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ALP Isoenzyme Separation in Type 1 Gaucher Disease

Sir, Gaucher type 1 disease is one of the most common sphingolipidoses , caused by deficiency of the enzyme acid β-glucosidase. Clinical signs include hepatosplenomegaly, pancytopenia and osteolytic and osteopenic degeneration of the skeleton (1). Enzyme replacement therapy (ERT) with modified β-glucosidase (alglucerase) results in a quick reduction in liver and spleen size and an improvement in platelet counts and hematocrits, while skeletal responses develop slowly (2, 3). Alkaline phosphatase (ALP) is one of the more extensively studied enzymes in laboratory practice and is the most frequently used marker for osteoblastic bone formation. The adult reference range is 45–122U/l for males and 34–100U/l for females (4), while the normal adult population of ALP isoenzymes is: bone 40–60%, liver 40–60%, intestine <15% (5). In the present letter, we report the effects of alglucerase on the changes in the total ALP and hepatic and bone ALP isoenzymes in 11 Gaucher type I adult patients (six males, five females, average age 33.4 years; range 25–45 years,) at baseline, before starting ERT, after a mean period of 4months (range 3–5 months) of therapy and finally after 1 year (range 10–13 months). The mean age of diagnosis in the 11 patients was 24.7 years; they had mild to moderate disease, as scored by Zimran et al. (6), three of them had been previously splenectomized. They started alglucerase at a dosage ranging 25–35 U/kg /month infused bi-weekly and maintained this regime throughout. Total ALP was determined using an automatic analyzer, Cobas Integra (Roche Basel, Switzerland) at 37 °C. ALP isoenzymes were separated by electrophoresis in agarose gel (HELENA, Beaumont, TX, USA). Analysis of variance for repeated measures design was used to test the differences of the mean values of the selected markers obtained at baseline and after 4 months and one year of treatment. Total ALP mean value decreased from 95.1 U/l at baseline to 77.1 U/l after 4 months of treatment, and to 66.8 U/l after 1 year. The corresponding values for liver ALP isoenzyme were 47.2 U/l, 34.7 U/l and 29.2 U/l respectively. The overall trend between baseline and one year was highly significant (p-values 0.0002 for total ALP, and 0.001 for hepatic isoenzyme). Bone ALP isoenzyme had a mean value of 35.3 U/l before starting therapy, 31.9 U/l after 4 months and 30.2 U/l after 12 months. The decrease was not statistically significant (p-value 0.46) (Figure 1 shows changes of individual ALP activities). No literature data are available on modification of ALP and isoenzymes during ERT. Liver ALP isoenzyme decreased significantly during follow-up. This may be