Contributions
Abstract: S1583
Type: Oral Presentation
Presentation during EHA23: On Sunday, June 17, 2018 from 08:45 - 09:00
Location: Room A8
Background
Hemophagocytic syndrome (HLH, hemophagocytic lymphohistiocytosis) is a rare syndrome of fatal hyperinflammation, where cytokine storm leads to bone marrow failure and death. Historically it was mostly diagnosed in pediatric population, but with raising awareness data form adults are emerging. Little is known about prognostic factors in HLH, especially in adult patients.
Aims
Aim of this study was search for prognostic factors in one of the largest cohorts of European adult HLH patients.
Methods
Data of 86 adult (≥ 18 years of age) patients form the HLH in Adults Database affiliated with PALG (Polish Adult Leukemia Group) were retrospectively analyzed. Risk factor analysis was made by Cox regression. Factors were analyzed in univariable analysis, and factors with p < 0.06 and n > 82 were retained in the multivariable model. Statistica 13 and MedCalc 18 softwares were used.
Results
A slight male predominance (62%; 53/86) was observed in the analyzed group. Median age was 38 (18-82). Median survival reached 144 days, 35% of patients died within one month. Among 29 patients who survived over one year, only two died (both due to HLH relapse). Longest observation exceeded 10 years.
There were 34 patients with malignancy-associated HLH, 20 with infection-associated HLH, 14 with HLH associated with autoimmune disease (MAS syndrome; Macrophage Activation Syndrome). In 18 patients triggering factor could not be determined.
In univariate analysis (Table 1) MAS syndrome, red blood cell count (RBC) and hypertriglyceridemia (>265mg/dl – HLH-2004 criterion cutoff) were established as positive prognostic factors. Additionally, a trend for hepatomegaly was observed (p=0.057). Interestingly, antithrombin III and ESR were also significant in univariate analysis, but low number of available results precluded them from being tested in multivariate analysis. Among four analyzed parameters in multivariate analysis only MAS and hepatomegaly retained the prognostic factor status (Table 1).
Conclusion
Hemophagocytic syndrome in adults confers a poor prognosis, but patients who survive the first critical period may expect survival with a low relapse risk. MAS syndrome and hepatomegaly may be associated with a relatively better prognosis in newly-diagnosed adult HLH patients.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Abstract: S1583
Type: Oral Presentation
Presentation during EHA23: On Sunday, June 17, 2018 from 08:45 - 09:00
Location: Room A8
Background
Hemophagocytic syndrome (HLH, hemophagocytic lymphohistiocytosis) is a rare syndrome of fatal hyperinflammation, where cytokine storm leads to bone marrow failure and death. Historically it was mostly diagnosed in pediatric population, but with raising awareness data form adults are emerging. Little is known about prognostic factors in HLH, especially in adult patients.
Aims
Aim of this study was search for prognostic factors in one of the largest cohorts of European adult HLH patients.
Methods
Data of 86 adult (≥ 18 years of age) patients form the HLH in Adults Database affiliated with PALG (Polish Adult Leukemia Group) were retrospectively analyzed. Risk factor analysis was made by Cox regression. Factors were analyzed in univariable analysis, and factors with p < 0.06 and n > 82 were retained in the multivariable model. Statistica 13 and MedCalc 18 softwares were used.
Results
A slight male predominance (62%; 53/86) was observed in the analyzed group. Median age was 38 (18-82). Median survival reached 144 days, 35% of patients died within one month. Among 29 patients who survived over one year, only two died (both due to HLH relapse). Longest observation exceeded 10 years.
There were 34 patients with malignancy-associated HLH, 20 with infection-associated HLH, 14 with HLH associated with autoimmune disease (MAS syndrome; Macrophage Activation Syndrome). In 18 patients triggering factor could not be determined.
In univariate analysis (Table 1) MAS syndrome, red blood cell count (RBC) and hypertriglyceridemia (>265mg/dl – HLH-2004 criterion cutoff) were established as positive prognostic factors. Additionally, a trend for hepatomegaly was observed (p=0.057). Interestingly, antithrombin III and ESR were also significant in univariate analysis, but low number of available results precluded them from being tested in multivariate analysis. Among four analyzed parameters in multivariate analysis only MAS and hepatomegaly retained the prognostic factor status (Table 1).
Conclusion
Hemophagocytic syndrome in adults confers a poor prognosis, but patients who survive the first critical period may expect survival with a low relapse risk. MAS syndrome and hepatomegaly may be associated with a relatively better prognosis in newly-diagnosed adult HLH patients.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical