Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review
DOI:
https://doi.org/10.17921/2447-8938.2019v21n4p422-8Resumo
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare, usually fatal and underdiagnosed autoimmune-activated disease. The present study aimed to perform a macroscopic, histopathological and immunohistochemical evaluation for CD68 and CD57 in organs of autopsied adults with HLH. A total of 604 autopsy reports were analyzed, and all the patients that filled the diagnostic criteria for HLH (n = 2) were selected. These patients were 18 and 37 years old. Were evaluated both clinical and autopsy reports and performed histopathological and immunohistochemical analysis of the liver and spleen. Both patients filled the diagnostic criteria for HLH, as well as presented common signs and symptoms of this disease, such as chills, abdominal pain, diaphoresis, and jaundice. Hemophagocytosis was observed in the spleen, bone marrow, and lymph nodes of the two patients at autopsy. Immunostaining in the liver and spleen of both patients was mainly severe for CD68, and predominantly mild for CD57, indicating a decrease in NKC numbers and an increase in the number of macrophages, respectively. This was the first study to evaluate CD57 and CD68 in autopsies of adults with HLH. Thus, more studies are required, not only to better elucidate the pathogenetic mechanisms involved in the secondary HLH, but also to disseminate the results in the clinical environment, contributing to the early diagnosis and treatment with consequent reduction of mortality rate.
Keywords: Autoimmune Diseases. Histiocytosis. Biomarkers.
Resumo
A Linfohistiocitose Hemofagocítica (HLH) é uma doença autoimune rara, geralmente fatal e subdiagnosticada. Este estudo tem como objetivo realizar avaliação macroscópica, histopatológica e imunohistoquímica para CD68 e CD57 em órgãos de pacientes adultos com HLH submetidos a autópsia. Um total de 604 laudos de autópsias foram analisados e todos os pacientes que preencheram os critérios diagnósticos para HLH (n = 2) foram selecionados. Esses pacientes tinham 18 e 37 anos de idade. Foram analisados tanto os prontuários quanto os laudos de autópsia, bem como foram realizadas análises histopatológicas e imunohistoquímicas do fígado e baço dos pacientes. Ambos preencheram os critérios diagnósticos para HLH e apresentarem sinais e sintomas comuns da doença, como calafrios, dor abdominal, sudorese e icterícia. A hemofagocitose foi observada no baço, medula óssea e linfonodos dos dois pacientes na autópsia. A imunohistoquímica do fígado e do baço de ambos os pacientes demonstrou imunomarcação acentuada para CD68 e predominantemente discreta para CD57, que indicam diminuição do número de NKC e aumento do número de macrófagos, respectivamente. Este foi o primeiro estudo a avaliar o CD57 e CD68 em autópsias de adultos com HLH. Assim, mais estudos são necessários, não apenas para melhor elucidar os mecanismos patogenéticos envolvidos na HLH secundária, mas também para disseminar os resultados no ambiente clínico, contribuindo para o diagnóstico e tratamento precoces com consequente redução da taxa de mortalidade.
Palavras-chave: Doenças Autoimunes. Histiocitose. Biomarcadores.
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