Cytokine Storm Syndrome by Randy Q. Cron & Edward M. Behrens

Author:Randy Q. Cron & Edward M. Behrens
Language: eng
Format: epub
ISBN: 9783030220945
Publisher: Springer International Publishing

Cytokine Storm Syndrome and Primary HIV Infection

CSS as a clinical syndrome has been associated with viral infections dating back to Risdall et al. in 1979 [4], and causes an aggressive and often fatal course. The syndrome is a consequence of profound cytokine secretion, macrophage activation and proliferation, leading to excessive phagocytosis. Case definitions have evolved since the initial description, and are characterized by fever, cytopenias (of at least 2 types), hypertriglyceridemia and/or hypofibrinogenemia, hyperferritinemia, hemophagocytosis, elevated CD25, decreased NK cell activity, splenomegaly, and hemophagocytosis in BM, LN, or spleen. Not coincidentally, there is significant overlap in the syndromes of acute retroviral syndrome and cytokine storm syndrome (Table 1). Fever, cytopenias, and splenomegaly/lymphadenopathy are cardinal features of ARS, and it is likely that CSS in the setting of primary HIV infection is underrecognized. Histological examination in CSS should reveal infiltration by histiocytes and lymphocytes with the presence of hemophagocytosis. Biopsy is not a routine component of clinical workup for ARS, although as documented below, when completed, biopsy was consistent with CSS in all reported cases [3, 5–13]. ARS is thought to be clinically evident in approximately 70% of individuals acutely infected with HIV. Gathering sufficient data for formal diagnosis of CSS is often difficult, particularly given the pace of presentation; thus, it is very likely that manifestations in HIV are underappreciated and may approach 20% and may be less severe in many cases.Table 1Cardinal features of ARS and CSS

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