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Published on: July 20, 2016
Hairy Cell Leukemia
Rossella Riccioni1, Sara Galimberti, Mario Petrini
1Department of Oncology, Transplantation and New Technology in Haematology, University of Pisa, Pisa, Italy.
Insights
Hairy cell leukemia (HCL) is a slow-growing B-cell cancer. Purine analogs like cladribine (2-CdA) and rituximab are effective treatments, inducing high remission rates and improving patient outcomes.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Hairy cell leukemia (HCL) is an indolent B-cell lymphoproliferative disorder.
- Key features include splenomegaly, pancytopenia, and characteristic lymphocyte morphology.
- Differential diagnosis relies on immunophenotyping (CD103, CD25, CD11c co-expression).
Purpose of the Study:
- To review the current understanding and treatment of Hairy cell leukemia.
- To highlight the efficacy of purine analogs and rituximab in managing HCL.
- To discuss the impact of novel therapies on disease course and patient outcomes.
Main Methods:
- Literature review of Hairy cell leukemia treatment strategies.
- Analysis of treatment outcomes with purine analogs (Cladribine) and rituximab.
- Evaluation of immunophenotyping in HCL diagnosis.
Main Results:
- Cladribine (2-CdA) induces over 80% complete remission rates in HCL patients.
- Rituximab, used after 2-CdA, achieves molecular responses in most cases.
- Combination therapies (purine analogs, rituximab, chemotherapy) improve clinical course with low toxicity.
Conclusions:
- Purine analogs are the established treatment of choice for Hairy cell leukemia.
- Rituximab enhances treatment efficacy, particularly in achieving molecular remission.
- Modern therapeutic approaches significantly modify HCL prognosis with manageable toxicity.
Abstract:
Hairy cell leukemia (HCL) is an indolent B-cell lymphoproliferative disease, characterized by splenomegaly and pancitopenia related to this. The lymphocytes present characteristic citoplasmatic projections and are positive for tartrate-resistant acid phosphatase (TRAP). Immunophenotyping is necessary to identify the co expression of CD103, CD25, CD11c associated with a typical B-cell clonally pattern and to make a differential diagnosis from other indolent malignancies. Despite the indolent clinical course, treatment is required to resolve symptoms related to splenomegaly and to reduce the incidence of severe infections that are the major complications and a common cause of death. In the past the treatment was only able to resolve the symptoms. In the revised literature, purine analog have been identified as the treatment of choice for this disease. Cladribrine (2-CdA) is able to induce more than 80% of complete remission and is also effective in relapsed patients. Rituximab after 2-CdA treatment can obtain a molecular response in most cases. The introduction of purine analog, and recently of Rituximab, in association with conventional chemotherapy can modify the clinical course of the disease with low toxicities.
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