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Re: Lymphopenia in lymphatic malformations

Hodge et al(1) draw our attention to the possible association of hypogammaglobulinemia and global lymphopenia with Proteus syndrome. They suggest that this may be secondary to loss of immunoglobulins and lymphocytes into lymphoedematous tissue. We have seen a similar phenomenon in a child with massive cystic hygroma in the neck. Immunological investigations showed persistent severe lymphopenia with low levels of all lymphocyte subsets. Lymphocyte proliferative responses to PHA were normal as were immunoglobulin levels and antibody responses to protein (diphtheria and tetanus toxoid) and polysaccharide (haemophilus b) vaccines. He initially suffered recurrent chest and skin infections and oral candidiasis but this responded well to treatment with prophylactic cotrimoxazole and nystatin mouthwashes. As in their case, we feel we may have been observing peripheral sequestration of circulating lymphocytes in this case and that, as a consequence, the clinical phenotype was milder than one would have expected in a child with similar results but caused by failure of lymphocyte production. We would like to extend their suggestion for immunological investigations in Proteus syndrome to other children with large lymphatic malformations.