Which mutation is most commonly positive in hairy cell leukemia?

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Multiple Choice

Which mutation is most commonly positive in hairy cell leukemia?

Hairy cell leukemia is driven by a B-cell–specific genetic event, with the BRAF V600E mutation being the most characteristic and commonly found in classic cases. This mutation causes constitutive activation of the MAPK pathway, promoting the malignant B cells’ growth and survival. Detecting BRAF V600E not only supports the diagnosis but also guides targeted therapy with BRAF inhibitors, which can be effective in relapsed or refractory disease. In contrast, the other mutations are associated with different hematologic diseases: BCR-ABL is typical of chronic myeloid leukemia and some ALL cases; JAK2 V617F is seen in polycythemia vera and other myeloproliferative neoplasms; FLT3-ITD is commonly seen in acute myeloid leukemia. Therefore, BRAF V600E is the best answer because it is the defining mutation most closely linked to classic hairy cell leukemia.

Hairy cell leukemia is driven by a B-cell–specific genetic event, with the BRAF V600E mutation being the most characteristic and commonly found in classic cases. This mutation causes constitutive activation of the MAPK pathway, promoting the malignant B cells’ growth and survival. Detecting BRAF V600E not only supports the diagnosis but also guides targeted therapy with BRAF inhibitors, which can be effective in relapsed or refractory disease. In contrast, the other mutations are associated with different hematologic diseases: BCR-ABL is typical of chronic myeloid leukemia and some ALL cases; JAK2 V617F is seen in polycythemia vera and other myeloproliferative neoplasms; FLT3-ITD is commonly seen in acute myeloid leukemia. Therefore, BRAF V600E is the best answer because it is the defining mutation most closely linked to classic hairy cell leukemia.

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