دورية أكاديمية

[Clinical Characteristics of CD4 - CD56 + Blastic Plasmacytoid Dendritic Cell Neoplasm].

التفاصيل البيبلوغرافية
العنوان: [Clinical Characteristics of CD4 - CD56 + Blastic Plasmacytoid Dendritic Cell Neoplasm].
المؤلفون: He HS; Department of Hematology, The First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui Province, China.E-mail: hhsmed2012@126.com., Wei YF; Department of Hematology, The First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui Province, China., Ji XY; Department of Hematology, The First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui Province, China., Xu YH; Department of Hematology, The First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui Province, China., Yang YQ; Department of Hematology, The First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui Province, China., Jin XK; Department of Hematology, The First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui Province, China.
المصدر: Zhongguo shi yan xue ye xue za zhi [Zhongguo Shi Yan Xue Ye Xue Za Zhi] 2024 Apr; Vol. 32 (2), pp. 588-594.
نوع المنشور: Case Reports; English Abstract; Journal Article
اللغة: Chinese
بيانات الدورية: Publisher: Zhongguo shi yan xue za zhi she Country of Publication: China NLM ID: 101084424 Publication Model: Print Cited Medium: Print ISSN: 1009-2137 (Print) Linking ISSN: 10092137 NLM ISO Abbreviation: Zhongguo Shi Yan Xue Ye Xue Za Zhi Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Beijing : Zhongguo shi yan xue za zhi she,
مواضيع طبية MeSH: CD56 Antigen*/metabolism , Dendritic Cells*, Aged ; Humans ; Male ; CD4 Antigens/metabolism ; Hematologic Neoplasms ; Immunophenotyping ; Prognosis ; Retrospective Studies
مستخلص: Objective: To explore the clinical manifestations, pathological features, immunophenotype, as well as diagnosis, treatment and prognosis of patients with CD4 - CD56 + blastic plasmacytoid dendritic cell neoplasm (BPDCN), in order to further understand the rare disease.
Methods: The clinical data, laboratory examinations and treatment regimens of two patients with CD4 - CD56 + BPDCN in the First Affiliated Hospital of Wannan Medical College were retrospectively analyzed.
Results: The two patients were both elderly males with tumor involved in skin, bone marrow, lymph nodes, etc. Immunohistochemical results of skin lesions showed that both CD56 and CD123 were positive, while CD4, CD34, TdT, CD3, CD20, MPO and EBER were negative. Flow cytometry of bone marrow demonstrated that CD56, CD123, and CD304 were all positive, while specific immune markers of myeloid and lymphoid were negative. Two patients were initially very sensitive to acute lymphoblastic leukemia or lymphomatoid chemotherapy regimens, but prone to rapid relapse. The overall survival of both patients was 36 months and 4 months, respectively.
Conclusion: CD4 - CD56 + BPDCN is very rare and easily misdiagnosed as other hematological tumors with poor prognosis. Acute lymphoblastic leukemia or lymphomatoid therapy should be used first to improve the poor prognosis.
فهرسة مساهمة: Keywords: blastic plasmacytoid dendritic cell neoplasm; diagnosis; treatment; clinicopathology
Local Abstract: [Publisher, Chinese] CD4 - CD56 + 母细胞性浆细胞样树突细胞肿瘤的临床特征. [Publisher, Chinese] 探讨CD4 - CD56 + 母细胞性浆细胞样树突细胞肿瘤患者的临床表现、病理特点、免疫表型、诊治经过及预后,以提高对此类罕见疾病的认识。. [Publisher, Chinese] 对本院就诊的两例CD4 - CD56 + 母细胞性浆细胞样树突细胞肿瘤患者的临床资料、实验室资料、治疗方案进行回顾性分析。. [Publisher, Chinese] 两例患者均为老年男性,肿瘤累及皮肤、骨髓、淋巴结等部位,皮肤病理显示,CD56、CD123均为阳性,而CD4、CD34、TdT、CD3、CD20、MPO、EBER均为阴性。骨髓流式细胞检测结果显示,CD56、CD123、CD304均为阳性,而髓系、淋系特异性免疫标志均为阴性。两例患者初始对急性淋巴细胞白血病或淋巴瘤样化疗方案非常敏感,但短期内复发,生存期分别为36、4个月。. [Publisher, Chinese] CD4 - CD56 + 母细胞性浆细胞样树突细胞肿瘤罕见,该病容易误诊且预后差,应优先采用急性淋巴细胞白血病或淋巴瘤样的治疗方案改善不良预后。.
تواريخ الأحداث: Date Created: 20240425 Date Completed: 20240425 Latest Revision: 20240517
رمز التحديث: 20240517
DOI: 10.19746/j.cnki.issn.1009-2137.2024.02.040
PMID: 38660871
قاعدة البيانات: MEDLINE
الوصف
تدمد:1009-2137
DOI:10.19746/j.cnki.issn.1009-2137.2024.02.040