Thrombopoietin in Patients With Congenital Thrombocytopenia and Absent Radii: Elevated Serum Levels, Normal Receptor Expression, But Defective Reactivity to Thrombopoietin
نویسندگان
چکیده
منابع مشابه
Thrombopoietin in patients with congenital thrombocytopenia and absent radii: elevated serum levels, normal receptor expression, but defective reactivity to thrombopoietin.
The pathophysiology of thrombocytopenia in the syndrome of thrombocytopenia with absent radii (TAR) is not yet understood. We examined thrombopoietin (TPO) serum levels and the in vitro reactivity of platelets to TPO in five patients affected with TAR syndrome. We found elevated TPO serum levels in all patients tested, excluding a TPO production defect as cause for thrombocytopenia in TAR syndr...
متن کاملTwo patterns of thrombopoietin signaling suggest no coupling between platelet production and thrombopoietin reactivity in thrombocytopenia-absent radii syndrome.
BACKGROUND Thrombocytopenia with absent radii syndrome is defined by bilateral radius aplasia and thrombocytopenia. Due to impaired thrombopoietin signaling there are only few bone marrow megakaryocytes and these are immature; the resulting platelet production defect improves somewhat over time. A microdeletion on chromosome 1q21 is present in all patients but is not sufficient to form thromboc...
متن کاملSerum thrombopoietin levels in cyclic thrombocytopenia.
Cyclic thrombocytopenia is a rare disorder characterized by periodic decreases and increases in platelet levels. Recent data suggest these fluctuations result from periodic failure of effective platelet production or periodic immune-mediated platelet destruction.’.’ However, the pathogenesis of this disorder is uncertain. We developed an enzyme-linked immunosorbent assay (ELISA) of serum thromb...
متن کاملPatients with thrombocytosis have normal or slightly elevated thrombopoietin levels.
BACKGROUND AND OBJECTIVE The distinction between clonal and reactive thrombocytoses is a frequent problem and implies different therapeutic options. As thrombopoietin (TPO) is the main regulator of megakaryocytopoiesis and thrombopoiesis, we measured TPO levels in patients with thrombocytosis in an attempt to understand the regulation and potential utility of distinguishing thrombocytoses. DE...
متن کاملserum thrombopoietin levels and its relationship with thrombocytopenia in patients with cirrhosis
conclusions our results suggest that liver cirrhosis does not cause impaired thrombopoietin production even in the late stage of disease. thrombopoietin has no contribution for the occurrence of thrombocytopenia in cirrhosis; splenic sequestration seems to be the main factor. results platelet counts were lower in patients with cirrhosis (97000 ± 8000/mm3) than in healthy subjects (240000 ± 5100...
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ژورنال
عنوان ژورنال: Blood
سال: 1997
ISSN: 1528-0020,0006-4971
DOI: 10.1182/blood.v90.2.612