母细胞性浆细胞样树突状细胞肿瘤(BPDCN)是一种侵袭性血液恶性肿瘤,罕见且预后不良。通常,它表现为皮肤浸润,然后传播,只有10%可能首先表现为骨髓(BM)浸润。在临床和病理上与急性髓系白血病(AML)和急性淋巴细胞白血病(ALL)具有重叠特征,有许多疾病需要与之鉴别诊断。治疗BPDCN没有共识,但是在该领域出现了一些新的疗法来治疗这类患者。47岁男性糖尿病病例报告,提交给曼苏拉大学肿瘤学中心血液科,前额附近前部的头皮肿块升高,皮肤和面部水肿上覆压力坏死,背部有类似的病变。颈部和腹股沟淋巴结肿大。皮肤活检显示单样中型囊样细胞浸润,染色质很好,细胞质很少,环状延伸,也没有检测到表皮性。免疫组织化学显示CD4,CD56和BCL2的弥漫性膜免疫反应性。Ki-67增殖指数约为90%。CD117,MPO,CD34,CD20,CD5,CD3,ALK,CD79a,CD30在肿瘤细胞中显示阴性免疫反应性。BM检查显示80%的大胚细胞浸润,具有嗜碱性细胞质和良好的染色质。多色流式细胞术(MCF)显示低CD45表达(位于爆炸袋)和阳性CD123,CD56谱(中度至阴性),小CD4、CD43、部分小CD33、CD38、CD81和部分CD36(主要为阴性),对小组的其他部分是负面的。患者接受Hyper-CVAD方案(环磷酰胺,硫酸长春新碱,盐酸多柔比星,地塞米松)和皮肤病变在第1周期化疗后显示出惊人的反应。BM和FDG-PET/CT评价无异常细胞浸润。现在,他正在完成化疗,并计划进行同种异体干细胞移植(SCT)。这类病例的诊断和处理从发病之初就在各个层面上都具有挑战性,通过诊断的步骤,最后在选择合适的治疗方案。我们建议基于ALL的化疗方案,尤其是Hyper-CVAD,然后是同种异体SCT,以获得更好的反应和总体生存率。
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematological malignancy with a rare incidence with poor outcome. Usually, it presents by skin infiltration then dissemination and only 10% may present first by bone marrow (BM) infiltration. It has overlapping features with acute myeloid leukemia (AML) and acute lymphoblastic leukemia (
ALL) clinically and pathologically, and there are many diseases to be differentially diagnosed from them. There is no consensus for treating BPDCN, but there are some novel therapies that have emerged in the field for treating this group of patients. A
case report of 47-year-old diabetic male, presented to the hematology unit of Oncology Center of Mansoura University with a large, elevated scalp mass on the anterior part near the forehead with overlying pressure necrosis of the skin and face edema and with similar lesions on the back. There were enlarged cervical and inguinal lymph nodes. Skin biopsy revealed infiltration by monomorphous medium-sized blastoid cells, with fine chromatin and scanty cytoplasm, pannicular extension, and no detected epidermotropism. Immunohistochemistry revealed diffuse membranous immunoreactivity for CD4, CD56, and BCL2. Ki-67 proliferation index was about 90%. CD117, MPO, CD34, CD20, CD5, CD3, ALK, CD79a, and CD30 showed negative immunoreactivity in the tumor cells. BM examination showed infiltration by 80% large blastoid cells with basophilic cytoplasm and fine chromatin. Multi-color-flowcytometry (MCF) revealed low CD45 expression (located in the blast pocket) and positive CD123, a spectrum of CD56 (moderate to negative), dim CD4, CD43, partial dim CD33, CD38, CD81, and partial CD36 (predominantly negative), and was negative for the rest of the panel. The patient received Hyper-CVAD protocol (cyclophosphamide, vincristine sulfate, doxorubicin hydrochloride, and dexamethasone) and skin lesions showed marvelous response after 1st cycle of chemotherapy. BM and FDG -PET/CT evaluation was free from abnormal cell infiltration. Now he is completing his chemotherapy and planned for allogenic stem cell transplantation (SCT). The diagnosis and management of such cases are challenging at
all levels from the beginning of the disease, through the steps of diagnosis and finally in choosing the appropriate treatment protocol. We recommend
ALL-based chemotherapy protocols especially Hyper-CVAD then allogenic SCT for better response and overall survival.