Abstract
This is a case report about a case of blastic plasmacytoid dendritic cell neoplasm (BPDCN), which relapsed twice and is currently in third remission. We describe the presentation and management of this case. We aim to illustrate the management of BPDCN, which is a rare disorder, and the development and characteristics for coping with changes in care, especially in a relapsed setting. This is a descriptive case report of a BPDCN from November 2017 until May 2021. Our facility is an academic referral center. Our patient is a married 37-year-old fisherman living in a fishing village in a nearby coastal district. The patient is a moderate cigarette smoker with no previous medical history. The patient presented to us in September 2017 with a history of having a scalp lesion for three months, with toxic symptoms in the form of night sweats and fever. A biopsy of the scalp lesion showed rounded cell tumor, positive LCA, negative CK and HMB 45, and CBC showing mild leukopenia. BMA showed hypercellular BM and 58% blasts, suggestive of AML. M5 for IPT and BMB hypercellular BM were suggestive of acute leukemia. On examination, he had bilateral bulky cervical LNs, approximately 3×3 cm, and bilateral parotid swellings with an unpalpable spleen. We repeated BMA, which showed hypercellular, 0 blasts, and was confirmed by BMB. Revision of the scalp lesion biopsy IHC was positive for LCA, CD4, CD56, and S100. The case was diagnosed as BPDCN. The patient started the hyperCVAD protocol in January 2018 with a good response and the disappearance of the scalp lesions. He was referred for HLA matching, but his siblings refused; therefore, he continued up to eight hyperCVAD courses and was prepared for the ninth in October 2018, when he was lost to follow-up. The patient presented five months later with a recurrent swelling in the left forearm, showing recurrence after medullary clearance. He started the HAM protocol in June 2019 and received a 2nd cycle, followed by four cycles of the HDA protocol in January 2020, after which PET/CT showed negative. In April 2020, he had a recurrent forearm lesion, showing recurrence. His PET/CT was negative, and he received steroids with local irradiation. OS of 44 months, DFS of 13, 9, and 9 months (31 months total). Treating BPDCN using chemotherapy only has a higher incidence of relapse.
Published Version
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