Early Findings of Novel Agent Show Promise in Treating Rare Blood Cancer


Patients with blastic plasmacytoid dendritic cell neoplasms can enroll in the ongoing CADENZA trial, which is investigating the safety and efficacy of the novel agent pivekimab sunirine in patients with the rare blood cancer.

The phase 2 CADENZA clinical trial evaluating frontline pivekimab sunirine (pivekimab) in patients with blastic plasmacytoid dendritic cell neoplasm is enrolling patients with the rare form of blood cancer, including those with de novo disease or those with prior or concomitant hematologic malignancy.

Blastic plasmacytoid dendritic cell neoplasm — previously known as natural killer (NK) cell leukemia/lymphoma — is a rare and aggressive type of blood cancer that has leukemia and lymphoma features. It commonly appears as skin lesions and can occur with or without bone marrow involvement.

Pivekimab sunirine works by targeting CD123, a cell surface antigen that is present on leukemia cells.

Findings from the first 10 patients in the CADENZA trial showed that two out of the four patients with de novo disease achieved a complete response or clinical complete response, meaning that there was no detectable cancer left after treatment with pivekimab.

Four out of the six patients with prior or concomitant hematologic malignancy experienced a complete response, clinical complete response or complete response with partial hematological recovery, meaning that cancer disappeared, and blood cell levels moved toward normal ranges.

There are no treatments for this specific patient population with this rare type of blood cancer. While pivekimab may not completely rid patients’ bone marrow of blastic plasmacytoid dendritic cell neoplasm, it may help them become eligible for transplant, according to an expert.

“What has been observed is that patients with (prior or concomitant hematologic malignancy) who’ve cleared their marrow of (blastic plasmacytoid dendritic cell neoplasm) with pivekimab may not fully recover counts, likely due to the underlying concurrent heme malignancy,” Dr. Kendra Sweet, associate member in the Department of Malignant Hematology at the Moffitt Cancer Center in Tampa, Florida, said in a press release. “Despite partial count recovery, complete clearance of (blastic plasmacytoid dendritic cell neoplasm) allows these patients to bridge to transplant, when eligible, the only curative option for (blastic plasmacytoid dendritic cell neoplasm).”

Additionally, three patients who received pivekimab in the frontline setting experienced a complete response or complete clinical response.

“We believe these initial frontline data from the CADENZA study further support the potential of pivekimab as an important treatment option for patients with (blastic plasmacytoid dendritic cell neoplasm),” said Dr. Anna Berkenblit, senior vice president and chief medical officer of ImmunoGen, the manufacturer of the agent.

ImmunoGen announced that they plan on enrolling up to 20 patients with de novo blastic plasmacytoid dendritic cell neoplasm on to the CADENZA trial. Top-line data from this research is expected in 2024, according to the pharmaceutical company.

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