disease, a male predominance (male/female ratio, 19:1) contrasted with the female predominance found in patients with hepatic vein thrombosis complicating primary myeloproliferative disorders (sex ratio = 1:3). The mean age at clinical onset was younger in patients with Behcet's disease than in those with primary myeloproliferative disorders (29 vs. 35 yr). Obstruction of the inferior vena cava was found in 90% of patients with hepatic vein thrombosis caused by Behcet's disease. Inferior vena caval thrombosis appears to be the main pathophysiological mechanism of hepatic vein thrombosis in patients with Behcet's disease. Unlike patients with primary myeloproliferative disorders who often had a progressive course, one third of patients with Behcet's disease had acute liver failure and died within 2 wk of clinical onset. These findings suggest that, in patients with Behcet's disease, hepatic vein thrombosis is a sudden event usually related to the extension of a caval thrombus to the ostium of the hepatic veins.
- - - - - - - - - -
ranking = 1627.3517456459
keywords = thrombosis, vein thrombosis, vein
(Clic here for more details about this article)

9/21. Membranous obstruction of the inferior vena cava associated with a myeloproliferative disorder: a clue to membrane formation?

    Although membranous obstruction of the inferior vena cava is frequently assumed to be a congenital defect, it has recently been hypothesized that it could be acquired and secondary to thrombosis at the ostium of hepatic veins. We report the case of a membranous obstruction of the inferior vena cava responsible for isolated hepatomegaly in a young Frenchman. No known cause of thrombosis was found but spontaneous formation of erythroid colonies occurred on bone marrow cultures. This indicated the existence of a latent myeloproliferative disorder, a frequent cause of hepatic vein thrombosis. We suggest that this association of a membranous obstruction of the inferior vena cava with a known, albeit often overlooked, cause of hepatic vein thrombosis provides strong support to the thrombosis theory of membrane formation.
- - - - - - - - - -
ranking = 575.4857535079
keywords = thrombosis, vein thrombosis, vein
(Clic here for more details about this article)

10/21. Cerebral venous thrombosis as presenting sign of myeloproliferative disorders.

    Two patients with cerebral venous thrombosis are described. In both patients laboratory findings suggested an underlying haematological disorder and bone marrow biopsy showed a myeloproliferative disorder. Both chronic cerebral venous thrombosis and early myeloproliferative disorders are difficult to diagnose. Their combined occurrence may be less rare than is frequently supposed.
- - - - - - - - - -
ranking = 639.87026947036
keywords = thrombosis, venous thrombosis
(Clic here for more details about this article)
| Next ->


Leave a message about 'Myeloproliferative Disorders'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.