[Chronic B-cell lymphoproliferative disorders with hairy cells]

Area of Science:

• Hematology
• Oncology
• Pathology

Context:

• Blood smear examination is crucial for diagnosing B-cell chronic lymphoproliferative disorders.
• Hairy cell proliferations encompass heterogeneous entities, making diagnosis challenging.
• Hairy cell leukemia (HCL) and splenic marginal zone lymphoma (SMZL) are distinct, while HCL variant (HCLv) and splenic diffuse red pulp small B-cell lymphoma (SDRPL) are provisional.

Purpose:

• To discuss the clinical and biological characteristics of four B-cell lymphoproliferative disorders.
• To outline methods for characterizing, identifying, and distinguishing these entities.
• To review current and emerging therapeutic strategies, including targeted drugs.

Summary:

• Standardized blood smear examination, multiparameter flow cytometry, immunoglobulin gene analysis, and molecular analyses (BRAF, MAP2K1 mutations) are key diagnostic tools.
• Distinguishing between HCL, SMZL, HCLv, and SDRPL requires a combination of these investigations.
• The study emphasizes the importance of accurate diagnosis for effective treatment selection.

Impact:

• Improved diagnostic accuracy for challenging B-cell lymphoproliferative disorders.
• Guidance for clinicians and laboratory professionals in identifying and differentiating these conditions.
• Informed therapeutic decisions, particularly with the advent of new targeted drugs.