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.

Summary. Systemic mastocytosis (SM) is characterized by proliferation of mast cells in various organs, which may release a wide variety of mediators, thereby explaining the broad clinical spectrum of disease manifestations. The potentially life-threatening systemic symptoms and tumoral proliferation are poorly controlled despite the use of several cytotoxic chemotherapies and/or symptomatic treatments. Twenty consecutive adult SM patients with histologically confirmed bone marrow (BM) involvement received interferon-a subcutaneously (1-5 million units/m 2 /d, with progressive dose intensification over the first month of treatment) and were evaluated after 6 months of therapy. Seven of them had previously received symptomatic treatments, including steroids, which were ineffective. Among the 13 patients treated for at least 6 months, seven partial and six minor responses, mainly concerning vascular congestion and skin lesions, were obtained, while BM infiltration remained unchanged in 12 patients. The significant reduction of mast-cell mediator levels after 6 months of treatment was not predictive of clinical remission. The rate of depression was unexpectedly high (seven patients; 35%). Two patients died soon after starting therapy (one myocardial infarction, one septic shock). Six months of interferon-a may relieve vascular congestion in adults with SM, probably by inhibiting mast-cell degranulation.

We report on the first case of documented Helicobacter cinaedi septic arthritis in an immunocompetent heterosexual young man. The patient presented no identified risk factor except for contact with animals that have been incriminated as a possible source of infection, particularly for these patients. Despite prolonged bacteremia, the response to long-term therapy with ciprofloxacin and rifampin was excellent.

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