Background/Purpose:
Macrophage activation syndrome (MAS) is a
group of diseases, especially autoimmune (AI) and hemato-oncology (HO). So it
will be interesting to find any clinical or analytical data to differentiate
both.

Methods:
Describe and compare demographics, clinical
and laboratories features, mortality during hospital stay and after the
discharge of patients diagnosed with MAS secondary HO and AI diseases in the
period December 2008 to January 2015, in Donostia
University Hospital. A cohort of patients
diagnosed with MAS was studied by reviewing medical records. We analyzed and compared
only patients with AI and HO disease. The variable were diagnosis, sex, age,
fever, organomegaly, hospital mortality and overall mortality (from diagnosis
to the end of the study), analytical findings, hospital stay in days, days from
admission to diagnosis and days from diagnosis to the end of the study (January
21, 2015) or death after discharge. Quantitative variables are shown with the
median and interquartile range. For the bivariate analysis Wilcoxon and Chi square
test was used. Survival in months and relative risk (RR) was calculated with
the Kaplan Meier plot.

Results: 19 patients with different etiologies MAS were found, 6 and 9 were due to AI and HO diseases. Table 1 shows the etiologic diagnosis of SAM due to AI and HO diseases.
Table 2 shows the descriptive analysis
of 19 patients and the bivariate analysis between AI and HO group. The median survival of HO disease group was 1.2
months and AI disease group cannot be calculated. The mortality oh
HO and AI disease
was 77.8% and
16.7% respectively with a p equal to 0.04 by the log rank test. The RR of death among the group
of HO against AI disease was 6.84.

Conclusion: In the present
study the following statistically significant differences were found:

1) Patients with MAS due AI diseases have higher elevated liver
enzymes compared to HO disease.

2) Patients with MAS due HO diseases have a greater pancytopenia
compared to AI diseases, especially in leukocytes and neutrophils.

3) Patients with MAS due HO have a higher mortality and lower
median survival after diagnosis compared to AI diseases.