Cellular immunodeficiency in protein-losing enteropathy

Abstract

Cellular immunological abnormalities were studied in a patient with protein-losing enteropathy associated with constrictive pericarditis. Analysis of lymphocyte subpopulations in peripheral blood showed lymphopenia with a decrease of CD3+ and CD4+ T cells, whereas CD8+ lymphocytes, B cells and NK cells were within the normal range. Fecal loss of lymphocytes as a cause of lymphopenia was evidenced by a marked excretion of 111-indium-labeled peripheral blood mononuclear cells via stool. Proliferative responses against several mitogens were severely reduced as was in vitro IgG production. Delayed-type hypersensitivity reaction against a variety of antigens was absent. Vaccination with tick-borne encephalitis virus, used for primary immunization, and with the recall antigen tetanus toxoid resulted in a blunted antibody response. After pericardectomy, the severity of enteric protein loss declined, serum immunoglobulin levels returned to the normal range, and total lymphocytes and CD3+ and CD4+ counts increased but remained low even 12 months after surgery. Fecal loss of lymphocytes was found to be reduced after pericardectomy, but was higher than that seen in a disease control patient with active inflammatory bowel disease. In vitro immunoglobulin production returned to normal, DTH could be demonstrated against purified protein derivative and proteus antigen, but mitogen-driven blastogenic response of lymphocytes remained low. Revaccination with tick-borne encephalitis and tetanus toxoid antigens seven months after surgery resulted in a dramatic increase of serum levels of antibodies against both antigens, comparable to that seen in healthy control individuals.(ABSTRACT TRUNCATED AT 250 WORDS)

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

“…One would predict that there should be marked differences in the hematologic findings in the three categories of PLE since in lymphangiectasia there is bulk loss of lymph, including the lymphoid cells, whereas the other two classifications have negligible cellular loss. As predicted, the characteristic findings in the three classical increased lymph pressure conditions discussed above (primary lymphangiectasis, constrictive pericarditis, Fontan procedure) are lymphopenia and a dramatic decrease in CD4 T cells (about 10% of normal) 53,72,73. This selective depletion of CD4 cells presumably reflects the tendency of CD4 cells to circulate in lymph, whereas other immunologic cells are more tissue bound 72.…”

Diagnostic accuracy of red blood cell distribution width-to-lymphocyte ratio for celiac disease

“…Possible explanations for the low lymphocyte counts could be migration of lymphocytes in the intestinal mucosa, enteric loss and apoptosis of lymphocytes in the setting of the immune alterations of CD, and not least malnutrition (12,15,16). This has also been seen in other small bowel disorders such as protein-losing enteropathies, where fecal loss of lymphocytes and alterations in lymphocyte subpopulations have been documented (17,18). In accordance with the findings of others (19) who reported lymphocyte counts returning to normal on GFD, we have also seen a significant increase in lymphocyte counts in GFD-treated patients; this is probably due to a decrease in lymphocyte leakage through the healed mucosa (Table 1).…”

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization

“…Further implication of the lymphatic system in the etiopathogenesis has been documented on GI endoscopic biopsies showing intestinal lymphangiectasia [14]. LLE and PLE patients can have profound nutritional disorders, abnormal bone mineral density, severe lymphopenia, decreased CD4 and CD3 T cell counts, and other immune consequences [151617]. In patients with CCHD, the addition of nutritional and infectious challenges would be an added burden increasing the morbidity and mortality rates.…”