Abstract:Hemophagocytic lymphohistiocytosis (HLH) is classified into primary and secondary HLH according to the etiology.Primary HLH includes familial HLH and primary immunodeficiency HLH.Primary immunodeficie...Hemophagocytic lymphohistiocytosis (HLH) is classified into primary and secondary HLH according to the etiology.Primary HLH includes familial HLH and primary immunodeficiency HLH.Primary immunodeficiency HLH includes Chediak-higashi syndrome,Griscelli syndrome Ⅱ,Hermansky-Pudlak syndrome Ⅱ,X-linked lymphoproliferative syndrome I,X-linked lymphoproliferative syndrome Ⅱ and so on.These diseases may develop into HLH because of mutations in genes resulting in impaired function of natural killer cells and T cells,macrophage activation and extreme inflammation.The common clinical manifestations are repeated infection,prolonged fever,hepatosplenomegaly,cytopenia,hemophagocytosis in bone marrow,elevated ferritin,low fibrinogen and so on.The immunochemotherapy of dexamethasone,etoposide,cyclosporine A can be an effective remission-inducing treatment.Hematopoietic stem cell transplantation is the only known cure of the primary immunodeficiency HLH.
Key words:
Hemophagocytic lymphohistiocytosis; Primary immunodeficiency; Diagnosis; TherapyRead More
Publication Year: 2012
Publication Date: 2012-11-26
Language: en
Type: article
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