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Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases.

Oto M, Yoshitsugu K, Uneda S, Nagamine M, Yoshida M - Hematol Rep (2015)

Bottom Line: 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).Adult-onset HLH has high diversity and various outcomes.The mechanism of adult-onset HLH is not fully understood and further research is required.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology and Medical Oncology, Japanese Red Cross Kumamoto Hospital , Japan.

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.

No MeSH data available.


Related in: MedlinePlus

Kaplan Meier estimates of Odds Ratio (OS). Tick marks show censored data. Median OS was not reached.
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fig001: Kaplan Meier estimates of Odds Ratio (OS). Tick marks show censored data. Median OS was not reached.

Mentions: After a median follow up of 1.9 months (range 0.1-132.5), 11 of the 34 patients (32.4%) had died. The causes of death were multi-organ failure (n=3), respiratory failure (n=3), septic shock (n=2), malignant tumor (n=1), liver failure (n=1) and cerebral hemorrhage (n=1). The survival curve is shown in Figure 1. Median OS of the entire population was not reached and median OS of the fatal cases was 1.2 month (range 0.1-25.5). We conducted survival analysis using patient baseline characteristics. In the univariable analysis, age at onset was a significant predictor of death (hazard ratio, 1.11; 95%CI, 1.03-1.19; P=0.007) (Table 3). Other baseline characteristics were not associated with prognosis.


Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases.

Oto M, Yoshitsugu K, Uneda S, Nagamine M, Yoshida M - Hematol Rep (2015)

Kaplan Meier estimates of Odds Ratio (OS). Tick marks show censored data. Median OS was not reached.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508551&req=5

fig001: Kaplan Meier estimates of Odds Ratio (OS). Tick marks show censored data. Median OS was not reached.
Mentions: After a median follow up of 1.9 months (range 0.1-132.5), 11 of the 34 patients (32.4%) had died. The causes of death were multi-organ failure (n=3), respiratory failure (n=3), septic shock (n=2), malignant tumor (n=1), liver failure (n=1) and cerebral hemorrhage (n=1). The survival curve is shown in Figure 1. Median OS of the entire population was not reached and median OS of the fatal cases was 1.2 month (range 0.1-25.5). We conducted survival analysis using patient baseline characteristics. In the univariable analysis, age at onset was a significant predictor of death (hazard ratio, 1.11; 95%CI, 1.03-1.19; P=0.007) (Table 3). Other baseline characteristics were not associated with prognosis.

Bottom Line: 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).Adult-onset HLH has high diversity and various outcomes.The mechanism of adult-onset HLH is not fully understood and further research is required.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology and Medical Oncology, Japanese Red Cross Kumamoto Hospital , Japan.

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.

No MeSH data available.


Related in: MedlinePlus