In patients with classic hairy cell leukemia, standard therapy involves induction with a purine nucleoside analog (Fig. 1). Patients treated with either pentostatin or cladribine are expected to achieve a durable complete remission in at least 76–91% of the cases [9], [37], [48] and [56]. In those treated with pentostatin, there is a lower reported frequency of febrile neutropenia [38]. Typically, patients are treated with pentostatin at two week intervals AZD6244 order in the outpatient clinic until a complete remission has been documented [38]. This induction therapy

could require six months or longer to secure a complete response. While many of these patients are then treated with two additional courses of pentostatin as consolidation, whether this additional therapy is required is still unclear. In contrast, patients treated with cladribine usually receive a single five to seven day course of therapy and are followed until a complete remission has been documented. In those patients achieving a complete response to either therapy, no evidence of residual hairy cell leukemia can be observed morphologically. Immunohistochemical stains on the bone marrow biopsy or immunophenotypic analysis of the bone marrow aspirate may reveal minimal residual disease (MRD) in these patients. Another area for a continued discussion entails establishing

a uniform definition of a complete Proteasome inhibitor remission, and the reproducibility of defining negative MRD status following therapy. Establishment of a consensus on the definition of a complete response and the necessity for quantifying the extent of MRD by various methods including immunohistochemistry, flow cytometry, or deep sequencing should be a priority. While the extent of MRD remaining after initial therapy is generally felt to be important with respect to predicting

long-term outcome, the quantitative extent and timing of these assessments are important [[32], [57] and [58]]. More work is needed in the context of organized clinical trials to validate these relationships. A consensus in terms of the importance of eradicating MRD requires GNA12 a general agreement on the definitions of complete remission, thresholds for identifying MRD, and relapse. While most definitions of complete remission require that no morphologic evidence of hairy cell leukemia can be observed on routine hematoxylin and eosin staining of the bone marrow, many hematopathologists report the percentage of residual hairy cell infiltration based upon immunohistochemical stains or immunophenotypic analyses of bone marrow flow cytometric studies. Following purine analog therapy, there can be delayed and continuous improvements in bone marrow leukemic cell infiltration [32]. The assessment of residual hairy cell leukemic infiltration thus may vary depending upon the time of analysis.