Case Reports
doi: 10.1016/s0140-6736(78)91028-0.
Immunodeficiency in familial erythrophagocytic lymphohistiocytosis
- PMID: 76124
- DOI: 10.1016/s0140-6736(78)91028-0
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Case Reports
Immunodeficiency in familial erythrophagocytic lymphohistiocytosis
Lancet.
.
Abstract
4 children with familial erythrophagocytic lymphohistiocytosis and hyperlipidaemia were found to have a previously unrecognised immunological deficiency syndrome which included defects in both humoral and cellular immunity and a plasma inhibitor of in-vitro lymphocyte blastogenesis. The inhibitory activity was proportional to the increase in the triglyceride concentration in the patients' plasma. Immunological deficiency, to which hyperlipidaemia may be a contributing factor, appears to be a significant feature of familial erythrophagocytic lymphohistiocytosis.
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