27 REEVALUATION OF 19 CHILDREN ONCE FOUND TO BE DEFICIENT IN SERUM IgA

Abstract

Sincc 1971, 7 children with AL ( 5 ALL, 2 ANL) have been transplanted a t the University Children's Hospital. 2 of the donors wcre i d e n t i c a l twins, 4 wcre s i b l i n g s , 1 the HLA-identical father. Thc ALL children were transplanted a f t e r 2 to 4 relapses, the ANL e a r l i c r . 3 were i n C R , 2 in PR and 2 i n relapse. A l l got i n t r n s i v e chemotherapy, and 5 t o t a l body i r r a d i a t i o n (TBI).3 died of early complications, a l l others showed a take. 1 boy ( W / O TBI) relapsed a f t e r 2 months, 1 with an AML had a t e s t i c u l a r relapse a f t e r 4 months, but i s i n CR again since 10+ months under chcmothcrapy. The most recent p a t i e n t shovs a normal bone marrow, and no acute G v H , a f t e r 1 month. 1 boy s t a y s i n CR since 40+ mo.Our r e s u l t s suggest, and those from S e a t t l e show t h a t bone marrow transplantation in AL i s f e a s i b l e . As i n t e r s t i t i a l pneumonia and relapse a r e the most important obstacles t o success, the children should be in remission ( t o s t a r t with a lower tumor load) and gct an intensive c y t o s t a t i c pretreatment, but -to avoid pneumonitis -should not get more than 750 r t o the lungs. The enzyme 5-formyl t e t r a h y d r o f o l a t e cyclodehydrase c o n v e r t s 5-formyl t e t r a h y d r o f o l a t e t o 5,lO-methenyl t e t r ahydrofo1ate.A method has been devised f o r t h e cytochemical demonstration of t h i s enzyme u s i n g 5-formyl t e t r a h y d r o f o l at e a s substrate.The a c t i v i t y of t h e enzyme has been s t u d i e d i n l e u k o c y t e s of c h i l d r e n with a c u t e 1eukaemia.Comparison of enzymatic a c t i v i t y i n t h e same t y p e s of c e l l s showed no s i g n i f i c a n t v a r i a t i o n between normal c o n t r o l s and t h e p a t i e n t s w i t h a c u t e 1eukaemia.The enzyme a c t i v i t y was found t o i n c r e a s e w i t h c e l l m a t u r a t i o n and was s t r o n g l y p o s i t i v e i n polymorphonuclear l e u k o c y t e s and eosinophils.The b l a s t c e l l s of p a t i e n t s w i t h a c u t e leukaemia were weakly p o s i t i v e o r negative. T h i s f i n d i n g i s of i n t e r e s t s i n c e t h e b l a s t c e l l s a r e c a p a b l e of division.Two p o p u l a t i o n s of lymphocyt e s were observedrone p o s i t i v e w i t h a few g r a n u l e s and one negative.Methotrexote decreased enzyme a c t i v i t y i n some p a t i e n t s ; t h e enzyme may be a t a r g e t f o r t h e a c t i o n of f o l i c a c i d a n t a g o n i s t s . T h e a b s olute number a n d t l~e p r o p o r t i o n of I & a n d I g I I -s u r f ace-bcarin;: 1 1 -c e l l s a n d t l~e " n u l l " -c e l l s w e r e e l e v at e d . I n c l~i l d r c n r r i t l~o u t i n c r e a s e d i n f e c t i o n s t i l e p r o p o r t i o n o f T -c e l l s o n l y was d c c r c a s e d . K i t h r e -{:art1 t o c l u r ; i t i o n , s c v c r i t : -o r d i s e a s e , s o r t of a n t i -::en, s e r u~.~ I c -: : -l c v c l s , c o s ...