[Clinical characteristics and therapy outcome in children with stage IV Hodgkin's lymphoma--the experience of two oncological centres].
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.


METHODS
The analyzed group comprised of 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 2nd line chemotherapy Salvage 95. One boy with very poor response to the 1st and 2nd 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.


CONCLUSIONS
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.
Bień
Ewa
E
Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii, Akademia Medyczna, ul. Debinki 7, 80-211 Gdańsk, Poland. ebien@amg.gda.pl
Stachowicz-Stencel
Teresa
T
Zawitkowska-Klaczyńska
Joanna
J
Adamkiewicz-Drozyńska
Elzbieta
E
Odój
Teresa
T
Połczyńska
Katarzyna
K
Mitura-Lesiuk
Małgorzata
M
Stefanowicz
Joanna
J
Sierota
Danuta
D
Szołkiewicz
Anna
A
Birkholz
Dorota
D
Hennig
Marcin
M
Kowalczyk
Jerzy R
JR
Balcerska
Anna
A
D000046:Academic Medical Centers; D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D002648:Child; D002662:Child Health Services; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006689:Hodgkin Disease / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008297:Male; D009367:Neoplasm Staging; D011044:Poland; D018714:Radiotherapy, Adjuvant; D012008:Recurrence; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome