Akhtar Saad, Rauf M Shahzad, Al-Kofide Amani, Elshenawy Mahmoud A, Mushtaq Ali Hassan, Maghfoor Irfan
King Faisal Specialist Hospital and Research Center, Oncology Center, P.O. Box 3354, MBC# 64, Riyadh, 11211, Kingdom of Saudi Arabia.
Department of Pediatric Hematology/Oncology, King Faisal Specialist Hospital and Research Center, Riyadh, 11211, Kingdom of Saudi Arabia.
Hered Cancer Clin Pract. 2021 Feb 9;19(1):17. doi: 10.1186/s13053-021-00175-0.
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.
淋巴和/或血液系统恶性肿瘤的家族聚集现象（FHM）为研究相关致病基因提供了契机。关于淋巴和血液系统恶性肿瘤患者的数据有限。
利用淋巴瘤数据库识别1998年至2019年在我们机构就诊的结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）患者。我们通过收集家族中任何恶性肿瘤的详细病史（FM）来研究FHM。
共识别出200例NLPHL患者。由于30例患者未接听电话（22例）或已死亡[1例]，无法取得联系。对170/200例患者进行了访谈；代表167个家庭（3例患者有家族成员患NLPHL）。这170例患者提供了8225名家庭成员的信息。这167个家庭共有329名家庭成员患334种恶性肿瘤（包括167例NLPHL患者，5名成员各患2种恶性肿瘤）。在这167例患者中，77例（46.1%）无家族恶性肿瘤病史，而90例（53.9%）患者有家族恶性肿瘤病史阳性；162名家庭成员患167种恶性肿瘤。在这167个家庭中，31个家庭（18.6%）有成员患FHM并伴有或不伴有实体癌。这31个家庭有35名家庭成员（25名男性：10名女性）患16种淋巴瘤：弥漫性大B细胞淋巴瘤[2例]、滤泡中心细胞淋巴瘤[3例]、慢性淋巴细胞白血病/小淋巴细胞淋巴瘤[3例]、非霍奇金淋巴瘤[2例]、经典型HL[2例]和NLPHL[4例]。共有8例白血病：急性淋巴细胞白血病[4例]、急性髓系白血病[3例]和白血病 - 未分型[5例]。这35名FHM成员是31例NLPHL患者的一级[6例]、二级（16例）和三级[7例]亲属。家庭成员中有4个家庭患NLPHL；所有这8例NLPHL患者均为男性且存活。一级+二级+三级成员的总数中位数为81。在13/17例患者中观察到从第一代到第二代诊断年龄的下降（遗传早现）；第二代诊断时的年龄中位数为29.7岁，而第一代年龄为53岁（患恶性肿瘤提前23.3年）。
FHM在NLPHL中很常见。本研究在访谈技巧、时间、资源分配和基因检测方面为我们规划未来研究提供了许多重要见解。
