Studio delle glicoproteine piastriniche di membrana in immunofluorescenza. Descrizione di un caso di mielofibrosi idiopatica con piastrinopatia acquisita

Translated title of the contribution: Study of platelet membrane glycoproteins in immunofluorescence. A case report of idiopathic myelofibrosis with acquired platelet dysfunction

A. Santagostino, R. Marl, V. Bolis, G. Camisasca, A. Bosetti, C. Rabascio, E. Figliuzzi, A. M. Mangione, C. Monzeglio, M. R. Conti

Research output: Contribution to journalArticle

Abstract

Clonal haematological disorders can be associated with acquired abnormality of platelet function. Authors describe a case of a 58 years old patient affected by idiopatic myelofibrosis who referred two episodes, occurred in last months, of continous bleeding, prolonged for 7-8 days, after extraction of a tooth; there was no other history of bleeding in the past. The platelet value before extraction was 80.000/μL and even if lower than normal it didn't justify the symptomatology. So we had performed the main coagulation tests. The coagulation screening (PT, PTT) resulted in the range of normality the bleeding time (Ivy test) was higher than 11', the adesivity test showed a reduction of platelet lower than 10% and aggregation test showed defects with ADP, collagen and adrenaline; so we supposed an acquired disorder of platelet function. To confirm this diagnosis, we studied with cytofluorimetric technique the expression of surface membrane glycoprotein of patient's platelets. We have used monoclonal antibodies directed against GPIb (CD42b), GPIX (CD42a), GP IIb (CD41b), GPIIb-IIIa (CD41a) and GPIIIa (CD61). Furthermore, we compared the results with those of two healthy donors, collecting the blood just before the donation. The significance of the differences observed was assessed by Kolmogorov-Smirnov test. Our data showed a significative difference in the expression of GPIb and GPIIb; the D/s(n) value of Kolmogorov-Smirnov test was 34 and 28 respectively with both donors. The reduction of the expression of these GP was simoultanoeusly confirmed by an other operator with a microscope equipped with fluorescence light. These results confirm the diagnosis of an acquired mixed disorder of platelet function with associated aspects of Bernard-Soulier and Glanzmann diseases and the utility of flow cytometric analysis in the study of platelet membrane GP.

Original languageItalian
Pages (from-to)37-41
Number of pages5
JournalTrasfusione del Sangue
Volume43
Issue number1
Publication statusPublished - 1998

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Platelet Membrane Glycoproteins
Primary Myelofibrosis
Fluorescent Antibody Technique
Blood Platelets
Membrane Glycoproteins
Nonparametric Statistics
Platelet Membrane Glycoprotein IIb
Hemorrhage
Integrin beta3
Tooth Extraction
Bleeding Time
Blood Donors
Adenosine Diphosphate
Epinephrine
Collagen
Fluorescence
Monoclonal Antibodies
Tissue Donors
Light
Membranes

ASJC Scopus subject areas

  • Hematology
  • Immunology and Allergy

Cite this

Studio delle glicoproteine piastriniche di membrana in immunofluorescenza. Descrizione di un caso di mielofibrosi idiopatica con piastrinopatia acquisita. / Santagostino, A.; Marl, R.; Bolis, V.; Camisasca, G.; Bosetti, A.; Rabascio, C.; Figliuzzi, E.; Mangione, A. M.; Monzeglio, C.; Conti, M. R.

In: Trasfusione del Sangue, Vol. 43, No. 1, 1998, p. 37-41.

Research output: Contribution to journalArticle

Santagostino, A, Marl, R, Bolis, V, Camisasca, G, Bosetti, A, Rabascio, C, Figliuzzi, E, Mangione, AM, Monzeglio, C & Conti, MR 1998, 'Studio delle glicoproteine piastriniche di membrana in immunofluorescenza. Descrizione di un caso di mielofibrosi idiopatica con piastrinopatia acquisita', Trasfusione del Sangue, vol. 43, no. 1, pp. 37-41.
Santagostino, A. ; Marl, R. ; Bolis, V. ; Camisasca, G. ; Bosetti, A. ; Rabascio, C. ; Figliuzzi, E. ; Mangione, A. M. ; Monzeglio, C. ; Conti, M. R. / Studio delle glicoproteine piastriniche di membrana in immunofluorescenza. Descrizione di un caso di mielofibrosi idiopatica con piastrinopatia acquisita. In: Trasfusione del Sangue. 1998 ; Vol. 43, No. 1. pp. 37-41.
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abstract = "Clonal haematological disorders can be associated with acquired abnormality of platelet function. Authors describe a case of a 58 years old patient affected by idiopatic myelofibrosis who referred two episodes, occurred in last months, of continous bleeding, prolonged for 7-8 days, after extraction of a tooth; there was no other history of bleeding in the past. The platelet value before extraction was 80.000/μL and even if lower than normal it didn't justify the symptomatology. So we had performed the main coagulation tests. The coagulation screening (PT, PTT) resulted in the range of normality the bleeding time (Ivy test) was higher than 11', the adesivity test showed a reduction of platelet lower than 10{\%} and aggregation test showed defects with ADP, collagen and adrenaline; so we supposed an acquired disorder of platelet function. To confirm this diagnosis, we studied with cytofluorimetric technique the expression of surface membrane glycoprotein of patient's platelets. We have used monoclonal antibodies directed against GPIb (CD42b), GPIX (CD42a), GP IIb (CD41b), GPIIb-IIIa (CD41a) and GPIIIa (CD61). Furthermore, we compared the results with those of two healthy donors, collecting the blood just before the donation. The significance of the differences observed was assessed by Kolmogorov-Smirnov test. Our data showed a significative difference in the expression of GPIb and GPIIb; the D/s(n) value of Kolmogorov-Smirnov test was 34 and 28 respectively with both donors. The reduction of the expression of these GP was simoultanoeusly confirmed by an other operator with a microscope equipped with fluorescence light. These results confirm the diagnosis of an acquired mixed disorder of platelet function with associated aspects of Bernard-Soulier and Glanzmann diseases and the utility of flow cytometric analysis in the study of platelet membrane GP.",
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