RESUMO
LGL leukemia is a rare chronic lymphoproliferative disorder of cytotoxic lymphocytes which can be immunophenotypically either T cell or NK cell-derived. According to the World Health Organization classification, it can be divided into three subtypes: chronic T-cell leukemia and chronic natural killer cell lymphocytosis, and aggressive natural killer cell LGL leukemia. Clonal proliferation of large granular lymphocytes can be because of stimulation of various molecular pathways namely JAK-STAT3 pathway, FAS/FAS-L pathway, RAS-RAF-1-MEK1-ERK pathway, PI3K/AKT pathway, NF-KB pathway, and Sphingolipid Rheostat pathways. The most common clinical features presenting with this leukemia are neutropenia, anemia, thrombocytopenia. This leukemia is also associated with various autoimmune conditions. It usually has an indolent course except for the aggressive NK cell LGL leukemia. The cause of death in the indolent cases was mostly due to infectious complications related to the neutropenia associated with the disease. The rarity of the disease coupled with the availability of only a handful of clinical trials has been a hindrance to the development of a specific treatment. Most of the cases are managed with immunomodulators. The advances in the knowledge of molecular pathways associated with the disease have brought few targeted therapies into the limelight. We discuss here the evolution, epidemiology, demographic profile, pathophysiology, differential diagnosis, the available treatment options along with the survival and prognostic variables which may help us in better understanding and better management of the disease and hopefully, paving the way for a targeted clinical approach.
Assuntos
Artefatos , Sangue , Colelitíase/complicações , Colelitíase/diagnóstico por imagem , Icterícia Obstrutiva/diagnóstico por imagem , Manejo de Espécimes/normas , Células Sanguíneas , Humanos , Malária Falciparum/diagnóstico , Masculino , Plasmodium falciparum , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
The diagnosis of systemic lupus erythematosus (SLE) has undergone radical change after the development of serological techniques. The in vitro demonstration of lupus erythematosus (LE) cell has less significance for the diagnosis of SLE in the present scenario. Although over the years, the spontaneous in vivo occurrence of LE cell in numerous body fluids as an initial presentation of SLE has been documented. The report of the presence of the LE cell can not only aid in the further workup of the patient but also suggest the involvement of a particular organ or body cavities by SLE. The morphology and mimickers of the LE cell should be cogitated and meticulous search of such cells should play an important role in the evaluation of body fluids. In our case, the patient presented at emergency with pericardial tamponade and cytological evaluation of the pericardial fluid demonstrated in vivo presence of LE cells. The serological work-up then confirmed the case to be SLE. This report and review of literature wish to highlight the fact that this cell still plays a significant role even in the era of immunoassays.
Assuntos
Citodiagnóstico/métodos , Lúpus Eritematoso Sistêmico/diagnóstico , Derrame Pericárdico/citologia , Adulto , Feminino , Humanos , Derrame Pericárdico/patologiaRESUMO
Malaria, an important parasitic disease worldwide, still has diagnostic challenges in the laboratory. Many studies have been conducted on the detection ability of haematology analysers for malaria. We evaluated the Sysmex XN-series analyser as a tool for detection of malaria by analysing the leukocyte cell population data (LCPD), scattergrams and associated Flow Cytometry Standard (FCS) data from both the WNR (white cell nucleated) and WDF (white cell differential) channels. 1281 clinically suspected cases of malaria were screened for malaria by peripheral blood smear examination and were run in the Sysmex XN-1000 for analysis of haematological parameter data, LCPD, all the scattergrams and FCS data. 1281 clinically suspected cases of malaria were screened for malaria by peripheral blood smear examination and were run in the Sysmex XN-1000 for analysis of haematological parameter data, LCPD, all the scattergrams and FCS data. 48 cases had malarial parasite on microscopy; of which, 41 cases were of Plasmodium vivax, 6 cases of Plasmodium falciparum and 1 case of mixed infection. 46 malaria-positive samples showed certain patterns of clusters in the scattergrams of both WDF and WNR channels. A case with only a few ring forms of P. vivax and another with very low parasite load having only gametocyte of P. falciparum didn't show the distinctive cluster. The most distinctive clusters for all other cases were seen in WNR (SFL-SSC) and WNR (SSC-FSC) scattergrams. FCS data for the same were analysed to gate for those events. The gated events correlated (Spearman ρ = 0.77, p < 0.01) with the parasite load of the patients. By observing the scattergrams and different parameters in the Sysmex XN-series analyser, malaria can be detected from the analyser itself.