A 50-year-old man with the rare McLeod syndrome, associated with glomerular
lesion to the end stage of chronic renal failure and death, is reported. M
cLeod syndrome is an X-linked recessive disorder on the basis of abnormal e
xpression of the I(ell blood group antigens and absence of erythrocyte surf
ace Kx antigen. Most often the clinical and pathological findings are retin
itis pigmentosa to blindness, progressive chronic neuropathy, cortical atro
phy, dilated cardiomyopathy, and glomerular lesion with chronic renal failu
re. Among the laboratory parameters the most important are very low level o
f cholesterol and triglycerides, then various numbers of acanthocytes in pe
ripheral blood smears and sometimes in urine (as in our case).