RESUMO
Therapeutic plasma exchange (TPE) is commonly used in many neurological disorders where an immune etiology was known or suspected. We report our experience with TPE performed for neuroimmunologic disorders at four university hospitals. The study was a retrospective review of the medical records of neurological patients (n=57) consecutively treated with TPE between April 2006 and May 2007. TPE indications in neurological diseases included Guillain-Barrè Syndrome (GBS) (n=41), myasthenia gravis (MG) (n=11), acute disseminated encephalomyelitis (ADEM) (n=3), chronic inflammatory demyelinating polyneuropathy (CIDP) (n=1) and multiple sclerosis (MS) (n=1). Patient median age was 49; there was a predominance of males. Twenty-two patients had a history of other therapy including intravenous immunoglobulin (IVIG), steroid, azothioprin, and pridostigmine prior to TPE. Another 35 patients had not received any treatment prior to TPE. All patients were classified according to the Hughes functional grading scores pre- and first day post-TPE for early clinical evaluation of patients. The TPE was carried out 1-1.5 times at the predicted plasma volume every other day. Two hundred and ninety-four procedures were performed on 57 patients. The median number of TPE sessions per patient was five, and the median processed plasma volume was 3075mL for each cycle. Although the pre-TPE median Hughes score of all patients was 4, it had decreased to grade 1 after TPE. While the pre-TPE median Hughes score for GBS and MG patients was 4, post-TPE scores were decreased to grade 1. Additionally, there was a statistically significant difference between post-TPE Hughes score for GBS patients with TPE as front line therapy and patients receiving IVIG as front line therapy (1 vs. 3.5; p=0.034). Although there was no post-TPE improvement in Hughes scores in patients with ADEM and CIDP, patients with MS had an improved Hughes score from 4 to 1. Mild and manageable complications such as hypotension and hypocalcemia were also observed. TPE may be preferable for controlling symptoms of neuroimmunological disorders in early stage of the disease, especially with GBS.
Assuntos
Encefalomielite Aguda Disseminada/terapia , Síndrome de Guillain-Barré/terapia , Esclerose Múltipla/terapia , Miastenia Gravis/terapia , Troca Plasmática/métodos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Adolescente , Adulto , Idoso , Encefalomielite Aguda Disseminada/sangue , Feminino , Síndrome de Guillain-Barré/sangue , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/sangue , Miastenia Gravis/sangue , Plasmaferese/métodos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/sangue , Estudos Retrospectivos , Resultado do TratamentoRESUMO
May-Hegglin is a rare disease characterized by macrothrombocytopenia and presence of Döhle-like bodies in white cells. We present a patient treated with acute myeloid leukemia had pale-blue colored inclusion bodies assuming Döhle in his neutrophils.
RESUMO
We report four patients with rheumatic disease (RD) and chronic myelogenous leukemia (CML). In two patients with Behcet's disease (BD) and rheumatoid arthritis (RA), CML developed after RD, in two patients with diffuse cutaneous systemic sclerosis and spondyloarthropathy, RD was diagnosed after CML. A variety of interactions have been described between hematological malignancies and RD. Nevertheless, few cases of RD have been documented associated with CML. It is unclear whether the development of CML in patients with RD and RD development after CML occurs by chance alone, is due to the underlying disease, or is facilitated by drugs. Whatever the cause is, it should be kept in mind that CML may develop in the course of RD and RD may be seen in CML patients.
Assuntos
Síndrome de Behçet/etiologia , Leucemia Mielogênica Crônica BCR-ABL Positiva/etiologia , Doenças Reumáticas/etiologia , Adulto , Idoso , Feminino , Humanos , Hidroxiureia/uso terapêutico , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Pessoa de Meia-Idade , Doenças Reumáticas/diagnóstico , Escleroderma Sistêmico/etiologia , Espondilartrite/etiologiaRESUMO
Multiple myeloma (MM) is a neoplastic disorder characterized by monoclonal multiplying of plasma cells. Although radiation, environmental factors, viruses and other factors have been suggested as potential causes of the disease, the aetiopathogenesis of MM is still obscure. This clinical study was designed to investigate the role of reactive oxygen species (ROS) in the aetiopathogenesis of the disease and the possible relationships between treatment and ROS production. For this purpose, erythrocyte superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), and catalase (CAT) activities as well as plasma nitric oxide (NO) and malondialdehyde (MDA) levels were measured in 14 MM patients newly diagnosed at stage III. The relationship between the above-mentioned parameters and vincristine-adriamycin-dexamethasone (VAD) therapy were also investigated in the same patients. All the enzyme activities and the parameters of oxidative stress were found to be significantly reduced after VAD therapy. These findings suggest that ROS may be involved in the aetiopathogenesis of MM. Further investigations with a larger cohort of MM patients are needed to provide definitive data about the role of ROS in MM and the alternative therapeutic approaches to MM.