May-June 03
03-06-03
16:55
™ÂÏ›‰·196
¶·È‰È·ÙÚÈ΋ 2003;66:195-201
Paediatriki 2003;66:195-201
(3 ·È‰È¿). ∆· ÌÈÛ¿ ·È‰È¿ Ì HD (50%) ‹Ù·Ó ËÏÈΛ·˜ >10 ¯ÚfiÓˆÓ, ÂÓÒ ÙÔ 72,7% ÙˆÓ NHL ‹Ù·Ó ËÏÈΛ·˜ 5-10 ¯ÚfiÓˆÓ. ∆Ô 77,3% ÙˆÓ NHL ‹Ù·Ó ·ÁfiÚÈ·, ÂÓÒ ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÔÛÔÛÙfi ÙˆÓ HD ‹Ù·Ó 62,5%. ¶˘ÚÂÙfi ›¯Â ÙÔ 56,3% ÙˆÓ HD, ¤Ó·ÓÙÈ 27,3% ÙˆÓ NHL. ªÂ ̤ÛÔ ¯ÚfiÓÔ ·Ú·ÎÔÏÔ‡ıËÛ˘ Ù· 4,8 ¤ÙË, Ë EFS Û ÚÒÙË ‡ÊÂÛË Â›Ó·È 77,05% (±6,58%) Î·È Ë OS 91,27% (±4,36%). ∏ ·Ó¿Ï˘ÛË Ù˘ Èı·ÓfiÙËÙ·˜ EFS Û ۯ¤ÛË Ì ÙÔÓ Ù‡Ô ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Â›Ó·È 71,79% (±11,58%) ÁÈ· Ù· ·È‰È¿ Ì HD, ¤Ó·ÓÙÈ 79,6% (±8,02%) ÁÈ· ÂΛӷ Ì NHL. ∞fi ÙÔ Û‡ÓÔÏÔ ÙˆÓ 16 ·È‰ÈÒÓ Ì HD Î·È ÙˆÓ 22 Ì NHL, ˘ÔÙÚÔ›·Û·Ó 5 Î·È 5 ·È‰È¿, ·ÓÙ›ÛÙÔȯ·. ∞fi ·˘Ù¿ Ù· ·ÔÙÂϤÛÌ·Ù· ÚÔ·ÙÂÈ fiÙÈ Ë OS Î·È Ë EFS, Ì ٷ Û‡Á¯ÚÔÓ· ıÂڷ¢ÙÈο ÚˆÙfiÎÔÏÏ·, ηٷÁÚ¿ÊËÎ·Ó Û ϋÚË ·ÓÙÈÛÙÔȯ›· Ì ٷ ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ. ™˘ÁÎÚÈÙÈο ÚÔ·ÙÂÈ Î·Ï‡ÙÂÚË ¤Î‚·ÛË ÁÈ· Ù· ·È‰È¿ Ì NHL, Ô˘ ÔÊ›ÏÂÙ·È Èı·ÓfiÓ ÛÙËÓ ·ÚÔ˘Û›· ÎÏÈÓÈÎÒÓ Û˘ÌÙˆÌ¿ÙˆÓ Ô˘ ÂÈ‚¿ÏÏÔ˘Ó ÙËÓ ¤ÁηÈÚË ÚÔۤϢÛË, ‰È¿ÁÓˆÛË Î·È ıÂڷ¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË.
NHL. Relapses occurred in 5/16 and 5/22 children with HD and NHL respectively. In conclusion, the OS and EFS in this group of children were equivalent to those treated with modern chemotherapy protocols reported in the international bibliography. Comparing HD and NHL, better outcome for children with NHL was noted, possibly due to more timely admission, diagnosis and start of treatment.
§¤ÍÂȘ ÎÏÂȉȿ: ÓfiÛÔ˜ Hodgkin, ÌË Hodgkin ϤÌʈ̷, ÂÈ‚›ˆÛË ÂχıÂÚË ÓfiÛÔ˘, ·È‰È΋ ËÏÈΛ·.
Key words: Hodgkin disease, non-Hodgkin lymphoma, event free survival, childhood.
∂ÈÛ·ÁˆÁ‹ ∆· ÏÂÌÊÒÌ·Ù· ·ÔÙÂÏÔ‡Ó ÙÔ ÙÚ›ÙÔ Î·Ù¿ Û˘¯ÓfiÙËÙ· ηÎfiËı˜ ÓfiÛËÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ÌÂÙ¿ ÙËÓ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Î·È ÙÔ˘˜ ÚˆÙÔ·ı›˜ fiÁÎÔ˘˜ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ·ÓÙÈÚÔۈ‡ÔÓÙ·˜ ÂÚ›Ô˘ ÙÔ 10-15% ÙˆÓ Î·ÎÔËıÂÈÒÓ (1). ¢È·ÎÚ›ÓÔÓÙ·È ÛÙ· ÌË Hodgkin ÏÂÌÊÒÌ·Ù· (NHL) Î·È ÛÙ· Hodgkin ÏÂÌÊÒÌ·Ù· (HD). ∆· NHL ÂÌÊ·Ó›˙ÔÓÙ·È Û˘¯ÓfiÙÂÚ· Û ۯ¤ÛË Ì ٷ HD ÛÙËÓ ·È‰È΋ ËÏÈΛ· (·Ó·ÏÔÁ›· 3/2), ÂÓÒ Ë Î·Ù·ÓÔÌ‹ ÛÙ· ‰‡Ô ʇϷ Â›Ó·È 3:1 Ì ˘ÂÚÔ¯‹ ÙˆÓ ·ÁÔÚÈÒÓ (1-3). ∆· NHL ·ÔÙÂÏÔ‡Ó Î·ÎfiËı˜ ÓfiÛËÌ· ÙÔ˘ ÏÂÌÊÈÎÔ‡ ÈÛÙÔ‡ (ÏÂÌÊ·‰¤Ó˜ ‹ Â͈ÏÂÌÊ·‰ÂÓÈο fiÚÁ·Ó·). ∞ÓÙÈÚÔÛˆÂ‡Ô˘Ó ÂÚ›Ô˘ ÙÔ 7-10% ÙˆÓ Î·ÎÔ‹ıˆÓ ÓÔÛËÌ¿ÙˆÓ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ (2,4,5). ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ NHL Â›Ó·È ˘„ËÏfiÙÂÚË ÛÙËÓ ∞ÊÚÈ΋, ÁÂÁÔÓfi˜ Ô˘ ÔÊ›ÏÂÙ·È ÛÙËÓ ·ÚÔ˘Û›· ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Burkitt ÛÙËÓ ÂÚÈÔ¯‹, ÙÔ ÔÔ›Ô Û¯ÂÙ›˙ÂÙ·È ¿ÌÂÛ· Ì ÙÔÓ Èfi Epstein-Barr (EBV) Û ÔÛÔÛÙfi ¤ˆ˜ Î·È 95% (2,4,6). ∞ÓÙ›ıÂÙ·, ÛÙËÓ ∂˘ÚÒË Î·È ÛÙË µfiÚÂÈ· ∞ÌÂÚÈ΋, ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÙˆÓ NHL Â›Ó·È ∆ ‹ µ (ÌË Burkitt) ΢ÙÙ·ÚÈ΋˜ ÚÔ¤Ï¢Û˘ Î·È ÌfiÓÔ ÙÔ 10-15% ʤÚÂÈ ÙÔ Á¤ÓˆÌ· ÙÔ˘ EBV (5). °ÂÓÂÙÈÎÔ› ÚԉȷıÂÛÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· ÙËÓ ·ıÔÁ¤ÓÂÛË ÙˆÓ NHL Â›Ó·È ÔÈ ÎÏËÚÔÓÔÌÈΤ˜ ·ÓÔÛÔ·Ó¿ÚÎÂȘ, fiˆ˜ ÙÔ Û‡Ó‰ÚÔÌÔ Wiskott-Aldrich, Ë ·Ù·Í›· ÙËÏ·ÁÁÂÈÂÎÙ·Û›· Î·È ÙÔ Ê˘ÏÔÛ‡Ó‰ÂÙÔ ÏÂÌÊÔ¸ÂÚÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ, ηıÒ˜ Î·È ÔÈ Â›ÎÙËÙ˜ ·ÓÔÛÔ·Ó¿ÚÎÂȘ, fiˆ˜ Ë ·ÓÔ-
ÛÔηٷÛÙÔÏ‹ Ô˘ ·ÎÔÏÔ˘ı› ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ‹ ÙÔ˘ ‹·ÙÔ˜ (1,2). ÕÏÏÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ Úԉȷı¤ÙÔ˘Ó ÛÙËÓ ÂΉ‹ÏˆÛË ÙˆÓ NHL Â›Ó·È Ë ·ÎÙÈÓÔ‚ÔÏ›· Î·È ÔÚÈṲ̂ÓÔÈ ÈÔ› fiˆ˜ Ô EBV Î·È Ô Èfi˜ Ù˘ ›ÎÙËÙ˘ ·ÓÔÛÔ·Ó¿ÚÎÂÈ·˜ (HIV). ∏ ÓfiÛÔ˜ ÙÔ˘ Hodgkin ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÚÔԉ¢ÙÈ΋, ·ÓÒ‰˘ÓË ‰ÈfiÁΈÛË ÙˆÓ ÏÂÌÊ·‰¤ÓˆÓ, Û˘¯ÓfiÙÂÚ· Ù˘ ÙÚ·¯ËÏÈ΋˜ ¯ÒÚ·˜. ∏ ÓfiÛÔ˜ ·Ú¯Èο ÚÔÛ‚¿ÏÏÂÈ ¤Ó· ÌÔÓ‹ÚË ÏÂÌÊ·‰¤Ó· ‹ ÔÌ¿‰· ÏÂÌÊ·‰¤ÓˆÓ, ÂÓÒ Û ÚÔ¯ˆÚË̤ÓÔ ÛÙ¿‰ÈÔ ÌÔÚ› Ó· ÂÈÓ¤ÌÂÙ·È Î·È fiÚÁ·Ó·. ∏ η̇ÏË Û˘¯ÓfiÙËÙ·˜ ·ÚÔ˘ÛÈ¿˙ÂÈ Ì›· ·È¯Ì‹ Û ËÏÈΛ· 15-35 ÂÙÒÓ Î·È Ì›· ‰Â‡ÙÂÚË Û ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘ (>50 ¯ÚfiÓÈ·) (1,4,7). ™ÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ÓfiÛÔ˘ ÂÓ¤¯ÔÓÙ·È ·ÓÔÛÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÂÓÒ ·Ú·ÙËÚÂ›Ù·È ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· Û ¿ÙÔÌ· Ì ÓÔÛ‹Ì·Ù· fiˆ˜ Ô Û˘ÛÙËÌ·ÙÈÎfi˜ ÂÚ˘ıËÌ·Ù҉˘ χÎÔ˜ (™∂§), Ë ÚÂ˘Ì·ÙÔÂȉ‹˜ ·ÚıÚ›Ùȉ· Î·È Ë ·Ù·Í›· ÙËÏ·ÁÁÂÈÂÎÙ·Û›· (1,7,8). ∂›Û˘, Ô EBV ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ HD, ·ÊÔ‡ ÌÂϤÙ˜ ·¤‰ÂÈÍ·Ó ÙËÓ ·ÚÔ˘Û›· ÙÔ˘ ÁÂÓÒÌ·ÙÔ˜ ÙÔ˘ EBV ÛÙ· ·ÙÙ·Ú· ReedSternberg Ì ÌÔÚȷΤ˜ Ù¯ÓÈΤ˜ (PCR), Û ÔÛÔÛÙfi Ô˘ ·Ó¤Ú¯ÂÙ·È ÛÙÔ 60% ÙˆÓ ÈÛÙÔÏÔÁÈÎÒÓ ·Ú·Û΢·ÛÌ¿ÙˆÓ ·È‰ÈÒÓ Ì HD.
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