Latent Myeloproliferative Disorder Research Articles

Adult ?idiopathic? extrahepatic venous thrombosis

The etiology of extrahepatic venous obstruction (EHVO) is unknown in 50% of cases. Recently the presence of a "latent" myeloproliferative disorder has been reported in adults with "idiopathic" EHVO. We evaluated the course of these patients to establish if any putative latent myeloproliferative disorder influenced the clinical course compared to those with a known cause. Among 132 EHVO patients, 78 (59%) had a known etiology, 7 (5%) with an overt myeloproliferative disorder. The "idiopathic" group had 54 patients; 24 (13 men, 11 women) were diagnosed after 15 years of age, (median 38 years, range 17-70) with a median follow up of 96 months (19-372). Only 2 (8%) developed an overt myeloproliferative disorder. These 24 had a similar pattern of bleeding and onset of ascites as those with known cause. In EHVO failure to diagnose a latent myeloproliferative disorder does not influence the course of variceal bleeding, and thus has little prognostic significance.

Ultrasound-Doppler Diagnosis of Budd-Chiari Syndrome

We report a case of apparently idiopathic Budd-Chiari syndrome, diagnosed by ultrasound and Doppler sonography, in a patient with latent myeloproliferative disease. This case proves that Doppler sonography shows in the hepatic veins a flow pattern suggestive of partial thrombotic obstruction. Moreover, we suggest that the search for a latent myeloproliferative disorder, by means of the spontaneous erythroid colonies formation in culture of bone marrow or blood mononuclear cells, should be routinely included in the diagnostic evaluation of each case of hepatic vein thrombosis without other recognizable causes.

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 colonics 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.