Table 1: Etiologies and mortality of each subgroup of patients with Hemophagocytic Syndrome.
Etiologies |
n = 30 |
Mortality during the admission+ |
Mortality after discharge++ |
Autoimmune diseases |
n = 10 |
n = 1 |
n = 0 |
Systemic Lupus Erythematosus (SLE) |
5 |
1 |
0 |
Adult Still's Disease (ASD) |
3 |
0 |
0 |
Rheumatoid arthritis |
1 |
0 |
0 |
IgG4 Sclerosing Disease |
1 |
0 |
0 |
Hematological Malignancies Diseases |
n = 12 |
n = 8* |
n = 3 |
Non-Hodgkin's Lymphoma |
3 |
1 |
1 |
Myelodysplastic síndrome |
3 |
2 |
1 |
Acute leukemia |
3 |
3 |
- |
Lymphoma extranodal NK cell |
1 |
1 |
- |
Multiple Myeloma |
1 |
0 |
1 |
Probably lymphoproliferative disease |
1 |
1 |
- |
Infectious diseases |
n = 2 |
n = 1 |
n = 0 |
Pneumocystis jirovencii in a patient with HIV |
1 |
1 |
0 |
Gastroenteritis by Campylobacter jejuni |
1 |
0 |
0 |
Malignant Solid tumor disease |
n = 1 |
n = 0 |
n = 1 |
Glioblastoma multiforme (GM) with temozolomide |
1 |
0 |
1 |
HPS without defined etiology |
n = 5 |
3 |
n = 0 |
HIV: Human Immunodeficiency Virus.
NK: Natural Killer.
+Mortality secondary to HPS.
++Mortality secondary to primary etiology.
*p = 0.029.