Haemolytic uraemic syndrome and the Thomsen Friedenreich antigen

Abstract

In three children with haemolytic uraemic syndrome (HUS), evidence of red cell polyagglutinability due to Thomsen Friedenreich antigen (T-antigen) exposure was demonstrated. This was suspected after difficulties in ABO typing and was confirmed using specific antisera. Further supportive evidence included elevation of plasma sialic acid, alteration in red cell surface charge and evidence of T-antigen exposure in the renal biopsy specimen of one patient. Although involvement of this antigen in the pathogenesis of HUS has been associated with a high mortality, all three children have made a complete recovery. With early recognition and subsequent avoidance of plasma products, prognosis of this condition may be improved.

Streptococcus pneumoniae-associated hemolytic uremic syndrome

“…Since 1987, 73 children [14,16,18,19,[22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] with pneumococcal HUS have been reported in the English language literature. Of these, 12.3% (9/73) died; 10.1% (7/69) progressed to ESRF; and 16% (11/69) survived, with chronic kidney disease or hypertension.…”

“…The improved mortality rate in the past 20 years may have been the result of the advances in critical care medicine as opposed to changes in disease virulence [31]. A careful analysis of the 69 children with comprehensive clinical data [14,16,18,19,[22][23][24][25][26][27][28][29][30][31][32][34][35][36][37][38] reveals that only 28% of the patients presented with meningitis. However, 88% of the deaths occurred in this subgroup (clinical data available on 8/9 patient deaths).…”

“…They also have longer periods of oligo-anuria, more frequent need for acute dialysis (75% vs 59%), longer hospital stays (33 days vs 16 days), more red blood cell and platelet transfusions, and a longer duration of thrombocytopenia [31]. In our review of the 73 patients reported from 1987 to 2007 [14,16,18,19,[22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] 10.1% developed endstage renal failure (ESRF) and 12.3% died in the acute phase. These rates are two-to-three-times higher than those of STEC HUS [43,44,45].…”

“…Immunofluorescence staining is negative except for weak staining for IgM [6,11,12]. Special staining with immunofluorescence-labeled peanut agglutinin has been found to be positive in the glomeruli [6,11,13,22] and tubules [12] of several patients. This test is not widely available.…”

“…All serotypes of S. pneumoniae can have neuraminidase activity capable of unmasking the TF antigen [22]. Therefore, any invasive pneumococcal infection may result in a range of abnormalities, ranging from isolated hemolytic anemia to full-blown HUS.…”