MULTILINEAGE HEMATOPOIETIC STEM-CELL DEFECTS IN BUDD-CHIARI SYNDROME

Background/Aims: Erythropoietin-independent endogenous growth of eryth roid colony from bone marrow cells has been shown in many patients wit h Budd Chiari syndrome in earlier studies. In another report, increase d megakaryocyte colony growth has also been documented in this disease . However, defects in granulocyte-macrophage cell lines in Budd Chiari syndrome have yet to be reported in the literature. Methods: Both in vitro erythroid and granulocyte-macrophage colony cultures from periph eral blood mononuclear cells with and without erythropoietin or granul ocyte-macrophage colony stimulating factor, respectively, were studied in 32 patients with Budd Chiari syndrome, along with 20 normal health y controls and ten patient controls with portal hypertension (five pat ients each with noncirrhotic portal fibrosis and liver cirrhosis), In 18 patients the occlusion was only in the inferior vena cava, in five patients only in hepatic veins, and in nine patients both inferior ven a cava and hepatic veins were blocked. Result: Endogenous erythroid or granulocyte-macrophage colony growth was not observed in any of the n ormal healthy controls or in patient controls with portal hypertension . However, 22 of the 32 (68.8%) patients showed endogenous erythroid c olony growth. Moreover, four of them also showed endogenous growth of granulocyte-macrophage colony, not previously reported in Budd Chiari syndrome.Conclusion: It may be inferred that stem cell defects affecti ng all three hemopoietic cell lines (erythroid, megakaryocyte and gran ulocyte-macrophage) occur in Budd Chiari syndrome, which may be the pr imary defect responsible for the pro-thrombotic state causing venous t hrombosis in them.