Understanding hairy cell leukemia in the context of mature B-cell neoplasms: tumor microenvironment and extracellular vesicle contribution to disease pathogenesis

Evidence from laboratory and animal studies provides groundwork for understanding mechanisms and potential before human studies continue.

• Level A · Stronger Clinical Evidence
• Level B · Emerging clinical evidence with positive signals
• Level C · Early human research exploring benefits
• Level D · Scientific groundwork from lab and animal studies
• Emerging · Emerging topic under active research

Abstract (original English)

Mature B-cell neoplasms constitute a biologically and clinically heterogeneous group of hematologic malignancies, defined by the clonal proliferation and accumulation of monotypic mature B lymphocytes, which may involve the peripheral blood, bone marrow (BM), lymphoid tissues, or present primarily in extranodal sites. While the pathogenesis of common subtypes, such as chronic lymphocytic leukemia, multiple myeloma, Hodgkin Lymphoma and Diffuse Large B-cell Lymphoma has been extensively studied, those of rare entities like Hairy Cell Leukemia (HCL) remains poorly understood. HCL is a distinct B-cell neoplasm marked by BM infiltration of atypical "hairy" cells, pancytopenia, BM fibrosis, and the BRAF V600E mutation, which is a defining molecular hallmark of the classic form of the disease. Analogous to solid tumors, growing evidence shows that the tumor microenvironment (TME) is a pivotal contributor in both the initiation and progression of all these malignancies. However, in rare mature B-cell neoplasms, understanding how tumor cells interact with their microenvironment, in terms of immune invasion, stromal crosstalk, and tissue remodeling, remains a challenge, partly due to the scarcity of patient samples and limited availability of preclinical models. In the context of TME, extracellular vesicles (EVs) have emerged as central mediators of intercellular communication within bo

Evidence level

Evidence from laboratory and animal studies provides groundwork for understanding mechanisms and potential before human studies continue.

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