Granulocyte mucopolysaccharide sulfation in patients with polycythemia vera

Prior studies have revealed defective mucopolysaccharide sulfation (MPS) in leukemic granulocyte precursors. Polycythemia Vera (PV) is regarded as a chronic myeloproliferative disorder involving all cell lines of the bone marrow. Therefore, granulocyte MPS was studied in bone marrow samples obtained from 9 patients with PV, to determine whether a MPS defect exists in this disease. Thirteeen patients with unrelated diseases served as controls. Sulfation with ³⁵SO₄ was performed in short term bone marrow suspension culture employing a 3 hr incubation with isotope. Protein bound radioactivity was separated by gel filtration of the cell lysate and then counted. Results were expressed as cpm/10⁷ cells incorporating isotope (myeloblasts, promyelocytes, as myelocytes). Values for MPS in controls ranged from 15,000 to 57,000, with a mean of 32,000 cpm/10⁷ cells. Four patients with PV had normal MPS, while in 5 patients, MPS ranged from 2,000 to 14,000 cpm/10⁷ cells. The defect in MPS in these 5 patients was significant when compared to controls (p<.001). MPS was correlated with clinical and chemical findings in the 9 PV patients. MPS did not correlate with Hgb, WBC, platelet count, red cell mass, serum B₁₂, or B₁₂ binding capacity. Four patients had normal leukocyte alkaline phosphatase (LAP) and low MPS; 4 had high LAP and normal MPS. One patient with low MPS and high LAP had been splenectomized prior to study. A defect in granulocyte mucopolysaccharide sulfation similar to that found in the granulocyte leukemias is therefore observed in polycythemia vera.