Abstracts SEVENTH ANNUAL MEETING

The European Society for Paediatric Haematology and lmmunology

Oslo, Norway, June 1 1-1 3, 1979

1 I

T I E NOII>IIIL BLOOD PLATELET AND ITS FUNCTION. 11. S t o r m o r k e n , I n s t i t u t e o f T h r o m b o s i s R e s e a r c h , N a t i o n a l H o s p i t a l , Univ. of O s l o , Nonqay.

l l c c c n t r e s e a r c h b o t h on t h e m o r p h o l o g i c a l a n d b i o c h e m i c a l l e v e l h a s g r e a t l y a d v a n c e d o u r knowledge about t h e p l a t e l e t and i t s i n t e r a c t i o n w i t h t h e v e s s e l w a l l a n d p l a s m a t i c f a c t o r s . The p l a t e l e t membrane makes p l a t e l e t s unique i n t h e i r p r o p e r t i e s t o adhere t o o t h e r s u r f a c e s ( a d h e s i o n ) and t o e a c h o t h e r (aggregat i o n ) , t h e main b a s i s f o r t h e i r f u n c t i o n a l t a s k : t o prevent and t o s t o p bleeding. T h i s b a s i c f u n c t i o n i s g o v e r n e d by v e s s e l w a l l f a c t o r s ( e g . c o l l a g e n ) r e p r e s e n t i n g t h e i n i t i a l s t i m u l u s , by p l a s m a t i c f a c t o r s s u c h a s von l l i l l e b r a n d f a c t o r a n d f i b r i n o g e n , b y p l a t e l e t me~nbrane r e c e p t o r s , of w h i c h some a r e i d e n t i f i e d , a n d o f p l a t e l e t membrane p h o s p h o l i p i d s p r o v i d i n g t h e a r a c h i d o n i c a c i d n e c e s s a r y f o r thromboxane s y n t h e s i s a n d f u r t h e r b y compounds c o n t a i n e d i n t h e p l a t e l e t cyt o s o l ( c y c l o o x y g e n a s e , thromboxane s y n t h e t a s e a n d t h e c y c l i c AMP s y s t e m ) , a n d i n t h e g r a n u l e s ( e g . A D P ) , which t o g e t h e r a r e n e c e s s a r y f o r t h e r e l e a s e r e a c t i o n and t h u s f o r p r o v i d i n g t h e p h y s i o l o g i c a l a g g r e g a t i n g s t i m u l u s , ADP. T h e r e l e a s e p r o c e s s c a n a l s o be i m i t a t e d by o t h e r b i o l o g i c a l compounds i n c l u d i n e t h r o m b i n , t h u s l i n k i n g coagulation and p l a t e l e t f u n c t i o n .

2

DE>IONSTTLY~IONOF PLATELEX ANTIBODIES I N I& FRACTION OF CHILDREK WITH ITP. O z s o y l u . S.. Oz-turk, $I., L i i l e l i , Y., S e c t i o n of l i e m a t o l o ~ ; y ,D e p a r t m e n t o f P e d i a t r i c s , H a c e t t e p e U n i v e r s i t y F a c u l t y o f Pledicine, Anlcara, '~'urkey.

Decreased p l a t e l e t s u r v i v a l i n remission a s well a s i n r e l a p s e i n c h i l d r e n w i t h ITP was shown by u s . A n t i p l a t e l c t i n t h e s e r a of t h e s e c h i l d r e n w e r e a l s o shown by u s by u s i n g o p s o n o p h a g o c y t i c t e s t o f I l a n d i n g a n d Stossel. I n t h i s s t u d y , I&, IgN and I&;,\ f r a c t i o n s of the s e r a of t h e s e p a t i e n t s were s e p a r a t e d by column c h r o mato,?raphv a n d t h e p l a t e l e t a n t i b o d i e s were l o o k e d f o r i n t l l e s e f r a c t i o n s by t h e a b o v e method. A l t h o u g h I & and w l ~ o l e serurn shoved s i r n i l a r o p s o n o p h a g o c y t i c a c t i v i t y , i t was n o t shown n e i t h e r i n IgA n o r IgP! f r a c t i o n s . T h i s a c t i v i t y i n I& f r a c t i o n d i s a p p e a r e d f o l l o w i n g b r e a k s o f i t s d i s u l p h i d bonds. F o l l o r i l n g a b s o r p t i o n w i t h e r y t h r o c y t e s , lymphocytes a n d p l a t e l e t s , t h e s p e c i f i c i t y of t h e a n t i b o d i e s t o p l a t c l e t s a r c indicated.

2

A MICRO-bErIIOD FOR DETECTION OF TIIROMl3OCYTE

3 ANTIBODIES. B e r g h , M.S. Beck, E. T h o r s b y , B.G. S o l T i s s u e Typing Laboratory, Rikshospit a l e t , The N a t i o n a l H o s p i t a l , O s l o 1 , Norway

O.J.

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A micro-method us in^ PITC and 1 2 5 ~ - l a b e l l e r lp r o t e i n A f o r d e t e c t i o n o f a u t o a n d a l l o a n t i b o d i c s t o human tllronmbocytes w i l l be p r e s c n t c d . The i s o t o p e method i s b a s e d o n t h e u s e of m i c r o t i t e r p l a t e s w i t h 96 w e l l s a n d Flow S u p e r n a t a n t h a r v e s t e r which p e r m i t s l a r g c s c a l e t e s t i n g of c e l l - s e r u m c o m b i n a t i o n s .

C l i n i c a l a p p l i c a t i o n s of t h e t e s t f o r d e t e c t i o n o f i n vivo and i n v i t r o s e n s i t i z a t i o n of p l a t e l e t s w i l l be d e m o n s t r a t e d t o g e t h e r w i t h c a s c r e p o r t s .

4

VIXCRISTISC LOADED P1,ATELIITS I N TIE TRZATP1:;NT OF IDIOPATIIIC TII:?OPlCOCYTOPCKIC PUItPURA (ITP). G . Schaison, J . L . Ilarousscau, G. Tobelan, Bussel, llopital St-Louis, P a r i s , Prance.

.\.

C h r o n i c ITP i s t h e most common autoitmnunc d i s o r d c r i n c l ~ i l d l l o o d . Splcnectomp i s p r o h i b i d e d b c f o r e 5 y e a r s o l d and a f t e r i s s u c c e s s f u l l i n o n l y 70 :: o f t l l c c a s c s liritll c o r t i c o s t c r o i d s some c h r o n i c ITP seein t o r e s p o n d b u t l o n g tcrnm t r e a t m e n t l e a d s t o u n d c s i r a b l c s i d e c f f c c t . Usc o f i m m u n o s u p p r e s s i v e a t ~ c n t si s open t o c r i t i cisin i n c i ~ i l ~ l i ~ o o d . Y . S . Alln h a s r e p o r L c d t r c a t r n c n t o f r e f r a c t o r y ITP i n a d u l t s r i i t h V i i l b l a s t i n l o a d e d p l a t e l e t s w i t h 6 complet e r e m i s n i o n s , 3 p a r t i a l r e m i s s i o n s and 2 f a i l u r e s . I t i s d e r n o n s t m t c ~ l t l l a t V i n c a i s c o n c e n t r a t c c l b:- t h c p l n t e l e t s . The cor::ple?: i s r a n i d l y c l e a r e d and i i l c : c s t e d by macrop!~a,:c:; leadin!: to their destruction. 10 c h i l d r c n front 2 - t o 1 j y e a r s o l d were t r e a t e d i n o u r departcicni: wit11 V i n c r i s t i n l o a d e d p l a t e l e t s . . \ l l had c h r o n i c IT1' l a s t i n t ; from 1 t o 1 2 y e a r s . s t e r o i d had g i v e n good t r a n s i t o r y r e s u l t s i n 4 c a s e s . 4 c a s c s ]mad been p r e v i o u s l y s p l c n e c t o r n i s e d r * . i t i l f a i l u r e . T h e r e l i e r e 1 food r e s u l t a n d 3 p a r t i a l t r a n s i t o r ) . r e r n i s s i o n s .lnonc 15 a d u l t s wc h a v e 2 , ~ o o dr e s u l t s . Tlle d i s c r e p a n c y between ~ i l n ' s and o u r r e s u l t s i s r l i s . c u s s e d a c c o r r l i n g t o t h e t o t a l amount of v i n c a a l c a l o i d incubated and t h e number o f p l a t e l e t s i n f u s c d .

Abstracts. Seventh annual meeting. The European Society for Paediatric Haematology and Immunology. Oslo, Norway, June 11-13, 1979.

Abstracts SEVENTH ANNUAL MEETING The European Society for Paediatric Haematology and lmmunology Oslo, Norway, June 1 1-1 3, 1979 1 I T I E NOII>II...
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