Thromb Haemost 1995; 74(04): 1045-1049
DOI: 10.1055/s-0038-1649879
Original Article
Clinical Studies
Schattauer GmbH Stuttgart

Alterations in Vascular Endothelial Cell-related Plasma Proteins In Thalassaemic Patients and their Correlation with Clinical Symptoms

P Butthep
1   The Blood Disease Research Unit, Sirikit Medical Centre, Bangkok, Japan
,
A Bunyaratvej
2   The Department of Pathology, Faculty of Medicine, Rhamathibodi Hospital, Bangkok, Japan
,
Y Funahara
5   The First Department of Physiology, Kobe University School of Medicine, Kobe, Japan
,
H Kitaguchi
5   The First Department of Physiology, Kobe University School of Medicine, Kobe, Japan
,
S Fucharoen
3   The Thalassaemia Centre, Bangkok, Japan
,
S Sato
6   The Department of Hygiene, Kobe University School of Medicine, Kobe, Japan
,
N Bhamarapravati
4   The Vaccine Centre, Salaya, Mahidol University, Thailand
› Author Affiliations
Further Information

Publication History

Received 07 November 1994

Accepted 27 June 1995

Publication Date:
27 July 2018 (online)

Summary

An increased level of plasma thrombomodulin (TM) in α- and β- thalassaemia was demonstrated using an enzyme-linked immunosorbent assay (ELISA). Nonsplenectomized patients with β-thalassaemia/ haemoglobin E (BE) had higher levels of TM than splenectomized cases (BE-S). Patients with leg ulcers (BE-LU) were found to have the highest increase in TM level. Appearance of larger platelets in all types of thalassaemic blood was observed indicating an increase in the number of younger platelets. These data indicate that injury of vascular endothelial cells is present in thalassaemic patients.

 
  • References

  • 1 Sonakul D, Suwanagool P, Sirivaidyapong P, Fucharoen S. Distribution of pulmonary thromboembolism lesions in thalassemic patients. Birth Defects 1988; 23 5A 375-384
  • 2 Sonakul D, Fucharoen S. Pulmonary thromboembolism in thalassemic patients. Southeast Asian J Trop Med Public Health 1992; 23 suppl (Suppl. 02) 25-28
  • 3 Dexter L. Thromboemboli as a cause of cor pulmonale. Bull NY Acad Med 1965; 41: 481-488
  • 4 Ferrer MI. Cor pulmonale (pulmonary heart disease): present day status. Am Heart J 1975; 89: 657-664
  • 5 Pascher F, Keen R. Ulcer of legs in Cooley’s anemia. New Engl J Med 1957; 256: 1220-1222
  • 6 Wong V, Yu Y, Lairig RH S, Tso WK, Li AM C, Chan TK. Cerebral thrombosis in β-thal/Hb E disease. Stroke 1990; 21: 812-816
  • 7 Butthep P, Bunyaratvej A, Kitaguchi H, Funahara Y, Fucharoen S. Interaction between endothelial cells and thalassemic red cells in vitro. Southeast Asian J Trop Med Public Health 1992; 23 suppl (Suppl. 02) 101-104
  • 8 Bunyaratvej A, Funahara Y, Butthep P, Kitaguchi H, Fucharoen S. Thalassemic serum impairs endothelial cell growth in vitro. Southeast Asian J Trop Med Public Health 1992; 23 suppl (Suppl. 02) 105-109
  • 9 Opartkiattikul N, Funahara Y, Hijikata-Okunomiya A, Yamaguchi N, Fucharoen S, Talalak P. Detection of PF3 activity in whole blood from volunteers and β thalassemia/Hb E patients: a promising method for prediction of thrombotic tendency. Southeast Asian J Trop Med Public Health 1992; 23 suppl (Suppl. 02) 52-59
  • 10 Sumiyoshi A, Thakerngpol K, Sonakul D. Pulmonary microthromboemboli in thalassemia cases. Southeast Asian J Trop Med Public Health 1992; 23 suppl (Suppl. 02) 29-31
  • 11 Ishii H, Majerus PW. Thrombomodulin is present in human plasma and urine. J Clin Invest 1985; 76: 2178-2181
  • 12 Ishii H, Nakano M, Tsubouchi J, Ishikawa T, Uchiyama H, Hiraishi S, Tahara C, Miyajima Y, Kazama M. Establishment of enzyme immunoassay of human thrombomodulin in plasma and urine using monoclonal antibodies. Thromb Haemost 1990; 63: 157-162
  • 13 Takano S, Kimura S, Ohdama S, Aoki N. Plasma thrombomodulin in health and disease. Blood 1990; 76: 2024-2029
  • 14 Amiral J, Adam M, Minilla F, Larrivaz I, Chambrette B, Boffa MC. A new assay for soluble forms of thrombomodulin in plasma. Thromb Haemost 1991; 65: 947
  • 15 Cejka J. Enzyme immunoassay for factor VUI-related antigen. Clin Chem 1982; 28: 1356-1358
  • 16 Dittman WA, Majerus PW. Structure and function of thrombomodulin. A natural anticoagulant Blood 1990; 75: 329-336
  • 17 Ogura M, Ito T, Maruyama I, Takamatsu J, Yamamoto S, Ogawa K, Nagura H, Saito H. Localization and biosynthesis of functional thrombomodulin in human megakaryocytes and a human megakaryoblastic cell line (MEG-01). Thromb Haemost 1990; 64: 297-301
  • 18 Conway E, Nowakowski B, Steiner-Mosonyi M. Human neutrophils synthesize thrombomodulin that does not promote thrombin-dependent protein C activation. Blood 1992; 80: 1254-1263
  • 19 McCachren SS, Diggs J, Weinberg JB, Dittman WA. Thrombomodulin expression by human blood monocytes and by human synovial tissue lining macrophages. Blood 1991; 78: 3128-3132
  • 20 Suzuki K, Nishioka J, Hayashi T, Kosaka Y. Functionally active thrombomodulin is present in human platelets. J Biochem 1988; 104: 628-632
  • 21 Maruyama I, Bell CE, Majerus PW. Thrombomodulin is found on endothelium of arteries, veins, capillaries, lymphatics, and syncytiotrophoblast of human placenta. J Cell Biol 1985; 101: 363-371
  • 22 Moore KL, Esmon CT, Esmon NL. Tumor necrosis factor leads to the internalization and degradation of thrombomodulin from the surface of bovine aortic endothelial cells in culture. Blood 1989; 73: 159-165
  • 23 Ishii H, Uchiyama H, Kazama M. Soluble thrombomodulin antigen in conditioned medium is increased by damage of endothelial cells. Thromb Haemost 1991; 65: 618-623
  • 24 Asakura H, Jokaji HM S, Uotani C, Kumabashiri I, Morishita E, Yamazaki M, Matsuda T. Plasma levels of soluble thrombomodulin increase in cases of disseminated intravascular coagulation with organ failure. Am J Hematol 1991; 38: 281-287
  • 25 Takahashi H, Ito S, Hamano M, Wada K, Niwano H, Seki Y, Shibata A. Circulating thrombomodulin as a novel endothelial cell marker: Comparison of its behavior with von Willebrand factor and tissue-type plasminogen activator. Am J Hematol 1992; 41: 32-39
  • 26 Seigneur M, Dufourcq P, Conri C, Constans J, Mercie P, Pruvost A, Amiral J, Midy D, Baste J-C, Boisseau MR. Levels of plasma thrombomodulin are increased in atheromatous artherial disease. Thromb Res 1993; 71: 423-431
  • 27 Ohdama S, Takano S, Miyake S, Kubota T, Sato K, Aoki N. Plasma Thrombomodulin as a Marker of Vascular Injuries in Collagen Vascular Diseases. Am J Clin Patho 1994; 101: 109-113
  • 28 Shirahata A, Funahara Y, Opartkiattikul N, Fucharoen S, Laosombat V, Yamada K. Protein C and protein S deficiency in thalassemic patients. Southeast Asian J Trop Med Public Health 1992; 23 suppl (Suppl. 02) 65-73
  • 29 Kumada T, Dittman WA, Majerus PW. A role for Ihrombomodulin in the pathogenesis of thrombin-induced thromboembolism in mice. Blood 1988; 71: 728-733
  • 30 Manned PM, Sharp AA. Platelet volume and shape in relation to aggregation and adhesion. Br J Haematol 1967; 13: 604-617
  • 31 Karpatokin S. Heterogeneity of human platelets. I. Metabolic and kinetic evidence suggestive of young and old platelets J Clin Invest 1969; 48: 1073-1082
  • 32 Eldor A, Kraus Y, Atlan H, Snyder D, Goldfarb A, Hy-AM H, Rachmilcwitz EA, Kotze HF. Heyns A du P. Platelet survival in patients with BetaThalassemia Am J Hematol 1989; 32: 94-99