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Autoimmune Cytopenia in CLL Prognosis and Management in the Era of Targeted Therapies

Journal

CANCER JOURNAL
Volume 27, Issue 4, Pages 286-296

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/PPO.0000000000000537

Keywords

Autoimmune cytopenia; autoimmune hemolytic anemia; chronic lymphocytic leukemia; immune thrombocytopenia; management; prognosis; targeted therapies

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Funding

  1. Instituto de Salud Carlos III [FIS PI19/00753]
  2. Jose Carreras Leukamie-Stiftung

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Chronic lymphocytic leukemia (CLL) is often accompanied by autoimmune cytopenia (AIC), and the success of treatment depends on the patient's response to therapy. A flexible strategy involving initial corticosteroid therapy and transitioning to effective CLL treatment when necessary is crucial for managing active CLL-related AIC.
Chronic lymphocytic leukemia (CLL) is frequently associated with autoimmune hemolytic anemia and immune thrombocytopenia and, less frequently, with pure red cell aplasia and immune neutropenia. The emergence of these complications is related to an intertwined and complex relationship between patient, disease, and treatment characteristics. The prognostic repercussion of autoimmune cytopenia (AIC) in patients with CLL mainly depends on its response to therapy. For patients with AIC and nonactive CLL, treatment is as in primary, uncomplicated AIC, keeping in mind that no response is an indication for CLL therapy. The success of treating active CLL-related AIC widely relies on a flexible strategy that should include initial therapy with corticosteroids and a rapid shift to effective CLL therapy in nonresponding patients. Targeted therapies (e.g., ibrutinib) that have already demonstrated to be effective in CLL-related AIC will likely offer a unique possibility of treating both AIC and CLL as a single target.

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