Successful Treatment of Hodgkin Lymphoma in Nijmegen Breakage Syndrome

被引：7

作者：

Jovanovic, Ankica ^{[1
,2
,3
,4
]}

Minic, Predrag ^{[1
,2
,3
,4
]}

Scekic-Guc, Marija ^{[1
,2
,3
,4
]}

Djuricic, Slavisa ^{[1
,2
,3
,4
]}

Cirkovic, Sanja ^{[1
,2
,3
,4
]}

Weemaes, Corry ^{[5
]}

Pasic, Srdjan ^{[1
,2
,3
,4
]}

机构：

[1] Mother & Child Hlth Inst Serbia, Dept Pediat Hematol, Belgrade 11070, Serbia

[2] Mother & Child Hlth Inst Serbia, Dept Pulm, Belgrade 11070, Serbia

[3] Mother & Child Hlth Inst Serbia, Dept Med Genet, Belgrade 11070, Serbia

[4] Mother & Child Hlth Inst Serbia, Dept Pathol & Immunol, Belgrade 11070, Serbia

[5] Univ Nijmegen Med Ctr, Dept Pediat Immunol, Nijmegen, Netherlands

来源：

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY | 2009年 / 31卷 / 01期

关键词：

Nijmegen breakage syndrome; lymphoid malignancy; Hodgkin disease; CANCER INCIDENCE; DISEASE; ABNORMALITIES; CHILDREN; CHILDHOOD;

D O I：

10.1097/MPH.0b013e318190d72c

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Nijmegen breakage syndrome (NBS) is a rare DNA repair disorder characterized by microcephaly. immunodeficiency. and predisposition to malignancy. We report on a 5-year-old patient with NBS who presented with nodural sclerosing type of Hodgkin disease stage IVB. Chemotherapy consisting of COPP/ ABV regimen with reduction at 75% of full doses was employed. During this treatment. no major toxic or infectious complications were observed. Complete remission was achieved lusting now for 20 months. In DNA repair disorders. prognosis of Hodgkin disease is poor as opposed to excellent overall prognosis in general pediatric population. Better survival may be achieved both with adopted, disease-specific regimens. and individualized approach considering patient's clinical condition. Also better recognition and treatment of infections during chemotherapy may reduce early deaths in patients with DNA repair disorders.

引用

页码：49 / 52

页数：4

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