Small B cell lymphomas and leukemias including hairy cell leukemia

Introduction 1123 Chronic lymphocytic leukemia 1123 Epidemiology 1124 Diagnosis 1124 Clinical features 1124 Clinical staging and prognostic markers 1125 Clinical staging systems 1125 Traditional prognostic markers 1125 New prognostic markers 1126 Treatment 1127 Pretreatment evaluation 1127 Criteria for response and minimal residual disease 1128 First-line therapy 1129 Treatment at relapse 1132 Stem cell transplantation 1134 New therapies 1135 Richter large cell transformation 1135 Management of complications 1136 B cell prolymphocytic leukemia 1137 Immunophenotype and cytogenetics 1137 Clinical course and management 1137 Hairy cell leukemia 1137 Morphology and immunophenotype 1138 Treatment 1138 Splenectomy 1138 Interferon alpha 1138 Pentostatin 1138 Cladribine 1138 Treatment at relapse 1138 Hairy cell leukemia variant 1139 Key points 1139 References 1140 Small B cell lymphoproliferative disorders comprise a variety of disease entities, the commonest of which is chronic lymphocytic leukemia (CLL). Rarer primary B cell leukemias include B prolymphocytic leukemia (PLL) and hairy cell leukemia (HCL). In addition, clonal B cells may appear in the peripheral blood originating from a primary non-Hodgkin lymphoma (NHL).