Mixed-phenotype large granular lymphocytic leukemia: a rare subtype in the large granular lymphocytic leukemia spectrum.
Hum Pathol
; 81: 96-104, 2018 11.
Article
en En
| MEDLINE
| ID: mdl-29949739
ABSTRACT
Large granular lymphocytic leukemia (LGLL) is a chronic proliferation of cytotoxic lymphocytes in which more than 70% of patients develop cytopenia(s) requiring therapy. LGLL includes T-cell LGLL and chronic lymphoproliferative disorder of natural killer (NK) cells. The neoplastic cells in LGLL usually exhibit a single immunophenotype in a patient, with CD8-positive/αß T-cell type being the most common, followed by NK-cell, γδ T-cell, and CD4-positive/αß T-cell types. We investigated a total of 220 LGLL cases and identified 12 mixed-phenotype LGLLs (5%) 7 cases with coexistent αß T-cell and NK-cell clones and 5 with coexistent αß and γδ T-cell clones. With a median follow-up of 48 months, the clinicopathological characteristics of these patients seemed similar to those of typical LGLL patients. Treatment was instituted in 9 patients, and 5 patients (55%) attained complete hematologic response or partial response. The therapeutic response rate of this cohort is comparable to the reported overall response rate of 40% to 60% in typical LGLL patients. Three patients who did not receive any treatment had progressive or persistent cytopenias. Interestingly, inverted proportions of 2 clones at disease recurrence were identified in 4 patients (36%) and stable clonal proportions in 7 patients (64%). Mixed-phenotype LGLL is rare, and this study underscores the importance of recognizing this rare type of LGLL in patients who may benefit from LGLL treatment.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Asunto principal:
Células Asesinas Naturales
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Receptores de Antígenos de Linfocitos T
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Linfocitos T
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Leucemia Linfocítica Granular Grande
Tipo de estudio:
Prognostic_studies
Límite:
Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Hum Pathol
Asunto de la revista:
PATOLOGIA
Año:
2018
Tipo del documento:
Article
País de afiliación:
Estados Unidos