Al-Alwan Ahmad, Khalid Farhan, Vyas Charmee, Sirpal Vishakha, Bader Husam
Department of Internal Medicine, Monmouth Medical Center, Long Branch, NJ, USA.
J Community Hosp Intern Med Perspect. 2023 Jun 29;13(4):79-81. doi: 10.55729/2000-9666.1208. eCollection 2023.
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy derived from type 2 Dendritic cells (immature Plasmacytoid dendritic cells). It is an aggressive lymphoma and most commonly presents as nonpruritic cutaneous lesions. It can also involve the bone marrow, lymph nodes, or circulating peripheral blasts. Here we present a 61-year-old female with skin bruises all over her body for the last three months associated with fatigue, night sweats, and unintentional weight loss. Her initial diagnosis was Acute Myeloid Leukemia (AML), but later she was diagnosed with BPDCN on tumor biopsy consistent with CD56+ neoplasm. The patient was treated with cyclophosphamide with steroid bridge. She was follow-up every week for the disseminated intravascular coagulation panel and monitored for tumor lysis syndrome. The management of the BPDCN is still unclear due to the condition's rarity. tagraxofusp has been used for remission induction as it has a higher response rate with an acceptable toxicity profile than conventional chemotherapy. Allogeneic hematopoietic stem cell transplantation (HCT) is recommended in patients with the first remission. For patients with relapsed/refractory disease, tagraxofusp demonstrates a good overall response, followed by HCT.
母细胞性浆细胞样树突状细胞肿瘤（BPDCN）是一种罕见的血液系统恶性肿瘤，起源于2型树突状细胞（未成熟浆细胞样树突状细胞）。它是一种侵袭性淋巴瘤，最常见的表现为无瘙痒性皮肤病变。它也可累及骨髓、淋巴结或循环外周母细胞。在此，我们报告一名61岁女性，在过去三个月全身出现皮肤瘀斑，伴有疲劳、盗汗和不明原因的体重减轻。她最初被诊断为急性髓系白血病（AML），但后来经肿瘤活检确诊为BPDCN，符合CD56+肿瘤。该患者接受了环磷酰胺联合类固醇桥接治疗。每周对她进行弥散性血管内凝血检查，并监测肿瘤溶解综合征。由于该病罕见，BPDCN的治疗仍不明确。塔格拉索夫单抗已被用于诱导缓解，因为它比传统化疗具有更高的缓解率和可接受的毒性特征。首次缓解的患者建议进行异基因造血干细胞移植（HCT）。对于复发/难治性疾病患者，塔格拉索夫单抗显示出良好的总体缓解率，随后进行HCT。
