Hairy cell leukaemia and its variant form: treatment in 2024
Inchiappa, Luca
Hairy cell leukaemia and its variant form: treatment in 2024 - 2025.
53
Hairy cell leukemia (HCL) is a rare form of leukemia that belongs to the group of mature and chronic B-cell lymphoid disorders. There are two distinct entities: the classic form and the variant form, each with different cytological, phenotypic, and molecular characteristics. Splenic diffuse red pulp lymphoma, a hairy cell proliferation, is very close to the variant form. The classic form is easily diagnosed with the currently available tools, as the BRAFV600E mutation is detected in more than 95% of patients. The introduction of purine analogs has revolutionized the prognosis of this disease, with overall survival curves approaching those of the general population. The current treatment of choice is cladribine in combination with rituximab, which has demonstrated the best outcomes in terms of complete remission (CR), progression-free survival (PFS) and minimal residual disease (MRD) negativity rates. However, relapses still occur and recent discoveries of targeted therapies have transformed the management of these HCL relapsed/refractory patients, in particular BRAF inhibitors (BRAFi) combined with anti-CD20 monoclonal antibodies. Other agents are available for subsequent relapses or for patients without the BRAFV600E mutation. The variant form has a less favorable prognosis, largely due to a poorer response to purine analogs. In cases of relapse, alternative therapies are available, although options are more limited. Finally, recent advances in the understanding of the pathogenic mechanisms of both forms including in signaling pathways are opening new therapeutic avenues? In this article, we present a review on the clinical and biological aspects of hairy cell leukemia and its variant form and we propose a treatment algorithm.
Hairy cell leukaemia and its variant form: treatment in 2024 - 2025.
53
Hairy cell leukemia (HCL) is a rare form of leukemia that belongs to the group of mature and chronic B-cell lymphoid disorders. There are two distinct entities: the classic form and the variant form, each with different cytological, phenotypic, and molecular characteristics. Splenic diffuse red pulp lymphoma, a hairy cell proliferation, is very close to the variant form. The classic form is easily diagnosed with the currently available tools, as the BRAFV600E mutation is detected in more than 95% of patients. The introduction of purine analogs has revolutionized the prognosis of this disease, with overall survival curves approaching those of the general population. The current treatment of choice is cladribine in combination with rituximab, which has demonstrated the best outcomes in terms of complete remission (CR), progression-free survival (PFS) and minimal residual disease (MRD) negativity rates. However, relapses still occur and recent discoveries of targeted therapies have transformed the management of these HCL relapsed/refractory patients, in particular BRAF inhibitors (BRAFi) combined with anti-CD20 monoclonal antibodies. Other agents are available for subsequent relapses or for patients without the BRAFV600E mutation. The variant form has a less favorable prognosis, largely due to a poorer response to purine analogs. In cases of relapse, alternative therapies are available, although options are more limited. Finally, recent advances in the understanding of the pathogenic mechanisms of both forms including in signaling pathways are opening new therapeutic avenues? In this article, we present a review on the clinical and biological aspects of hairy cell leukemia and its variant form and we propose a treatment algorithm.
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