Reactive hemophagocytic syndrome in adult systemic disease: Report of twenty‐six cases and literature review

Abstract

Objective. To analyze specific clinical findings, underlying disorders, treatments, outcomes, and prognostic factors for reactive hemophagocytic syndrome (RHS) in systemic disease. Methods. Data were collected using standardized forms as part of a French national survey. Adult cases without an underlying malignancy, diagnosed on bone marrow or lymph node biopsy, were included. Results. Twenty-six cases (7 men, 19 women, mean age 47.4 ؎ 17.7 years) were studied. Systemic diseases included systemic lupus erythematosus (n ‫؍‬ 14), rheumatoid arthritis (n ‫؍‬ 2), adult onset systemic Still's disease (n ‫؍‬ 4), polyarteritis nodosa (n ‫؍‬ 2), mixed connective tissue disease (n ‫؍‬ 1), pulmonary sarcoidosis (n ‫؍‬ 1), systemic sclerosis (n ‫؍‬ 1), and Sjö gren's syndrome (n ‫؍‬ 1). RHS occurred in 2 distinct clinical settings in the course of systemic disease. RHS was associated with an active infection in 15 patients (bacterial infections, 10 cases; viral, 3 cases; tuberculosis, 1 case; and aspergillosis, 1 case) and with the onset of a systemic disease alone in 9 cases. Isolated RHS occurred in 2 cases. The overall mortality rate was 38.5%. Two factors were associated with mortality: corticosteroid treatment at the time of RHS diagnosis, and thrombocytopenia (odds ratio ‫؍‬ 28, 95% confidence interval ‫؍‬ 13.3؊238.9). Conclusions. When RHS occurs in the course of an active systemic disease (situation only reported in cases of systemic lupus or adult Still's disease), immunosuppressive therapy should be used. In contrast, when RHS is present concomitantly with an active infection, immunosuppressive therapy needs to be lowered and antibiotic therapy should be instituted.

Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

“…The prevalence of MAS was 1.4% among all patients followed at our center, and was similar to that reported in the literature in adult SLE patients (3)(4)(5)(6). In our experience, in all cases MAS was diagnosed in untreated SLE patients with active disease.…”

“…Although in our experience, MAS and SLE were diagnosed simultaneously and this was also the most frequent scenario in some other previous series (4,8), recent data indicate that MAS may arise in patients with long disease duration in up to 50% of the cases (7). 3 and/or a platelet count of <110,000/mm 3 ; °the cut-off suggested for the classification of reactive MAS in the original study was 169 (11). Other authors suggested a higher cutoff (190.5), in patients with rheumatic diseases (15).…”

“…The first cases were described in 1991 as acute lupus hemophagocytic syndrome (2). Afterwards, several small case series were described, with an estimated prevalence of MAS among SLE patients ranging from 0.9% to 4.6% (3)(4)(5)(6)(7)(8). To our knowledge, only one paper described adult SLE-associated MAS cases collected in Italy (9).…”

Macrophage activation syndrome and other systemic inflammatory conditions after BMT

“…1,3 Hemophagocytic lymphohistiocytosis-like episodes were also described in association with systemic lupus erythematosus, rheumatoid arthritis, polyarteritis nodosa, mixed connective tissue disease and others. 18,19 These patients may be considered as having 'secondary or acquired' HLH, commonly referred to as MAS in the rheumatologic literature. Macrophage activation syndrome shares many clinical/ laboratory features with HLH, and several authors classify MAS as 'secondary' or 'acquired' hemophagocytic syndrome.…”

Adult-onset Still’s disease

“…It is important to bear in mind that AOSD may begin with an HS [42]. Furthermore, malignant lymphoma should not only be excluded for diagnosis, but they may be added to the large variety of complications associated with this disease [43].…”