Abstract
Adult-onset hemophagocytic lymphohistiocytosis (HLH) has features that are distinct from that of HLH in pediatric patients. The clinical records at the Japanese Red Cross Kumamoto Hospital were reviewed. We retrospectively analyzed 34 patients who fulfilled the diagnostic criteria of HLH-2004. The median age of patients was 60.0 (range 15-86). Underlying diseases were diagnosed in 17 patients. They consisted of malignant lymphoma (n=3), other neoplastic disease (n=3), viral infection (n=4), collagen vascular disease (n=3), Kikuchi’s disease (n=3) and drug (n=1). Underlying diseases were not diagnosed in 17 patients despite examination. The treatments were steroids (n=18), dexamethasone + cyclosporine A (CSA) + etoposide (n=4), multidrug chemotherapy (n=2), steroids and CSA (n=3). Eleven patients died during observation. In a multivariate analysis, the significant predictor for death was age at onset (hazard ratio, 1.22; 95%CI, 1.02-1.44; P=0.027). Autopsy was performed in 4 cases, but the underlying disease remained unknown in 3 of those cases. Adult-onset HLH has high diversity and various outcomes. The mechanism of adult-onset HLH is not fully understood and further research is required.
Highlights
P-value of
Similar to the univariate underwent biopsy of bone marrow and skin shown that age at onset is the strongest proganalysis, the other baseline characteristics were with a suspicion of intravascular lymphoma (IVL) before death, but the nostic factor regardless of the etiology of HLH
The major findings of the autopsies A previous Japanese survey showed that the hand, the median age of the 23 patients who sur- were myelodysplastic syndrome (MDS) major etiology of adult-onset HLH is malignant vived was 39
Summary
The clinical records between November 2001 and September 2014 at the Japanese Red Cross Kumamoto Hospital were reviewed for the terms hemophagocytic syndrome and hemophagocytic lymphohistiocytosis. All adult patients aged 15 and over, whose medical record contained those words, were extracted. The underlying conditions of HLH were diagnosed in 17 patients (50.0%) (Table 2). The underlying diseases were neoplastic disease (n=6; 17.6%), viral infection (n=4; 11.8%), Kikuchi’s disease (n=3; 8.8%), collagen vascular disease (n=3; 8.8%) and drugs (n=1; 2.9%). Among the 4 not fully understood and further research is sion model was used to assess predictors of viral infection-associated HLH cases, the required. The cutoff value of laboratory data used in influenza virus was detected in 1 patient and the survival analysis was based on the diagnos- cytomegalovirus (CMV) was detected in 1 tic criteria in the HLH-2004. A diagnosed by a rapid antigen test and the CMV
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