Clinical course and prognosis of the lymphoproliferative disease of granular lymphocytes. A multicenter study
| Autor: | Alberto Mantovani, G. Semenzato, P. Mancini, F Pandolfi, Marshall E. Kadin, R. G. Steis, Teodoro Chisesi, A. S. Fauci, A. Cafaro, M. C. Garcia Rodriguez, Giovanni Pizzolo, Felice Salsano, F. Herrmann, Tiziano Barbui, Robert W. McKenna, T. P. Loughran, G. De Rossi, G. Gastl, J. W. Vanoostveen, G. Starkebaum, G. Schlimok, J. C. Brouet, J. U. Brisbane, Wing C. Chan, M. Palutke, H. F. Pross, S. J. Proctor, S. Tagawa, Fernando Aiuti, Christian H. Geisler, Renato Zambello |
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| Jazyk: | angličtina |
| Rok vydání: | 1990 |
| Předmět: |
Adult
Male Cancer Research medicine.medical_specialty Pathology Adolescent Aged Aged 80 and over Analysis of Variance Child Child Preschool Female Follow-Up Studies Humans International Cooperation Lymphocytosis Lymphoproliferative Disorders Middle Aged Multicenter Studies as Topic Predictive Value of Tests Prognosis Retrospective Studies Survival Analysis Anemia Lymphoproliferative disorders Neutropenia Gastroenterology Asymptomatic Internal medicine medicine 80 and over Preschool T-Cell Large Granular Lymphocyte Leukemia Survival analysis Univariate analysis business.industry medicine.disease Oncology medicine.symptom business |
| Popis: | Lymphoproliferative disease of granular lymphocytes (LDGL) is a recently recognized, relatively rare atypical lymphocytosis characterized by the presence of over 2000 lymphocytes with cytoplasmic azurophilic granules/mm3 in the peripheral blood. The clinical course is heterogeneous, varying from spontaneous regression to progressive, malignant disease. As a consequence, clinical intervention is not standardized. In a worldwide multicenter study, the authors observed 151 patients with LDGL for a mean follow-up time of 29 months. Forty-three patients were asymptomatic at the time of diagnosis. In the remaining cases, clinical symptoms included fever (41 cases), infections (58), neutropenia (47), anemia (17), and thrombocytopenia (12). In 69 cases, LDGL coexisted with an associated disease. Most patients had a nonprogressive clinical course despite the presence of severe symptoms. In 19 patients, death related to LDGL occurred within 48 months. The authors investigated which features at diagnosis were significantly associated with increased mortality. In the univariate analysis, lymph node and liver enlargement, fever at presentation, skin infiltration, a low (less than or equal to 5000/mm3) or high (greater than 20,000/mm3) peripheral leukocyte count, relatively low (less than or equal to 3000) or high (greater than 7000/mm3) absolute peripheral granular lymphocyte (GL) count, and a low (less than or equal to 15%) percentage of HNK-1-positive cells were found to be predictors of increased mortality. In the multivariate analysis, significant independent predictors were fever at diagnosis, a low (less than or equal to 15%) percentage of HNK-1-positive peripheral blood mononuclear cells (PBMC) and a relatively low (less than or equal to 3000) GL count. These results showed that about 25% of the patients with LDGL were diagnosed after a routine blood count and had no clinical symptoms. The remaining patients were symptomatic, with some experiencing a fatal clinical course. The author's analysis of the significant prognostic features of LDGL may help in understanding the heterogeneous nature of this syndrome. |
| Databáze: | OpenAIRE |
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