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Histiocitose das células de Langerhans e síndrome hemofagocítica : natural killers cells (caso clínico)
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Descreve-se uma situação clínica em idade pediátrica, caracterizada por um quadro defebre, coriza, tosse produtiva e adenomegália esquerda, com agravamento progressivo associado a pancitopenia, alterações hepáticas e da ferritina. Discute-se a etiopatogenia, diagnóstico diferencial, terapêutica e prognóstico perante um doente com esta presentação.
A Histiocitose de Células de Langerhans (HCL) ou a Síndrome Hemofagocítica/Linfohistiocitose Hemofagocítica (SH/LHH) são quadros clínicos semelhantes, com diagnósticos diferenciais variados,dificultando a terapêutica a instituir.
Mais rara é a associação da HCLàSH/LHH, sendo que o diagnóstico definitivo destas duas entidades pode ser fornecido pela avaliação anatomopatológica, exame imunohistológico, citológico e o mielograma. Ambas são rapidamente progressivas e habitualmente fatais, caracterizando-se por pancitopénias graves, insuficiência hepática, alterações da ferritina, coagulopatia e diferentes sintomas neurológicos. A principal característica no primeiro caso consiste numa acumulação de células de Langerhans modificadas em vários órgão e tecidos, promovendo lesão e necrose exagerada, já no segundo estamos perantea acumulação de macrófagos em diferentes órgãos e tecidos.
Levanta-se também a hipótese de a patogenia das células Natural Killer ser um impulsor da gravidade desta doença e um possível foco de futura investigação.
A sua importância prende-se com o facto de muitas vezes estarem associados a reactividade a infecções e/ou à existência de processos neoplásicos, levando inevitavelmente à morte por falência multiorgânica.
It is described a condition in children, characterized by fever, runny nose, productive cough and left adenomegaly, with progressive deterioration associated with pancytopenia, liver and ferritinchanges. It is discussed the etiology, differential diagnosis, therapy and prognosis standing before a patient with this presentation. The Langerhans CellsHistiocytosis (LCH) or Hemophagocytic Syndrome/Hemophagocytic Lymphohistiocytosis (HS/HLH) are similar clinical diseases, varying differential diagnoses, difficult the therapy to be imposed. More rare is the association of LCH to HS/HLH, but the definitive diagnosis of these two entities can be provided by pathologic evaluation, imunohistologic examination, cytology and myelogram. Both are rapidly progressive and usually fatal and is characterized by severe pancytopenia, liver failure, ferritin changes, coagulopathy and different neurological symptoms. The main characteristic in the first case is a Langerhans modifiedcell accumulation in various tissues and body, promoting necrosis injury and exaggerated, and the second is the accumulation of macrophages in different organs and tissues. It also raises the hypothesis of the pathogenesis of natural killer cells as an impeller of the worsening of this disease and a possible focus for future research. Its importance concerns the fact that they often are associated with reactivity to infection and/or to the existence of neoplasic processes, inevitably leading to death from multiorgan failure.
It is described a condition in children, characterized by fever, runny nose, productive cough and left adenomegaly, with progressive deterioration associated with pancytopenia, liver and ferritinchanges. It is discussed the etiology, differential diagnosis, therapy and prognosis standing before a patient with this presentation. The Langerhans CellsHistiocytosis (LCH) or Hemophagocytic Syndrome/Hemophagocytic Lymphohistiocytosis (HS/HLH) are similar clinical diseases, varying differential diagnoses, difficult the therapy to be imposed. More rare is the association of LCH to HS/HLH, but the definitive diagnosis of these two entities can be provided by pathologic evaluation, imunohistologic examination, cytology and myelogram. Both are rapidly progressive and usually fatal and is characterized by severe pancytopenia, liver failure, ferritin changes, coagulopathy and different neurological symptoms. The main characteristic in the first case is a Langerhans modifiedcell accumulation in various tissues and body, promoting necrosis injury and exaggerated, and the second is the accumulation of macrophages in different organs and tissues. It also raises the hypothesis of the pathogenesis of natural killer cells as an impeller of the worsening of this disease and a possible focus for future research. Its importance concerns the fact that they often are associated with reactivity to infection and/or to the existence of neoplasic processes, inevitably leading to death from multiorgan failure.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2015
Palavras-chave
Linfo-histiocitose hemofagocítica Células de Langerhans Células Natural Killer Pediatria