Polycythemia and the budd-chiari syndrome: Study of serum erythropoietin and bone marrow erythroid progenitors
✍ Scribed by Victor Georges Levy; Agnès Ruskone; Claude Baillou; Diana Thierman-Duffaud; Albert Najman; Georges Albert Boffa
- Publisher
- John Wiley and Sons
- Year
- 1985
- Tongue
- English
- Weight
- 382 KB
- Volume
- 5
- Category
- Article
- ISSN
- 0270-9139
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✦ Synopsis
The mechanism of polycythemia associated with the Budd-Chiari syndrome is unknown. Erythropoiesis in 10 patients with Budd-Chiari syndrome was studied in an attempt to distinguish prior unrecognized polycythemia Vera from secondary polycythemia.
Serum erythropoietin was assayed using a mouse fetal liver erythroblast assay. High concentrations of serum erythropoietin were observed in 6 of 7 patients with acute primary Budd-Chiari syndrome. Levels were normal in four patients who were investigated during the chronic phase and were increased in one with persisting polycythemia. In one patient, erythropoietin concentration in the hepatic vein was twice the level measured in peripheral, caval and renal venous blood.
Bone marrow erythroid progenitors developed in vitro in the absence of exogenous erythropoietin in all polycythemia Vera cases studied in acute and chronic phases, whether polycythemia persisted or not.
These findings indicate that hepatic erythropoietin production occurs in the acute phase of Budd-Chiari syndrome and suggest that, in some cases of Budd Chiari syndrome, polycythemia which resolves after the acute phase may be secondary to liver disease.
Polycythemia is frequently observed in the course of the Budd-Chiari syndrome (BCS). In the current study, 39 of 50 patients displayed a significant increase in total red blood cell volume. The relationship between polycythemia and BCS is controversial. BCS with increased red blood cell count is considered a complication of preexisting, unrecognized polycythemia Vera (PV) by some authors (1). For others, polycythemia is considered secondary to the hepatic vein occlusion (2). Until recently, it was impossible to differentiate between these possibilities for lack of reliable diagnostic criteria. Two types of polycythemia can be distinguished: in primary polycythemia, erythroid progenitors cells are unusually sensitive to erythropoietin (Epo) and proliferate despite the presence of low serum Epo levels. In secondary polycythemia, erythroid progenitors cells respond normally to Epo but their numbers are raised in response to