Hereditary nephritis associated with May-Hegglin anomaly.
Hereditary nephritis associated with hematologic abnormalities seems to be an exceptional occurrence. We have observed a family which nephritis was combined with May-Hegglin anomaly. A girl and her father suffered from proteinuria; a paternal uncle received kidney graft; a paternal grand aunt died on periodic hemodialysis. The girl, the father and the uncle presented macrothrombocytopenia (40-100 X 10(9)/l, size 4-8 mum) with prolonged bleeding time (which precluded renal biopsy) and cytoplasmic inclusions in neutrophils (Döhle bodies). These hematologic abnormalities characterize the May-Hegglin anomaly. This kind of association has not been reported so far.
D001760:Bleeding Time; D001778:Blood Coagulation Disorders / Q000150:complications*; D001792:Blood Platelets / Q000473:pathology; D001856:Bone Marrow Examination; D002648:Child; D005260:Female; D006098:Granulocytes / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D009394:Nephritis, Hereditary / Q000150:complications*; D009504:Neutrophils / Q000473:pathology; D010375:Pedigree; D010974:Platelet Aggregation; D010979:Platelet Function Tests; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications*