RESUMEN
A series of 24 consecutive patients affected by myelofibrosis with myeloid metaplasia was reviewed. The clinical-pathological features at onset were similar to those reported in literature; in particular in all the patients we observed splenomegaly and the typical leuko-erythroblastic picture in peripheral blood. The median survival of our series was of 57 months; the deaths were caused by severe anaemia and/or infection and/or haemorrhage; the blastic terminal transformations were rare. According to other authors, unfavourable prognostic factors in our patients were: male sex, advanced age, hepatomegaly, the presence of systemic signs, anaemia, leukocytosis, leukopenia, high number of circulating erythroblasts, thrombocytopenia, osteomyelosclerosis. We have confirmed the clinical value of three staging prognostic systems: the system proposed by Njoku based on haemoglobin level and reticulocytes number, the system proposed by Visani based on haemoglobin and circulating myeloid precursors number, the system proposed by Dupriez based on haemoglobin and white blood cells number.
Asunto(s)
Mielofibrosis Primaria/complicaciones , Adulto , Anciano , Anemia/etiología , Femenino , Hemoglobinas/metabolismo , Hepatomegalia/etiología , Humanos , Leucocitosis/etiología , Leucopenia/etiología , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Mielofibrosis Primaria/sangre , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Esplenomegalia/etiología , Trombocitopenia/etiología , Trombocitosis/etiologíaRESUMEN
OBJECTIVE: The "International Prognostic Index" (IPI) has been published for patients with histological intermediate grade malignancy non-Hodgkin's lymphoma (NHL) according to the Working Formulation (WF). The IPI is based on pre-treatment clinical characteristics: age, performance status, Ann Arbor stage, extranodal sites, serum lactate-dehydrogenase concentration. We investigated whether the IPI also had prognostic value for NHL patients with a low grade malignancy or high grade malignancy according to the WF. PATIENTS AND METHODS: Our series included 192 patients with NHL, diagnosed in a single institution between 1986 and 1998. In each patient the relationship among IPI, response to therapy and survival was investigated. RESULTS: The IPI turned out to be of prognostic value for response rate and survival in our unselected cohort of patients, as well. In each of the three WF classes separately (low, intermediate, high grade malignancy), the four IPI classes showed going from low to high risk substantially decreasing response rates and survival percentages. CONCLUSIONS: The IPI is confirmed as an important tool for prognostic evaluation of NHL patients: an integration of IPI, histological grading and serum beta 2-microglobulin concentration is supported.
Asunto(s)
Linfoma no Hodgkin/mortalidad , Índice de Severidad de la Enfermedad , Adulto , Factores de Edad , Anciano , Biomarcadores de Tumor/sangre , Estudios de Cohortes , Femenino , Humanos , L-Lactato Deshidrogenasa/sangre , Tablas de Vida , Linfoma no Hodgkin/sangre , Linfoma no Hodgkin/patología , Masculino , Persona de Mediana Edad , Pronóstico , Riesgo , Análisis de Supervivencia , Microglobulina beta-2/análisisRESUMEN
The aim of the present research was to verify the prognostic value of some histologic bone marrow parameters in chronic myeloproliferative disorders. Diagnostic bone marrow biopsies were revised in 38 patients with chronic myeloid leukaemia, 30 with polycythemia vera, 14 with essential thrombocythemia and 16 with idiopathic myelofibrosis-myeloid metaplasia. An unfavourable clinical evolution was associated to "granulocytic" histotype in chronic myeloid leukaemia, to "erythrocytic/granulocytic and/or megacaryocytic" histotype in polycythemia vera, to "cluster" distribution of megacaryocytes in essential thrombocythemia, to classical myelofibrosis without osteomyelosclerosis in myelofibrosis-myeloid metaplasia. Bone marrow biopsy in chronic myeloproliferative disorders provides independent diagnostic and prognostic data; we support its routine execution in this group of hematologic malignancies.