Quick complete response achievement with venetoclax and azacitidine in a case of relapsed disseminated blastic plasmacytoid dendritic cell neoplasm

Piccini, Matteo; Loscocco, Giuseppe Gaetano; Gianfaldoni, Giacomo; Grieco, Pietro; Palmieri, Giuseppina; Pilerci, Sofia; Scappini, Barbara; Bosi, Alberto

doi:10.1007/s00277-020-03939-1

Cited by 9 publications

(9 citation statements)

References 6 publications

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“…In another case report by Beziat et al , the patient did not exhibit a very good response, as some cutaneous lesions remained stable while others responded [ 11 ]. Then, two other papers in combination therapy with hypometilating agents report pretty good results, with rapid and durable responses [ 12 , 13 ]. A summary of cases in which venetoclax was used, including ours, is available in Table 1 .…”

Section: Discussionmentioning

confidence: 99%

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Venetoclax in relapsed/refractory blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement: a case report and review of the literature

et al. 2021

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Background We describe a patient with blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement and the outcome of venetoclax use in this setting. Case presentation A 54-year-old Caucasian male was referred to the Haematology Unit with an enlarged inguinal lymph node which was diagnostic of a blastic plasmacytoid dendritic cell neoplasm. The staging revealed disseminated disease (skin, visceral, lymph nodes, and bone marrow). He received chemotherapy with an acute myeloid leukaemia-like regime. Afterwards, he underwent allogeneic haematopoietic stem cell transplantation, though it was not successful, showing a relapse 14 months later with hepatic and central nervous system dissemination. Intrathecal chemotherapy was administered, and venetoclax (anti-bcl2 agent) was started in an off-label indication based on most recent literature. The disease halted its course for 3 months. In the end, the patient’s disease progressed and so he succumbed due to infectious complications. Conclusions Venetoclax monotherapy seems not enough to control the disease progression under CNS involvement and other treatments should be investigated.

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Section: Discussionmentioning

confidence: 99%

“…Ann Hematol. (2020)[ 12 ] 1 64 2 Azacitidine CR >11 months 11 months Samhouri et al . J Oncol Pharm Pract.…”

Section: Discussionmentioning

confidence: 99%

Venetoclax in relapsed/refractory blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement: a case report and review of the literature

et al. 2021

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“…An in vitro study by Montero et al found that BPDCN cells were dependent on BCL‐2, and that these cells were responsive to the BCL‐2 inhibitor venetoclax 10 . In 2020, a case of relapsed BPDCN was successfully treated with venetoclax and azacytidine, a cytosine analog used for myelodysplastic syndrome 11 . CD123 inhibitors are another potential and emerging mode of therapy.…”

Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm with cutaneous presentation: A case series in children

Chan

Akintorin

Luu

et al. 2021

Pediatric Dermatology

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy that can present with multiorgan involvement including the skin. While this entity accounts for 0.1% of all cutaneous lymphomas in adults, fewer than 90 pediatric cases have been described in the literature. We present three cases of pediatric BPDCN, highlighting the variable cutaneous and clinical presentation.

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“…Piccini and colleagues described a 64 year old male with BPDCN involving the skin, lymph node, and bone marrow who received azacitidine (75 mg/m 2 once daily on days 1-7 of a 28 day cycle) and venetoclax (400 mg daily, escalating dose) with a quick and durable response for 11 cycles (and ongoing). 14 DiNardo and colleagues described two patients who received the same combination as a third and fifth line with cutaneous response and a decrease >50% of bone marrow blasts in one patient. 15 This quick and durable response in our patient with combination therapy and those reported cases are different from previous experience with monotherapies.…”

Section: Discussionmentioning

confidence: 99%

Tagraxofusp followed by combined azacitidine and venetoclax in blastic plasmacytoid dendritic cell neoplasm: A case report and literature review

Samhouri

Ursu

Dutton

et al. 2020

J Oncol Pharm Pract

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Introduction Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy that originates from plasmacytoid dendritic cells. It can involve skin, bone marrow, and/or lymph nodes. There is no consensus recommendation regarding treatment especially in the relapsed setting. Tagraxofusp, a CD123 directed agent, was recently approved by the Food and Drug Administration to treat BPDCN. We report a case of an elderly patient with diagnosis of BPDCN who was treated initially with tagraxofusp followed by azacitidine and venetoclax combination on relapse. Case report A 79 year old male presented with violaceous skin lesions. He had no other symptoms. Biopsy of these lesions was consistent with a diagnosis of BPDCN. Further testing showed no extracutaneous involvement. Management and outcome: Tagraxofusp was started at full dose (12 mcg/kg). This dose was not tolerated well. Patient could only tolerate the lowest dose (5 mcg/kg). Toxicities included elevated liver function tests, hyperglycemia, capillary leak syndrome, and pancreatitis. Dose escalation on progression was not possible due to side effects. Treatment was switched to venetoclax and azacitidine. Combination treatment was tolerated very well and patient showed major cutaneous response after 5 cycles and continues to do well. Discussion Tagraxofusp is a novel therapy that needs more real-world experience. This case describes the clinical course of an elderly patient on tagraxofusp. We also review the literature of azacytidine/venetoclax combination as a potential yet tolerable treatment option for this rare disease entity. This is the fourth case in literature to be treated with this combination.

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Quick complete response achievement with venetoclax and azacitidine in a case of relapsed disseminated blastic plasmacytoid dendritic cell neoplasm

Cited by 9 publications

References 6 publications

Venetoclax in relapsed/refractory blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement: a case report and review of the literature

Venetoclax in relapsed/refractory blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement: a case report and review of the literature

Blastic plasmacytoid dendritic cell neoplasm with cutaneous presentation: A case series in children

Tagraxofusp followed by combined azacitidine and venetoclax in blastic plasmacytoid dendritic cell neoplasm: A case report and literature review

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