Abstract：
BACKGROUND: Splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN) aka hairy cell leukemia variant (HCL-v) is a rare B-cell chronic lymphoproliferative disorder. The main diagnostic challenge is to differentiate SBLPN from Classical hairy cell leukemia (HCL-c), as the former faces inferior responses to therapies and a poor prognosis.
OBJECTIVE: The aim is to discuss the clinic-hematological and immunophenotyping findings of three cases of SBLPN.
METHODS: This is a retrospective observational study.
METHODS: From the year 2011 to 2021, flow cytometry of all the cases with HCL diagnosis was reviewed, and three cases with negative or dim CD25 and hematological presentation matching with SBLPN were picked up.
METHODS: Descriptive statistics is used.
RESULTS: All the cases were male. The age ranges from 43 to 64 years. Median hemoglobin concentration, total leucocyte count, and platelet count were 8.6 g/dL, 6.9 × 109/L, and 53 × 109/L, respectively. The atypical cells were medium to large. All three showed prominent nucleoli. Bone marrow biopsies showed an interstitial pattern of infiltration in all the cases. The hairy cells were positive for CD20, CD11c, and CD103. CD25 was dim positive in one case. Annexin A1 was negative in all three cases. BRAF V600E mutation analysis was done in one case and turned out negative for the mutation.
CONCLUSIONS: SBLPN is a rare entity, usually on-flow cytometry CD25 negative. However, in dim CD25-positive cases, BRAFV600E mutational analysis helps in discerning SBLPN diagnosis and differentiating it from HCL-c.
OBJECTIVE: The aim is to discuss the clinic-hematological and immunophenotyping findings of three cases of SBLPN.
METHODS: This is a retrospective observational study.
METHODS: From the year 2011 to 2021, flow cytometry of all the cases with HCL diagnosis was reviewed, and three cases with negative or dim CD25 and hematological presentation matching with SBLPN were picked up.
METHODS: Descriptive statistics is used.
RESULTS: All the cases were male. The age ranges from 43 to 64 years. Median hemoglobin concentration, total leucocyte count, and platelet count were 8.6 g/dL, 6.9 × 109/L, and 53 × 109/L, respectively. The atypical cells were medium to large. All three showed prominent nucleoli. Bone marrow biopsies showed an interstitial pattern of infiltration in all the cases. The hairy cells were positive for CD20, CD11c, and CD103. CD25 was dim positive in one case. Annexin A1 was negative in all three cases. BRAF V600E mutation analysis was done in one case and turned out negative for the mutation.
CONCLUSIONS: SBLPN is a rare entity, usually on-flow cytometry CD25 negative. However, in dim CD25-positive cases, BRAFV600E mutational analysis helps in discerning SBLPN diagnosis and differentiating it from HCL-c.
摘要：
背景：脾B细胞淋巴瘤/具有突出核仁的白血病（SBLPN），即毛细胞白血病变体（HCL-v）是一种罕见的B细胞慢性淋巴增殖性疾病。主要的诊断挑战是区分SBLPN与经典毛细胞白血病（HCL-c），因为前者对治疗的反应较差，预后较差。
目的：目的探讨3例SBLPN的临床血液学和免疫表型。
方法：这是一项回顾性观察性研究。
方法：从2011年到2021年，对所有诊断为HCL的病例进行了流式细胞术，收集3例CD25阴性或暗淡且血液学表现与SBLPN匹配的病例。
方法：使用描述性统计。
结果：所有病例均为男性。年龄从43岁到64岁不等。血红蛋白浓度中位数，白细胞总数,血小板计数为8.6g/dL，6.9×109/L,53×109/L，分别。非典型细胞为中等至大。所有三个都显示出突出的核仁。在所有病例中，骨髓活检均显示间质浸润。毛细胞CD20、CD11c、CD103CD25在一例中呈暗阳性。膜联蛋白A1在所有三个病例中均为阴性。在一个病例中进行了BRAFV600E突变分析，结果为突变阴性。
结论：SBLPN是一种罕见的实体，通常在流式细胞术CD25阴性。然而,在昏暗的CD25阳性病例中，BRAFV600E突变分析有助于辨别SBLPN诊断并将其与HCL-c区分开。
目的：目的探讨3例SBLPN的临床血液学和免疫表型。
方法：这是一项回顾性观察性研究。
方法：从2011年到2021年，对所有诊断为HCL的病例进行了流式细胞术，收集3例CD25阴性或暗淡且血液学表现与SBLPN匹配的病例。
方法：使用描述性统计。
结果：所有病例均为男性。年龄从43岁到64岁不等。血红蛋白浓度中位数，白细胞总数,血小板计数为8.6g/dL，6.9×109/L,53×109/L，分别。非典型细胞为中等至大。所有三个都显示出突出的核仁。在所有病例中，骨髓活检均显示间质浸润。毛细胞CD20、CD11c、CD103CD25在一例中呈暗阳性。膜联蛋白A1在所有三个病例中均为阴性。在一个病例中进行了BRAFV600E突变分析，结果为突变阴性。
结论：SBLPN是一种罕见的实体，通常在流式细胞术CD25阴性。然而,在昏暗的CD25阳性病例中，BRAFV600E突变分析有助于辨别SBLPN诊断并将其与HCL-c区分开。
