Leucemia de células pilosas: Tratamiento–Versión para profesionales de salud (PDQ®)
SECCIONES
- Información general sobre la leucemia de células pilosas
- Información sobre los estadios para la leucemia de células pilosas
- Aspectos generales de las opciones de tratamiento para la leucemia de células pilosas
- Tratamiento de la leucemia de células pilosas
- Leucemia recidivante o resistente de células pilosas
- Modificaciones a este sumario (07/22/2015)
- Información sobre este sumario del PDQ
- Ver todas las secciones
Información general sobre la leucemia de células pilosas
Factores pronósticos
La leucemia de células pilosas es un linfoma de células B de crecimiento lento, de grado bajo que se caracteriza por lo siguiente:
- Células B circulantes con proyección citoplásmica (de apariencia "pilosa").
- Esplenomegalia.
- Linfadenopatía ausente.
- Pancitopenia.
- Monocitopenia.
Diagnóstico
Además de los antígenos de células B CD19, CD20 y CD22, las células coexpresan CD11c, CD25 y CD103. La mutación BRAF-V600E es una lesión genética que define la leucemia de células pilosas y que se puede usar como diagnóstico.[1] La decisión de dar tratamiento se basará en la presencia de citopenias sintomáticas, esplenomegalia masiva u otras complicaciones. Cerca de 10% de los pacientes nunca necesitarán tratamiento.
Bibliografía
- Tiacci E, Schiavoni G, Forconi F, et al.: Simple genetic diagnosis of hairy cell leukemia by sensitive detection of the BRAF-V600E mutation. Blood 119 (1): 192-5, 2012. [PUBMED Abstract]
Hairy Cell Leukemia Treatment (PDQ®)—Health Professional Version - National Cancer Institute
Hairy Cell Leukemia Treatment–Health Professional Version (PDQ®)
SECTIONS
- General Information About Hairy Cell Leukemia
- Stage Information for Hairy Cell Leukemia
- Treatment Option Overview for Hairy Cell Leukemia
- Treatment for Hairy Cell Leukemia
- Relapsed or Refractory Hairy Cell Leukemia
- Changes to This Summary (03/18/2016)
- About This PDQ Summary
- View All Sections
Changes to This Summary (03/18/2016)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Added level of evidence: 3iiiDiv to data from a multicenter trial about the first treatment option, cladribine.
Added text to state that a retrospective review of 83 patients, aged 40 years and younger, reported a median time to first relapse of 54 months for all responders and a median overall survival of 21 years from diagnosis (cited Rosenberg et al. as reference 7).
Added Kreitman et al. as reference 16. Added text to state that interferon-alpha and splenectomy are therapeutic options that can be considered when other options have been exhausted.
Added text to state that the BRAF-V600E mutation occurs in almost 100% of classic-form hairy cell leukemia patients and almost never in other B-cell lymphomas and leukemias, including hairy cell leukemia variants (cited Pettirossi et al. as reference 17). Also added that two phase II, multicenter studies in the United States and Italy evaluated the BRAF inhibitor, vemurafenib, given orally for 4 months; after a median follow-up of 23 months, the overall response rate for 50 patients was 98%, the complete response rate was 38%, and the median treatment-free survival was 25 months and 18 months in the two studies (cited Tiacci et al. as reference 18 and level of evidence 3iiiDiv).
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.
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