Presentation
We present the case of a 2-month-old girl who presented fever, anemia and thrombocytopenia, enlarged liver and spleen, hyperferritinemia, hypertriglyceridemia, and hypertransaminasemia without the finding of hemophagocytosis in bone marrow. [analesdepediatria.org]
Entire Body System
- Anemia
We present the case of a 2-month-old girl who presented fever, anemia and thrombocytopenia, enlarged liver and spleen, hyperferritinemia, hypertriglyceridemia, and hypertransaminasemia without the finding of hemophagocytosis in bone marrow. [analesdepediatria.org]
Caracteriza-se pela presença de febre persistente ou intermitente acompanhada de hepatoesplenomegalia (aumento do fígado e do baço), trombocitopenia e anemia, anormalidades da coagulação sanguínea (hipofibrinogenemia) e disfunção hepática.Afeta crianças [fr.dbpedia.org]
Liver, Gall & Pancreas
- Hepatosplenomegaly
[…] hemophagcytic syndrome, RHS, macrophage activation syndrome, MAS oder lymphohistiocytic syndrome (LHS), ist eine seltene, außerordentlich schwer verlaufende Erkrankung des Abwehrsystems (Immunsystem), welche durch hohes Fieber, Vergrößerung von Leber und Milz (Hepatosplenomegalie [fr.dbpedia.org]
Workup
Serum
- Cytopenia
Diagnostic guidelines include fever, splenomegaly, cytopenia, hypertriglyceridemia and/or hypofibrinogenemia with hemophagocytosis in the bone marrow, spleen or lymph nodes. [analesdepediatria.org]
Treatment
Alemtuzumab treatment for hemophagocytic lymphohistiocytosis. Nat Rev Clin Oncol. 2010;7(10). DOI 10.1038/nrclinonc.2010.40-c1. [ Links ] [scielo.org.co]
When there is strong clinical suspicion of FHL, chemotherapy and immunosuppressor treatment should be started early to achieve complete cure and should be followed by hematopoietic stem cell transplantation. [analesdepediatria.org]
Etiology
Two of the girl’s relatives had died of fulminant hepatic failure of unknown etiology. The patient improved spontaneously but presented reactivation of the disease 3 weeks later and died after splenic biopsy. [analesdepediatria.org]
Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69-86. DOI 10.2147/JBM.S46255. [ Links ] 46. Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. [scielo.org.co]