Creput C, et al. Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. Intensive Care Med (2008) 34:1177-87.
This review discusses how HLH relies on the association of clinical abnormalities and hemophagocytosis in bone marrow, spleen, or lymph node specimens. Liver, pulmonary, renal, cardiac and skin involvement may occur at various degrees possibly leading to multiple organ failure. Three main etiologies can be found, namely infections, lymphoproliferative diseases, or connective tissue diseases. Immune deficiency is often retrieved. Mortality can be as high as 50%. Although clinically mimicking severe sepsis, HLH has a distinct pathophysiology on which specific therapy is based. Early diagnosis and treatment is mandatory to increase the chances of survival.
Table 1 lists the 8 criteria (must meet 5) for diagnosing HLH.
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