Bcellymphoproliferatieve

B cell lymphoproliferative disorders (BCLPDs) are a heterogeneous group of conditions characterized by abnormal proliferation of B lymphocytes. They encompass malignant neoplasms such as chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL); mantle cell lymphoma; follicular lymphoma; marginal zone lymphomas including extranodal MALT lymphomas; hairy cell leukemia; and other less common B cell neoplasms. The category also includes reactive or lymphoproliferative processes associated with immune deficiency or immunosuppression, such as post-transplant lymphoproliferative disorder (PTLD).

Pathophysiology involves clonal expansion of malignant or dysregulated B cells driven by genetic alterations, microenvironmental interactions, and, in some cases, viral factors such as Epstein-Barr virus. Alterations in specific genes or chromosomal abnormalities influence prognosis and treatment response and help distinguish subtypes.

Clinical presentation varies by subtype. Common features include lymphadenopathy, splenomegaly, cytopenias, and B symptoms (fever, night sweats, weight loss). Some diseases are indolent and asymptomatic for years, while others are aggressive and rapidly progressive. Diagnosis relies on a combination of complete blood counts, peripheral blood or tissue biopsy, flow cytometry to identify B-cell immunophenotype (for example, CD19, CD20, often CD5 in CLL), cytogenetics, and molecular studies. Staging uses conventional systems tailored to lymphoma subtypes, such as Ann Arbor for many B-cell lymphomas.

Management is subtype- and stage-specific. Indolent diseases may be monitored initially; treatment often includes immunochemotherapy (for example, rituximab-containing regimens) and targeted therapies such as BTK inhibitors. Aggressive lymphomas typically require combination chemotherapy with rituximab or other targeted approaches. Prognosis varies widely by subtype and risk factors, with ongoing advances improving outcomes through personalized therapies and novel agents.