Division of Hematology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Cytometry B Clin Cytom. 2010;78 Suppl 1(Suppl 1):S35-41. doi: 10.1002/cyto.b.20546.
The pathology and clinical course of patients with CD5+ chronic B-cell lymphoproliferative disorders, excluding those that present with typical chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL) or mantle cell lymphoma, (i.e. CD5+B-CLPD) are poorly defined.
We studied patients with CD5+B-CLPD to (1) more completely define the clinical features and pathology of CD5+B-CLPD, (2) compare these features to patients presenting with typical CLL, and (3) test the hypothesis that a subset of patients with CD5+B-CLPD could have a unique B-cell malignancy.
We identified 229 patients with CD5+B-CLPD. A definitive pathological diagnosis was made in all 61 (27%) CD5+B-CLPD patients with nonbone marrow (BM) biopsy specimens considered adequate for a comprehensive pathological examination. The most common diagnosis among these 61 patients was CLL (44%) followed by the leukemic phase of marginal zone lymphoma (34%), lymphoplasmacytic lymphoma (11%), diffuse large B cell lymphoma (8%), and high-grade B cell lymphoma not otherwise specified (2%). In contrast, among 168 patients without a non-BM tissue biopsy specimen, a specific diagnosis could be made on review of all available data in only 24 (14%) with 144 (86%) remaining "unclassified."
In patients with CD5+B-CLPD, a definitive diagnosis can be made on an adequate non-BM tissue biopsy suggesting that this entity does not include a novel disease. We recommend that all patients with CD5+B-CLPD should have a non-BM tissue biopsy to make a definitive diagnosis prior to initiation of treatment.
除了那些表现为典型慢性淋巴细胞白血病/小淋巴细胞淋巴瘤（CLL）或套细胞淋巴瘤的患者外，CD5+慢性 B 细胞淋巴增生性疾病（即 CD5+B-CLPD）患者的病理学和临床过程定义不明确。
我们研究了 CD5+B-CLPD 患者，以（1）更完全地定义 CD5+B-CLPD 的临床特征和病理学，（2）将这些特征与表现为典型 CLL 的患者进行比较，以及（3）检验假设，即一部分 CD5+B-CLPD 患者可能具有独特的 B 细胞恶性肿瘤。
我们确定了 229 例 CD5+B-CLPD 患者。61 例（27%）CD5+B-CLPD 患者的非骨髓（BM）活检标本被认为足以进行全面的病理检查，在这些患者中均做出了明确的病理诊断。在这 61 例患者中，最常见的诊断是 CLL（44%），其次是边缘区淋巴瘤的白血病期（34%）、淋巴浆细胞淋巴瘤（11%）、弥漫性大 B 细胞淋巴瘤（8%）和未另指定的高级别 B 细胞淋巴瘤（2%）。相比之下，在 168 例无非 BM 组织活检标本的患者中，仅在 24 例（14%）患者中通过回顾所有可用数据可以做出明确诊断，而 144 例（86%）患者仍被“未分类”。
在 CD5+B-CLPD 患者中，通过充分的非 BM 组织活检可以做出明确诊断，这表明该实体不包括一种新疾病。我们建议所有 CD5+B-CLPD 患者在开始治疗之前应进行非 BM 组织活检以做出明确诊断。
