Hemophagocytic Syndrome: A Suspect to Have in Mind
DOI:
https://doi.org/10.24950/rspmi.740Keywords:
Adrenal Cortex Hormones, Ferritins/blood, Lymphohistiocytosis, Hemophagocytic/diagnosis, Lymphohistiocytosis, Hemophagocytic/therapyAbstract
The hemophagocytic syndrome is a rare and potentially fatal
condition, characterized by uncontrolled immune system
activation, with symptoms and signs of extreme systemic inflammation.
As the manifestations are mostly unspecific, its
diagnosis requires a high degree of suspicion, in order to
implement adequate and timely therapy. The authors present
the case of a 19 year-old boy, previously healthy, with a suspected
lung infection due to a period of three weeks fever.
He presented a sudden increase of liver enzymes and slight
pancytopenia, skin rash and hepatosplenomegaly. The elevated
values of ferritin and interleukin-2 soluble receptor, as
well as the presence of hemophagocytosis in bone marrow,
confirmed the diagnosis of hemophagocytic syndrome. After
four weeks of dexamethasone, total resolution of symptoms
and laboratorial normalization were achieved.
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References
Hayden A, Park S, Giustini D, Lee AY, Chen LY. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review. Blood Rev. 2016;30:411-20.
Jordan MB, Allen CE, Weitzman S, Filipovich AH, Mcclain KL. How I treat How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118:4041–52.
Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood. 2016;125:2908–15.
Scott R, Robb-Smith A. Histiocytic medullary reticulosis. Lancet. 1939;234:194–8.
Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis. Arch Dis Child. 1952;27:519–25.
Rivière S, Galicier L, Coppo P, Marzac C, Aumont C, Lambotte O, et al. Reactive hemophagocytic syndrome in adults: A retrospective analysis of 162 patients. Am J Med. 2014;127:1118–25.
Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C. Infections associated with haemophagocytic syndrome. Lancet Infect Dis. 2007;7:814–22.
Maakaroun NR, Moanna A, Jacob JT, Albrecht H. Viral infections associated with haemophagocytic syndrome. Rev Med Virol. 2010;20:93–105.
Cascio A, Pernice LM, Barberi G, Delfino D, Biondo C, Beninati C, et al. Secondary hemophagocytic lymphohistiocytosis in zoonoses. A systematic review. Eur Rev Med Pharmacol Sci. 2012;16:1324–37.
Henter J, Horne A, Aricó M, Egeler R, Filipovich A, Imashuku S, et al. HLH-2004 : Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–31.
Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and validation of the hscore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66:2613–20.
Allen CE, Yu X, Kozinetz CA, McClain KL. Highly Elevated Ferritin Levels and the Diagnosis of Hemophagocytic Lymphohistiocytosis. Pediatr Blood Cancer. 2008;50:1227–35.
Schram AM, Campigotto F, Mullally A, Fogerty A, Massarotti E, Neuberg D, et al. Marked hyperferritinemia does not predict for HLH in the adult population. Blood. 2015;125:1548–52.
Ho C, Yao X, Tian L, Li FY, Podoltsev N, Xu ML. Marrow assessment for hemophagocytic lymphohistiocytosis demonstrates poor correlation with disease probability. Am J Clin Pathol. 2014;141:62–71.
Gupta A, Weitzman S. The role of hemophagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008;50:192–4.
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