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2015 ; 94
(4
): e451
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Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features,
predictive factors, and prognosis in 21 patients
#MMPMID25634183
Bae CB
; Jung JY
; Kim HA
; Suh CH
Medicine (Baltimore)
2015[Jan]; 94
(4
): e451
PMID25634183
show ga
Hemophagocytic syndrome (HPS) is a potentially life-threatening complication of
systemic inflammatory disorders. Adult-onset Still disease (AOSD) is one of the
systemic autoimmune diseases associated with reactive hemophagocytic syndrome
(RHS). This study aimed to evaluate the characteristic findings, predictive
factors, and prognosis of RHS in patients with AOSD. We retrospectively evaluated
109 patients diagnosed with AOSD and reviewed their clinical data and laboratory
findings, including the biopsy results of 21 AOSD patients with RHS. Moreover,
data from 17 hemophagocytic lymphohistiocytosis (HLH) patients evaluated during
the same period were compared with those from the RHS patients. Twenty-one
patients (19.3%) developed RHS during the course of AOSD, and only 7 patients
(6.4%) were confirmed by bone marrow, liver, or lymph node biopsy. AOSD patients
with RHS showed significantly higher frequencies of splenomegaly, hepatomegaly,
and lymphadenopathy than did those without RHS. Moreover, patients with RHS
showed significantly higher relapse rates than those without RHS (61.9% vs 18.2%,
P < 0.001). Possible triggering factors inducing hemophagocytosis were detected
in 16 of 21 RHS patients (76.2%): disease flare in 12 patients (75%), infection
in 3 patients (18.8%), and drug use in 1 patient (6.3%). AOSD patients with RHS
showed higher frequencies of leukopenia, anemia, thrombocytopenia,
hypoalbuminemia, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, and
elevated lactate dehydrogenase levels than did those without RHS. Multivariate
logistic regression with forward selection procedure showed that low platelet
count (<121,000/mm³), anemia, and hepatomegaly were independent predictors of
RHS. Patients with definite RHS and those with probable RHS showed comparable
results. Although RHS is a life-threatening complication of AOSD, long-term
prognosis was observed to be similar in patients with and those without RHS.
Compared to RHS patients, HLH patients had poor prognosis, such as higher death
rates (52.9% vs 9.5%, P?=?0.005). RHS can be considered when an AOSD patient
shows at least 2 of the following 3 findings: low platelet count, anemia, and
hepatomegaly. Diagnostic confirmation by biopsy may not be essential if typical
clinical findings of RHS are present. Moreover, prognosis of RHS was better than
that of HLH diagnosed by the presence of trilineage cytopenia at admission.