Maroteaux-Lamy is Type VI MPS due to deficient enzyme activity of Arylsulphatase B. It shows an autosomal recessive inheritance & onset is late infantile. Dermatan sulphate is excreted in urine. Patients present with hepatosplenomegaly, skeletal dysplasia, corneal clouding, coarse facies & valvular heart disease. An important hematologic finding is granulated neutrophils & lymphocytes. No cognitive degeneration is present.