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What second-line therapies would you recommend for Rosai-Dorfman disease following a modest partial response to prednisone?

January 9, 2024

January 2024

We asked, and you answered! Here are the responses from this month’s “You Make the Call” question on what second-line therapies you would recommend for Rosai-Dorfman disease following modest response to prednisone.


Disclaimer: ASH does not recommend or endorse any specific tests, physicians, products, procedures, or opinions, and disclaims any representation, warranty, or guaranty as to the same. Reliance on any information provided in this article is solely at your own risk.


Steroid refractory cases can be given a trial of chemotherapy with vincristine, etoposide, or vinblastine. If the patient’s disease has a BRAF mutation, vemurafenib or dabrafenib may be used. The MEK inhibitor cobimetinib may also be an option.

Saifudeen Abdulrahman, MD
Salalah, Oman

My approach to this case would be surgical resection or radiotherapy, if possible, because of the bulky disease and lymphedema. I might also use sirolimus, cladribine, clofarabine, methotrexate, mercaptopurine, vincristine, immunomodulatory drugs such as thalidomide or lenalidomide, or rituximab.

Nelson Hamerschlak, MD, PhD
Sao Paulo, Brazil

The notion of first- or second-line therapies should be avoided in RDD. However, other therapies for RDD after steroids are sirolimus, chemotherapy with 6-mercaptopurine or methotrexate, and vinca alkaloids in combination, or immunotherapy with tumor necrosis factor-alpha inhibitors.

Israr Khan, MD
Chicago, IL

 

I would give sirolimus.

Sanjay Tewari, MD, PhD
London, England

I would treat the patient with four to six cycles of rituximab at 500 mg/m2.

Michalis Michael, MD
Nicosia, Cyprus

There is no standard approach to this scenario; however, for long-term disease control, I suggest chemotherapy with the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone) or cladribine because of the patient’s age. Other options like sirolimus, talidomide, lenalidomide, interferon, or imatinib have less efficacy and controversial outcomes.

Armando Norato Delgado, MD
Mexico City, Mexico

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