Severe tumor lysis syndrome during the induction therapy for the treatment of blastic plasmacytoid dendritic cell neoplasm arising from myelodysplastic/myeloproliferative neoplasms

Sagou, Ken; Itō, Makoto; Kawamura, Yuma; Ukai, Shun; Goto, Miyo; Fukushima, Norishige; Ozeki, Kazutaka; Fukuyama, Ryuichi; Kohno, Akio

doi:10.1002/ccr3.3690

Cited by 3 publications

(2 citation statements)

References 12 publications

(11 reference statements)

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“…In this patient, the lymphadenopathy caused superior vena cava syndrome. Similar cases of BPDCN with life-threatening TLS shortly after treatment with chemotherapy or tagraxofusp have been reported 15,16 . DIC has been reported in BPDCN patients, but mostly in elderly adults 17,18 .…”

Section: Discussionsupporting

confidence: 65%

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Frontline Hyper-CVAD Plus Venetoclax for Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm

He,

Garcia,

Connors

et al. 2023

Journal of Pediatric Hematology/Oncology

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy, especially in pediatrics, that can involve the bone marrow, skin, lymph nodes, and central nervous system (CNS). Given its variable clinical presentation, coupled with an immunohistochemistry pattern (CD4, CD56, TCF4, TCL-1, and CD123 positivity) that differs from other myeloid neoplasms, the diagnosis of BPDCN can be missed. Limited data are available to guide the treatment of pediatric BPDCN. Herein, we report a case of a pediatric patient who had BPDCN with central nervous system, orbital, and skin involvement. This patient achieved complete remission after receiving modified hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone with venetoclax and intrathecal chemotherapy. He remains disease-free 200 days after receiving a stem cell transplant. This represents the first known published pediatric case using a modified hyper-CVAD plus venetoclax regimen for treating a pediatric BPDCN patient in the frontline setting.

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Section: Discussionsupporting

confidence: 65%

“…Similar cases of BPDCN with life-threatening TLS shortly after treatment with chemotherapy or tagraxofusp have been reported. 15,16 DIC has been reported in BPDCN patients, but mostly in elderly adults. 17,18 These findings emphasize the importance of monitoring BPDCN patients for TLS and DIC.…”

Section: Discussionmentioning

confidence: 99%