What Is the Treatment of Hairy-Cell Leukemia with Symptomatic Splenomegaly and Cytopenias?
This protocol addresses classical hairy-cell leukaemia in patients presenting with active, symptomatic disease who are not pregnant.
Clinical Scenario
Treatment should be initiated when symptomatic disease is present, defined by one or more of the following findings:
- Bulky or progressive symptomatic splenomegaly
- Cytopenias: haemoglobin <10 g/dl, and/or platelets <100 × 109/l, and/or neutrophils <1 × 109/l
- Recurrent or severe infections
- Systemic symptoms
Treatment Approach
In selected patients with resistant massive symptomatic splenomegaly, a surgical intervention may be appropriate, subject to specific anatomical and bone marrow criteria. Prophylactic measures are required prior to any such intervention.
The complete eligibility criteria, prerequisites, and full management algorithm are available in the structured protocol below.
References
DOI: 10.1093/annonc/mdv200
- Treatment should be initiated in patients with symptomatic disease manifested by bulky or progressive, symptomatic splenomegaly cytopaenias (haemoglobin <10 g/dl and/or platelets <100 × 109/l and/or neutrophils <1 × 109/l), recurrent or severe infections and/or systemic symptoms [II, A] [17, 18].
- Splenectomy may be indicated in patients with resistant massive symptomatic splenomegaly (>10 cm below the costal margin) with accompanied low-level bone marrow infiltration [IV, B] [48].
- Vaccination against Haemophilus influenza and pneumococcus before splenectomy is recommended [IV, B] [49].
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