Student Research Committee, School of Medicine, Islamic Azad University of Medical Sciences, Yazd, Iran.
Department of Hematology and Oncology, Islamic Azad University, Yazd Branch, Yazd, Iran.
J Med Case Rep. 2021 May 23;15(1):292. doi: 10.1186/s13256-021-02860-z.
Blastic plasmacytoid dendritic cell neoplasm represents a rare type of hematologic malignancy that often manifests itself through various skin lesions. It commonly affects the elderly male population. Lymph nodes, peripheral blood, and bone marrow involvement are the typical findings that justify its aggressive nature and dismal prognosis. On histopathological assessment, malignant cells share some similarities with blastic cells from the myeloid lineage that make immunohistochemistry staining mandatory for blastic plasmacytoid dendritic cell neoplasm diagnosis.
A 35-year-old Asian man presented with cervical lymphadenopathy followed by an erythematous lesion on his left upper back. At first, the lesion was misdiagnosed as an infectious disease and made the patient receive two ineffective courses of azithromycin and clarithromycin. Six months later, besides persistent skin manifestations, he felt a cervical mass, which was misdiagnosed as follicular center cell lymphoma. Tumor recurrence following the chemoradiation questioned the diagnosis, and further pathologic assessments confirmed blastic plasmacytoid dendritic cell neoplasm. The second recurrence occurred 3 months after chemotherapy. Eventually, he received a bone marrow transplant after complete remission. However, the patient expired 3 months after transplant owing to the third recurrence and gastrointestinal graft versus host disease complications.
Early clinical suspicion and true pathologic diagnosis play a crucial role in patients' prognosis. Moreover, allogenic bone marrow transplant should be performed with more caution in aggressive forms of blastic plasmacytoid dendritic cell neoplasm because of transplant side effects and high risk of cancer recurrence.
原始血液细胞瘤是一种罕见的血液恶性肿瘤，常以多种皮肤损伤为主要表现，常见于老年男性。其典型表现为淋巴结、外周血和骨髓累及，具有侵袭性和不良预后。在组织病理学评估中，恶性细胞与髓系来源的原始细胞具有一些相似性，因此免疫组织化学染色对于原始血液细胞瘤的诊断是必需的。
一名 35 岁的亚裔男性，最初表现为颈部淋巴结病，随后左背部出现红斑性皮损。起初，皮损被误诊为传染病，导致患者接受了两剂无效的阿奇霉素和克拉霉素治疗。6 个月后，除了持续的皮肤表现外，他还感到颈部肿块，被误诊为滤泡中心细胞淋巴瘤。化疗和放疗后的肿瘤复发使诊断受到质疑，进一步的病理评估证实为原始血液细胞瘤。化疗后 3 个月再次复发。最终，在完全缓解后，他接受了骨髓移植。然而，患者在移植后 3 个月因第三次复发和胃肠道移植物抗宿主病并发症去世。
早期临床怀疑和准确的病理诊断对患者的预后起着至关重要的作用。此外，由于移植副作用和癌症复发的高风险，对于侵袭性原始血液细胞瘤，应更加谨慎地进行同种异体骨髓移植。
