SUMMARY In vitro and in vivo lymphocyte function was studied in six patients with primary hypogammaglobulinaemia and nodular lymphoid hyperplasia (NLH) of the bowel. Lymphocyte transformation, numbers of circulating T Nodular lymphoid hyperplasia (NLH) of the bowel is a condition virtually confined to patients with primary immunodeficiency. It is extremely rare in children and is generally a complication affecting adults with primary common variable late onset hypogammaglobulinaemia. Six patients were found to have NLH in the course of screening for gastrointestinal complications and we have analysed in vitro and in vivo lymphocyte function in these patients to establish whether they have any characteristics in common. In addition, we have attempted to identify the cell types within the nodules.
Methods
SUBJECTSThirty-four patients with primary hypogammaglobulinaemia were screened for NLH. They were selected on the basis of their willingness to be inpatients and there was no tendency for patients with gastrointestinal complaints to be referred. Their ages at the time of investigation ranged from 7 to 63 years (mean 33 years). Seventeen patients were receiving regular weekly intramuscular gammaglobulin at a dose of 25-50 mg/kg body weight.
Received for publication 2 March 1977Twenty-eight patients had grossly depressed levels of serum IgG-that is, <50 IU/ml-mean = 29 IU/mi) and only six patients had levels above 50 IU/ml. Serum IgA concentrations were unrecordable in 29 patients-that is, <3 5 IU/mland only one patient had a level within the normal limits. Twenty-eight patients had serum IgM concentrations below 47 IU/ml (mean = 12).
A patient with a 15-year history of urticarial vasculitic rashes and chronic vasculitic skin ulceration has been followed at our Connective Tissue Disease Clinic for the past five years. Serum complement levels have been persistently normal. She has been unresponsive to a variety of medications including steroids, dapsone and hydroxychloroquine. She had a dramatic response to colchicine 500 micrograms daily with total clearing of the urticarial vasculitic rash.
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