Blastic Transformation after Splenectomy in a Patient with Nonvillous Splenic Marginal Zone Lymphoma with p53 Overexpression: A Case Report
Blastic Transformation after Splenectomy in a Patient with Nonvillous Splenic Marginal Zone Lymphoma with p53 Overexpression: A Case Report
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p53 过度表达的非绒毛状脾边缘区淋巴瘤患者脾切除后的母细胞转化：病例报告
DOI：
10.1532/ijh97.05017
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发表时间：
2005
影响因子：
2.1
通讯作者：
Y. Tokusashi
中科院分区：
文献类型：
--
作者：
Y. Kakinoki;Hiroya Kubota;H. Sakurai;Teiko Sato;Y. Tokusashi
患者男，61岁，无自觉症状，因半年前体检时提示贫血（血红蛋白，9.5 g/dL）、血小板减少（血小板，9.2 × 104/μL）原因入院。骨髓检查显示73%的淋巴细胞。免疫表型为CD 19 + CD 20 + CD 3-CD 5-CD 10-CD 23-，荧光激活细胞分选分析轻链限制性酶（κ）阳性。计算机断层扫描显示轻度脾肿大。为了从组织学上确认诊断，我们进行了脾切除术。最后，我们诊断病人的疾病为非绒毛脾边缘区淋巴瘤（SMZL）。脾切除后1个月，白色血细胞计数显著增加，达7 × 104/μL，淋巴细胞发生急变。我们首先用氟达拉滨治疗患者，然后用CHOP方案（环磷酰胺，羟基柔红霉素，长春新碱[Oncovin]和泼尼松）治疗，但这种疾病是如此难治，以至于患者在脾切除术后13个月死于这种疾病。免疫组织化学染色和分子检测p53进行了标本从脾切除术。我们发现p53蛋白在淋巴细胞中的过度表达和第8外显子280密码子的点错义突变，将阿加（Arg）变为AGT（Ser）。这种情况下，可能表明存在一个更积极的子集SMZL，提示脾切除术和p53过表达的诊断和治疗方法与SMZL患者的作用重新考虑。
A 61-year-old man with no subjective symptom was admitted to our hospital for further examination of the causes of anemia (hemoglobin, 9.5 g/dL) and thrombocytopenia (platelets, 9.2 × 104/μL), which had been pointed out in a medical checkup half a year previously. A bone marrow examination showed 73% lymphoid cells. Immunophenotyping of these cells were CD19+CD20+CD3-CD5-CD10-CD23-, and light chain restriction (κ) was positive by fluorescence-activated cell sorting analysis. A computed tomography scan showed mild splenomegaly. To confirm the diagnosis histologically, we performed a splenectomy. Finally, we diagnosed the patient’s disease as nonvillous splenic marginal zone lymphoma (SMZL). A month after the splenectomy, the white blood cell count was remarkably increased to 7 × 104/μL with the blastic transformation of lymphoid cells. We first treated the patient with fludarabine and then with the CHOP regimen (cyclophosphamide, hydroxy-daunomycin, vincristine [Oncovin], and prednisone), but the disease was so refractory that the patient died of the disease 13 months after the splenectomy. Immunohistochemical staining and a molecular examination for p53 were carried out with specimens from the splenectomy. We found overexpression of the p53 protein in lymphoid cells and a point missense mutation in codon 280 at exon 8 that changed AGA (Arg) to AGT (Ser). This case may indicate the existence of a more aggressive subset of SMZL, suggesting a reconsideration of the roles of splenectomy and p53 overexpression in the diagnostic and therapeutic approaches to patients with SMZL.