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Risk of hematological malignancies in the families of patients treated for nodular lymphocyte-predominant Hodgkin lymphoma
Hereditary Cancer in Clinical Practice ( IF 2.0 ) Pub Date : 2021-02-09 , DOI: 10.1186/s13053-021-00175-0
Saad Akhtar , M. Shahzad Rauf , Amani Al-Kofide , Mahmoud A. Elshenawy , Ali Hassan Mushtaq , Irfan Maghfoor

Familial clustering of lymphoid and/or hematological malignancies (FHM) provides an opportunity to study the responsible genes. The data is limited in patients with lymphoid and hematological malignancies. The lymphoma database was used to identify patients seen in our institution from 1998 to 2019 with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). We studied FHM by collecting detailed history of any malignancy in the family (FM). Two hundred NLPHL patients were identified. Contacting was not possible in 30 patients due to no response to the phone calls (22) and death [1]. 170/200 patients were interviewed; represented 167 families (3 patients with a family member with NLPHL). These 170 patients provided information about 8225 family members. These 167 families had a total of 329 family members with 334 malignancies (including 167 NLPHL patients and 5 members with 2 malignancies each). Of these 167 patients, 77 (46.1%) had no FM while 90 (53.9%) patients had a positive FM; 162 family members with 167 malignancies. Among these 167 families, 31 families (18.6%) had members with FHM +/− solid cancers. These 31 families had 35 family members (25 males:10 females) with 16 lymphomas: diffuse large B cell lymphoma [2], follicular center cell lymphoma [3], chronic lymphocytic leukemia/small lymphocytic lymphoma [3], non-Hodgkin lymphoma [2], classical HL [2], and NLPHL [4]. Total of 8 leukemia: acute lymphoblastic leukemia [4], acute myeloid leukemia [3], and leukemia - no subtyping [5]. These 35 FHM members are 1st [6], 2nd (16), and 3rd [7] degree relatives of 31 NLPHL patients. There are 4 families with NLPHL in family members; all these 8 NLPHL patients are male and are alive. The median total number of 1st + 2nd +3rd degree members are 81. The decrease in the age of diagnosis from 1st generation to the 2nd generation (anticipation) was noted in 13/17 patients; 2nd generation median age at diagnosis was 29.7 years vs 1st generation age 53 years (developed malignancy 23.3 years earlier). FHM is frequent in NLPHL. This study provided us many important insights for planning future studies in terms of interviewing technique, time, and resource allocation and genetic testing.

中文翻译:

结节性淋巴细胞为主的霍奇金淋巴瘤治疗的患者家庭中血液系统恶性肿瘤的风险

家族性淋巴和/或血液恶性肿瘤(FHM)的聚类为研究相关基因提供了机会。淋巴和血液系统恶性肿瘤患者的数据有限。淋巴瘤数据库用于鉴定1998年至2019年在我们机构见过的结节性淋巴细胞为主的霍奇金淋巴瘤(NLPHL)的患者。我们通过收集家族中任何恶性肿瘤(FM)的详细历史来研究FHM。确定了200名NLPHL患者。30名患者由于无法回复电话(22)和死亡[1]而无法联系。采访了170/200名患者;代表167个家庭(3例家庭成员患有NLPHL)。这170名患者提供了有关8225个家庭成员的信息。这167个家庭共有329个家庭成员,患有334例恶性肿瘤(包括167名NLPHL患者和5个成员,每个成员具有2个恶性肿瘤)。在这167名患者中,有77名(46.1%)没有FM,而90名(53.9%)则有FM阳性。162位家庭成员,患有167例恶性肿瘤。在这167个家族中,有31个家族(18.6%)的成员患有FHM +/-实体癌。这31个家庭有35个家庭成员(男25例,女10例),其中16例淋巴瘤:弥漫性大B细胞淋巴瘤[2],滤泡中心细胞淋巴瘤[3],慢性淋巴细胞白血病/小淋巴细胞淋巴瘤[3],非霍奇金淋巴瘤[2],经典HL [2]和NLPHL [4]。共有8种白血病:急性淋巴细胞白血病[4],急性髓细胞性白血病[3]和白血病-无亚型[5]。这35名FHM成员是31名NLPHL患者的1级[6],2级(16)和3级[7]亲戚。有4个家庭的NLPHL家庭成员;所有这8位NLPHL患者均为男性,并且还活着。1st + 2nd + 3rd成员的中位数总数为81。在13/17例患者中,发现从1代到2代(预期)的诊断年龄减少;诊断的第二代中位年龄为29.7岁,而第一代年龄为53岁(较23.3年发展为恶性肿瘤)。在NLPHL中,FHM很常见。这项研究为我们在访谈技术,时间,资源分配和基因测试方面计划未来的研究提供了许多重要的见识。从第1代到第2代(预期)的诊断年龄降低了,发现有13/17例患者。诊断的第二代中位年龄为29.7岁,而第一代年龄为53岁(较23.3年发展为恶性肿瘤)。在NLPHL中,FHM很常见。这项研究为我们在访谈技术,时间,资源分配和基因测试方面计划未来的研究提供了许多重要的见识。从第1代到第2代(预期)的诊断年龄降低了,发现有13/17例患者。诊断的第二代中位年龄为29.7岁,而第一代年龄为53岁(较23.3年发展为恶性肿瘤)。在NLPHL中,FHM很常见。这项研究为我们在访谈技术,时间,资源分配和基因测试方面计划未来的研究提供了许多重要的见识。
更新日期:2021-02-09
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