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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

Distribution of ages according to patient outcomes. Boxes contain 50% of data with the inside horizontal line representing the median value.
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fig002: Distribution of ages according to patient outcomes. Boxes contain 50% of data with the inside horizontal line representing the median value.

Mentions: We conducted multivariable analysis with variables used in the univariate analysis. The multivariate Cox regression analysis showed that age at onset was a significant predictor of death (hazard ratio, 1.22; 95%CI, 1.02-1.44; P=0.027) (Table 3). Similar to the univariate analysis, the other baseline characteristics were not associated with prognosis. Figure 2 shows distribution of ages according to the outcomes of the patients. The median age of the 11 fatal patients was 75 (range 53-83). On the other hand, the median age of the 23 patients who survived was 39 (range 15-86). The survivors constituted a younger populations among all cases.


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)

Distribution of ages according to patient outcomes. Boxes contain 50% of data with the inside horizontal line representing the median value.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig002: Distribution of ages according to patient outcomes. Boxes contain 50% of data with the inside horizontal line representing the median value.
Mentions: We conducted multivariable analysis with variables used in the univariate analysis. The multivariate Cox regression analysis showed that age at onset was a significant predictor of death (hazard ratio, 1.22; 95%CI, 1.02-1.44; P=0.027) (Table 3). Similar to the univariate analysis, the other baseline characteristics were not associated with prognosis. Figure 2 shows distribution of ages according to the outcomes of the patients. The median age of the 11 fatal patients was 75 (range 53-83). On the other hand, the median age of the 23 patients who survived was 39 (range 15-86). The survivors constituted a younger populations among all cases.

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