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Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder of cytotoxic cell function that results in abnormal proliferation of benign lymphocytes and histiocytes in response to infectious stimuli. FHLH generally occurs in very young children, and typically presents with fever, cytopenias, coagulopathy, lymphadenopathy, and hepatosplenomegaly. Central nervous system involvement occurs frequently and may precede the development of systemic symptoms by months to years. We report a case of an 18-year-old male with a 2-year history of symptoms attributed to a demyelinating disorder, who succumbed to rapidly progressive hemophagocyte lymphohistiocytosis. Post-mortem, two distinct perforin mutations were identified. We discuss the central nervous system and genetic findings in this unusual presentation of familial hemophagocytic lymphohistiocytosis.

Fish oil supplements are popular alternative medicines. Many manufacturers label their products with the warning "avoid this product if you are allergic to fish." The objective of this study was to determine if finned fish (FF)-allergic patients could safely tolerate fish oil supplements. Six FF-sensitive subjects as determined by history and skin testing were selected. They were skin tested with two different fish oil supplements and given an oral challenge of each supplement 1 hour apart. Vital signs were measured at baseline and at 20-minute intervals after each challenge. Spirometry was measured at baseline and 1 hour after each challenge. Six of six patients with positive skin tests to at least one FF had negative skin tests to both fish oil supplements. All six subjects then had negative oral challenges to both supplements. In this pilot study, FF-sensitive patients tolerated fish oil supplements.

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