RESUMO
Abroad array of conditions can lead to neurological symptoms in chronic lymphocytic leukemia patients and distinguishing between clinically significant involvement of the central nervous system by chronic lymphocytic leukemia and symptoms due to other etiologies can be challenging. Between January 1999 and November 2014, 172 (4%) of the 4174 patients with chronic lymphocytic leukemia followed at our center had a magnetic resonance imaging of the central nervous system and/or a lumbar puncture to evaluate neurological symptoms. After comprehensive evaluation, the etiology of neurological symptoms was: central nervous system chronic lymphocytic leukemia in 18 patients (10% evaluated by imaging and/or lumbar puncture, 0.4% overall cohort); central nervous system Richter Syndrome in 15 (9% evaluated, 0.3% overall); infection in 40 (23% evaluated, 1% overall); autoimmune/inflammatory conditions in 28 (16% evaluated, 0.7% overall); other cancer in 8 (5% evaluated, 0.2% overall); and another etiology in 63 (37% evaluated, 1.5% overall). Although the sensitivity of cerebrospinal fluid analysis to detect central nervous system disease was 89%, the specificity was only 42% due to the frequent presence of leukemic cells in the cerebrospinal fluid in other conditions. No parameter on cerebrospinal fluid analysis (e.g. total nucleated cells, total lymphocyte count, chronic lymphocytic leukemia cell percentage) were able to offer a reliable discrimination between patients whose neurological symptoms were due to clinically significant central nervous system involvement by chronic lymphocytic leukemia and another etiology. Median overall survival among patients with clinically significant central nervous system chronic lymphocytic leukemia and Richter syndrome was 12 and 11 months, respectively. In conclusion, clinically significant central nervous system involvement by chronic lymphocytic leukemia is a rare condition, and neurological symptoms in patients with chronic lymphocytic leukemia are due to other etiologies in approximately 80% of cases. Analysis of the cerebrospinal fluid has high sensitivity but limited specificity to distinguish clinically significant chronic lymphocytic leukemia involvement from other etiologies.
Assuntos
Biomarcadores Tumorais/líquido cefalorraquidiano , Neoplasias do Sistema Nervoso Central/diagnóstico , Sistema Nervoso Central/metabolismo , Síndrome de Hermanski-Pudlak/diagnóstico , Síndromes de Imunodeficiência/diagnóstico , Leucemia Linfocítica Crônica de Células B/diagnóstico , ADP-Ribosil Ciclase 1/líquido cefalorraquidiano , ADP-Ribosil Ciclase 1/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Contagem de Células , Sistema Nervoso Central/patologia , Neoplasias do Sistema Nervoso Central/líquido cefalorraquidiano , Neoplasias do Sistema Nervoso Central/genética , Neoplasias do Sistema Nervoso Central/mortalidade , Diagnóstico Diferencial , Feminino , Citometria de Fluxo , Síndrome de Hermanski-Pudlak/líquido cefalorraquidiano , Síndrome de Hermanski-Pudlak/genética , Síndrome de Hermanski-Pudlak/mortalidade , Humanos , Cadeias Pesadas de Imunoglobulinas/líquido cefalorraquidiano , Cadeias Pesadas de Imunoglobulinas/genética , Síndromes de Imunodeficiência/líquido cefalorraquidiano , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/mortalidade , Integrina alfa4/líquido cefalorraquidiano , Integrina alfa4/genética , Leucemia Linfocítica Crônica de Células B/líquido cefalorraquidiano , Leucemia Linfocítica Crônica de Células B/genética , Leucemia Linfocítica Crônica de Células B/mortalidade , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Doenças da Imunodeficiência Primária , Estudos Retrospectivos , Punção Espinal , Análise de Sobrevida , Proteína-Tirosina Quinase ZAP-70/líquido cefalorraquidiano , Proteína-Tirosina Quinase ZAP-70/genéticaRESUMO
Human immunodeficiency virus (HIV) could be isolated from the cerebrospinal fluid (CSF) of the majority (62%) of 72 patients in various stages of HIV infection. This high rate of successful virus isolation was achieved only when the time from lumbar puncture to initiation of the cell cultures was short, i.e. not exceeding 5 h. The HIV isolation rates were equally high in patients with persistent generalized lymphadenopathy (PGL), AIDS-related complex (ARC) and AIDS. Although the HIV recovery rate was low in patients with normal immunological parameters it was not correlated with the degree of severity of the immunodeficiency in the other patients. Furthermore, the recovery rates were not significantly correlated to the duration of the infection. HIV was recovered as often from patients with neurological symptoms as from patients without such symptoms. These findings suggest that in the majority of patients there is central nervous system (CNS) involvement early in the course of HIV infection and that HIV replication in the CNS may occur in the absence of a pronounced systemic cellular immunodeficiency and frequently without causing overt neurological symptoms.
