Medycyna Wieku Rozwojowego

632
Ewa Bieƒ i wsp.
si´cy po transplantacji komórek krwiotwórczych z powodu drugiego nowotworu – ostrej bia-
∏aczki nielimfoblastycznej.
Wnioski: skojarzona chemio-i radioterapia stosowana wed∏ug protoko∏ów leczniczych przyj´tych
przez PPGBCh u dzieci z chorobà Hodgkina nawet w IV stadium zaawansowania zapewnia
osiàgni´cie ca∏kowitej klinicznej remisji i trwa∏ego wyleczenia u wi´kszoÊci pacjentów.
Wykrycie choroby we wczeÊniejszych stadiach umo˝liwi∏oby zastosowanie mniej agresywnej
terapii, a tym samym obni˝y∏oby ryzyko wystàpienia póênych powik∏aƒ.
CLINICAL CHARACTERISTICS AND THERAPY OUTCOME
IN CHILDREN WITH STAGE IV HODGKIN’S LYMPHOMA
– THE EXPERIENCE OF TWO ONCOLOGICAL CENTRES
Abstract. The cure rate in children with Hodgkin’s disease (HD), at present time exceeds
90% but the prognosis in stage IV HD is much worse
The aim of the study was to analyze the initial symptoms, course and results of oncological
therapy in children with stage IV of Hodgkin’s disease.
Material and methods: the analyzed group comprised 15 patients with IV stage HD (M/F:
11/4, mean age: 12 years), treated from January 1993 to March 2005, in two Polish centres
of paediatric oncology in Gdansk and Lublin. The diagnosis and therapy were carried
out according to the current protocols approved by the Polish Paediatric Leukaemia/Lymphoma
Study Group (PPGBCh).
Results: mean duration of initial symptoms was 4,5 months, with most children presenting
general symptoms of HD. At diagnosis, the involvement of mediastinal and/or hilar lymph
nodes was found in nine patients, lung infiltrations in six, involvement of the spleen, liver
and bones in five, three and one patient, respectively. The nodular sclerosis histopathological
type of HD predominated.
Poor response to standard treatment was observed in five children. One patient received additional
cycles of chemotherapy MVPP/B-DOPA, four children were administered the II nd
line chemotherapy Salvage 95. One boy with very poor response to the I st and II nd therapy
lines additionally underwent megachemotherapy with peripheral blood stem cells transplantation.
Radiotherapy was given to 13 children.
13 out of 15 children are alive and free of disease with mean follow-up duration of 6 years.
In two of them late complications affecting hormonal status, cardio-pulmonary disorders
and chronic B and C hepatitis were observed. Two children died including one admitted in
a very severe condition, after long-lasting medical history who died of neutropenia-related
sepsis. The second boy died 12 months after stem cell transplantation because of a second
neoplasm – acute myeloblastic leukaemia.
Conclusion: chemo-and radiotherapy implemented according to protocols approved by the
PPGBCh for children with stage IV HD, result in complete remission in most patients. Diagnosis
made at earlier stages would result in giving less aggressive therapy, connected with
a lower risk of durable late complications.
WPROWADZENIE
Choroba Hodgkina (HD) u dzieci jest schorzeniem rozrostowym o pomyÊlnym
rokowaniu z ponad 90% prawdopodobieƒstwem trwa∏ego wyleczenia. Gorsze wyniki
terapii uzyskuje si´ w grupie pacjentów z chorobà rozsianà, czyli w IV stadium zaawansowania
(1, 2).