TY - JOUR
T1 - Disturbance of plasma and platelet thrombospondin levels in sickle cell disease
AU - Browne, Paul V.
AU - Mosher, Deane F.
AU - Steinberg, Martin H.
AU - Hebbel, Robert P.
PY - 1996/4
Y1 - 1996/4
N2 - Thrombospondin (TSP), a large protein found in platelet α-granules (as TSP-1), mediates adhesion of sickle reticulocytes to cultured vascular endothelium. To further explore the physiologic relevance of this observation, we have measured plasma TSP levels and platelet TSP-1 content in subjects with sickle cell disease. Plasma TSP levels were similar for normal-controls (mean 491 ng/ml, range 331-723) and steady-state HbSS patients (mean 536, range 333-1107) and were significantly (P = 0.912) but variably elevated for HbSS patients presenting with acute painful crisis (mean 868, range 442-2780). Some of these elevated plasma TSP levels reached those previously observed to support maximal red cell adhesion to endotheliumin vitro, compared to normals, both steady-state and in-crisis HbSS patients had significantly (P < 0.001) depressed platelet TSP-1 content (82.6 ± 11.9, 47.1 ± 16.0 and 45.9 ± 20.7 ng/106 platelets, respectively, mean ±SD). HbSC disease patients, all examined during steady state, had low-normal plasma levels of TSP and either normal or depressed platelet TSP-1 content. Serial observations on three sickle cell anemia subjects indicated a probable relationship between platelet TSP-1 release, elevated plasma TSP levels, and acute vasoocclusive episodes. These results suggest a State of ongoing release and depletion of TSP-1 from activated platelets in patients with sickle cell disease.
AB - Thrombospondin (TSP), a large protein found in platelet α-granules (as TSP-1), mediates adhesion of sickle reticulocytes to cultured vascular endothelium. To further explore the physiologic relevance of this observation, we have measured plasma TSP levels and platelet TSP-1 content in subjects with sickle cell disease. Plasma TSP levels were similar for normal-controls (mean 491 ng/ml, range 331-723) and steady-state HbSS patients (mean 536, range 333-1107) and were significantly (P = 0.912) but variably elevated for HbSS patients presenting with acute painful crisis (mean 868, range 442-2780). Some of these elevated plasma TSP levels reached those previously observed to support maximal red cell adhesion to endotheliumin vitro, compared to normals, both steady-state and in-crisis HbSS patients had significantly (P < 0.001) depressed platelet TSP-1 content (82.6 ± 11.9, 47.1 ± 16.0 and 45.9 ± 20.7 ng/106 platelets, respectively, mean ±SD). HbSC disease patients, all examined during steady state, had low-normal plasma levels of TSP and either normal or depressed platelet TSP-1 content. Serial observations on three sickle cell anemia subjects indicated a probable relationship between platelet TSP-1 release, elevated plasma TSP levels, and acute vasoocclusive episodes. These results suggest a State of ongoing release and depletion of TSP-1 from activated platelets in patients with sickle cell disease.
KW - Platelets
KW - Sickle cell anemia
KW - Thrombospondin
UR - http://www.scopus.com/inward/record.url?scp=0029979392&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029979392&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1096-8652(199604)51:4<296::AID-AJH8>3.0.CO;2-R
DO - 10.1002/(SICI)1096-8652(199604)51:4<296::AID-AJH8>3.0.CO;2-R
M3 - Article
C2 - 8602630
AN - SCOPUS:0029979392
SN - 0361-8609
VL - 51
SP - 296
EP - 301
JO - American Journal of Hematology
JF - American Journal of Hematology
IS - 4
ER -