结节型淋巴细胞性霍奇金淋巴瘤（NLPHL），最近也被称为结节型淋巴细胞为主的B细胞淋巴瘤（NLPBL），占霍奇金淋巴瘤（HL）的5%-10%。NLPBL存在不同的形态学类型，可分为典型型（A型和B型）和变异组织学类型（C型、D型、E型和F型）。我们在埃及儿童癌症医院对诊断为NLPBL的儿童患者进行了不同形态学类型、CD30和IgD表达的研究。其中46名患者（53%）表现为典型组织学类型，而其余患者（47%）表现为变异组织学类型。变异组织学类型与不良的临床特征相关，如晚期疾病阶段、B症状和淋巴结外受累，尤其是骨髓和骨浸润，p值分别为0.06、0.05和0.01%。此外，39%的变异组织学类型患者出现了疾病进展或复发，而仅有15.2%的典型型患者出现了同样情况（p = 0.009）。C型和D型与事件无进展生存（EFS）降低相关，其p值为0.05。变异组织学类型患者采用利妥昔单抗、环磷酰胺、长春碱、阿霉素和泼尼松（RCHOP）方案的5年EFS为94.4%，而使用阿霉素、博莱霉素、长春碱和达卡巴嗪（ABVD）方案的5年EFS为33.3%。44名患者（50%）的淋巴细胞性为主（LP）细胞表达了IgD，39名患者（44%）的LP细胞表达了CD30。NLPBL的变异组织学类型与晚期疾病分期和不良预后相关，而LP细胞中的IgD和CD30表达与此无关。变异组织学类型患者采用RCHOP方案不良预后有所改善。© 2023 Wiley Periodicals LLC.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), recently known as nodular lymphocyte-predominant B-cell lymphoma (NLPBL), accounts for 5%-10% of Hodgkin lymphoma (HL). Different morphologic patterns of NLPBL are identified and categorized as typical patterns (type A and B) and variant histologic patterns (types C, D, E, and F).We investigated different morphologic patterns, CD30 and IgD expression in pediatric patients with NLPBL diagnosed at the Children's Cancer Hospital Egypt.Forty-six (53%) of the patients exhibited a typical histologic pattern, whereas the remaining (47%) exhibited variant histologic pattern. Variant histology is associated with unfavorable clinical characteristics, such as advanced stages, B-symptoms, and extranodal involvements, particularly bone marrow and bone infiltration, with p-values of .06, .05, and 0.01%, respectively. Additionally, 39% of patients with variant histology experienced disease progression or relapse, compared to only 15.2% of patients with typical patterns (p = .009). Types C and D are related to decreased event-free survival (EFS), as shown by a p-value of .05. The 5-year EFS for patients with variant histology was 94.4% for the rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisone (RCHOP) versus 33.3% for the adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD). IgD expression in lymphocyte-predominant (LP) cells was detected in 44 (50%) patients, while CD30 expression in LP cells was found in 39 (44%) patients.Variant histology of NLPBL was associated with advanced disease stages and a poor prognosis, while expression of IgD and CD30 in LP cells was not. The poor outcome of variant histology improved with the RCHOP regimen.© 2023 Wiley Periodicals LLC.