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Treatment for rare cancer associated with skin manifestations


Researchers make headway in determining life-prolonging treatment for blastic plasmacytoid dendritic cell neoplasm, a rare cancer that is often associated with skin manifestations.

doctor comforting patient

High-dose methotrexate with asparaginase offers blastic plasmacytoid dendritic cell neoplasm patients a relatively high response rate and lower toxicity profile than other chemotherapy treatments. The treatment paves the way for hematopoietic cell transplantation, which is the best chance for a possible cure, according to a study published in Blood Advances.

RELATED: Skin manifestations an early sign of rare hematologic cancer

Blastic plasmacytoid dendritic cell neoplasm is a rare, aggressive hematologic malignancy with a likelihood of skin manifestations. There is no consensus about how to best treat the neoplasm. What is known is that while most patients respond to different chemotherapy treatments, almost all patients relapse regardless of the chemotherapy regimen used. Patients’ overall survival with such treatment is an average of one year.

French researchers developed a nationwide network of blastic plasmacytoid dendritic cell neoplasm patients to better understand the cancer and optimal treatment. They conducted a retrospective, observational analysis of 86 blastic plasmacytoid dendritic cell neoplasm patients. Nearly 85% of those patients had tumoral skin involvement.

“Cutaneous lesions were largely predominant and preceded hematological or visceral involvement in 41 patients (47.7%) by a mean time of 2.5months. A single cutaneous lesion was present in 40 patients (46.5%), while disseminated skin involvement was visible in 33 patients (38%), and disease remained localized to the skin for only two patients,” the authors wrote.

Their analysis revealed tumors expressed markers associated with plasmacytoid dendritic cell origin, including HLA-DRhigh, CD303+, CD304+, and cTCL1+, plus CD4 and CD56. They noted frequent expression of isolated markers from the myeloid, B-, and T-lymphoid lineages.

Myeloperoxidase, CD14, cCD3, CD19, and cCD22 were not expressed.

“Fifty-one percent of cytogenetic abnormalities impact chromosomes 13, 12, 9, and 15. Myelemia was associated with an adverse prognosis,” they wrote.

Researchers differentiated the patients’ treatment regimens as acute myeloid leukemia–like; acute lymphoid leukemia–like; lymphoma (cyclophosphamide, doxorubicin, vincristine, and prednisone, or CHOP)–like; high-dose methotrexate with asparaginase chemotherapies; and not-otherwise-specified treatments.

Providers had treated 30 patients in the series with allogeneic hematopoietic cell transplantation and four received autologous hematopoietic cell transplantation.

They found survival was the same in patients treated with acute myeloid leukemia–like, acute lymphoid leukemia–like, and high-dose methotrexate with asparaginase chemotherapies. Patients who received one of those three regimens tended to have better median duration of remission than patients treated with CHOP-like or unspecified regimens.

At the time of the study, which was published in December 2019, 11 of the patients were in persistent complete remission after allogeneic hematopoietic cell transplantation. Researchers reported that patients who had allogeneic hematopoietic cell transplantation had a median survival 49 months, while other patients had a median 8 months survival. Fourteen patients relapsed post hematopoietic cell transplantations, including all of those who received autologous hematopoietic cell transplantation.

They concluded that blastic plasmacytoid dendritic cell neoplasm patients who had the highest complete remission rates after first-line treatment were most likely to achieve prolonged survival if they also had allogeneic hematopoietic cell transplantation. Toxicity is a challenge especially among elderly patients treated with leukemia-like regimens.

“Our series confirms the potential for a high response rate, similar to leukemia-like regimens, with the association of [methotrexate] and asparaginase, with a low toxicity profile, even in elderly patients. We believe that this approach offers the vast majority of [blastic plasmacytoid dendritic cell neoplasm] patients their best chance at gaining access to [hematopoietic cell transplantation] as a consolidation treatment, which remains the best way to ensure prolonged response,” the authors wrote.



Reference: Garnache-Ottou F, Vidal C, Biichlé S, et al. How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients?. Blood Adv. 2019;3(24):4238–4251. doi:10.1182/bloodadvances.2019000647

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