Mann Georg, Attarbaschi Andishe, Burkhardt Birgit, Niggli Felix, Klapper Wolfram, Ludwig Wolf-Dieter, Schrappe Martin, Zimmermann Martin, Gadner Helmut, Reiter Alfred
Department of Paediatric Haematology and Oncology, St. Anna Children's Hospital, Vienna, Austria.
Br J Haematol. 2007 Nov;139(3):443-9. doi: 10.1111/j.1365-2141.2007.06796.x. Epub 2007 Sep 14.
Non-Hodgkin lymphoma (NHL) is rarely observed during infancy and data on its incidence, characteristics and outcome are scarce. The present study aimed to assess the prevalence, clinical presentation and treatment results of all infants who were diagnosed with NHL between October 1986 and December 2002 among 2084 patients treated according to the NHL-BFM (Berlin-Frankfurt-Münster) multicentre trials 86, 90 and 95. We identified 20 (1%) infants with NHL including five with T-cell lymphoblastic lymphoma (T-cell LBL), seven with precursor B-cell LBL (pB-cell LBL), two with a mature Burkitt neoplasm, five with anaplastic large cell lymphoma (ALCL) and one with peripheral T-cell lymphoma (PTCL). The PTCL patient, 3/7 pB-cell LBL and 1/5 ALCL patients relapsed. One patient each from the T-cell LBL and Burkitt lymphoma groups suffered from a second malignancy and one patient each with ALCL and Burkitt leukaemia died from treatment-related toxicity. The 5-year event-free survival rate was 53 +/- 12% for the 20 cases. This study has provided preliminary evidence that infants with NHL have a dismal prognosis and showed that infant NHL differed to lymphomas in older patients with respect to the distribution of gender, histopathologic subtypes as well as the ratio of T- to pB-cell LBL and the frequency of relapses of pB-cell LBL.
非霍奇金淋巴瘤（NHL）在婴儿期很少见，关于其发病率、特征和转归的数据也很匮乏。本研究旨在评估1986年10月至2002年12月期间，在按照NHL-BFM（柏林-法兰克福-明斯特）多中心试验86、90和95治疗的2084例患者中，所有被诊断为NHL的婴儿的患病率、临床表现和治疗结果。我们确定了20例（1%）患有NHL的婴儿，其中5例为T细胞淋巴母细胞淋巴瘤（T细胞LBL），7例为前体B细胞LBL（pB细胞LBL），2例为成熟伯基特肿瘤，5例为间变性大细胞淋巴瘤（ALCL），1例为外周T细胞淋巴瘤（PTCL）。PTCL患者、3/7的pB细胞LBL患者和1/5的ALCL患者复发。T细胞LBL组和伯基特淋巴瘤组各有1例患者发生了第二种恶性肿瘤，ALCL组和伯基特白血病组各有1例患者死于治疗相关毒性。20例患者的5年无事件生存率为53±12%。本研究提供了初步证据，表明患有NHL的婴儿预后不佳，并表明婴儿NHL在性别分布、组织病理学亚型以及T细胞与pB细胞LBL的比例和pB细胞LBL的复发频率方面与老年患者的淋巴瘤有所不同。
