A Review on Chronic Neutropenia of the Adult: Emerging Clinical Dilemmas

Chronic neutropenia of the adult is a cause of often unjustified anxiety for both the patients and the medical community. Various studies show that the condition is generally benign with little risk of infection and clonal evolution. Here we provide a review of the available literature, focusing on patient evaluation and clinical management of the adult with chronic idiopathic/autoimmune neutropenia. Data presented advise to evaluate the clinical severity basing on neutrophil counts (< or > 0.5×109/L) and on the history of infections, to carefully examine personal and family history and to exclude congenital and benign forms of the infancy. Furthermore, nutritional deficit, infections, autoimmune diseases and neoplasms should be looked for and excluded. Hematologic constitutional symptoms, LDH, beta2microglobulin levels and lymphocytosis may hint the diagnosis of an underlying hematologic disease (lymphoproliferative syndrome). Once secondary forms have been excluded, the research for anti-neutrophils autoantibodies and bone marrow evaluation should be performed in case of persistent severe neutropenia, whilst follow up may be enough for mild/moderate cases. The use of G-CSF and prompt broad-spectrum antibiotics, covering for pseudomonas aeruginosa, is suggested in case of febrile neutropenia only. Finally, the introduction of new clinical entities (ICUS, IDUS, CCUS, and CHIP) within the myeloid diseases classification is going to change the diagnostic scenario in the next future