Urinary 11β-PGF2α and N-methyl histamine correlate with bone marrow biopsy findings in mast cell disorders

Background The utility of measuring histamine and prostaglandin metabolites in the urine of patients with mastocytosis has not been critically examined in a large series of patients. This study examined the relationship between the extent of increase in urinary excretion of 11β-prostaglandinF2_α and N-methyl histamine, with serum tryptase, whole blood serotonin, and bone marrow findings including morphology, percentage involvement, and abnormal mast cell phenotype. Methods This was a retrospective analysis of 90 patients who were continuously enrolled in the study for a period of 6 years (2008-2014). We recorded serum tryptase, whole blood serotonin, levels of urinary mast cell metabolites 11β-prostaglandinF2α and N-methyl histamine (NMH), and bone marrow findings. Results Urinary mast cell metabolites 11β-prostaglandinF2α and N-methyl histamine correlated with levels of serum tryptase, mast cell burden in the bone marrow, the presence of mast cell aggregates, and atypical mast cells on bone marrow biopsy. Whole blood serotonin did not have a significant correlation with the serum tryptase or mast cell burden in the bone marrow. Urinary NMH was significantly different between c-kit D816V-positive and c-kit D816V-negative patients, while 11β-prostaglandinF2α was not. Urinary 11β-prostaglandinF2α 24-h excretion >3500 ng and NMH levels >400 μg/gm Cr corresponded with the high degree of bone marrow biopsies positive for atypical mast cells, the presence of aggregates, and c-kit mutation. Conclusions Easily obtained and quantified urinary metabolites of histamine (greater than twice the upper limit of normal) and prostaglandin D₂ (>3.4 times the upper limit of normal) correlate well with bone marrow findings of mastocytosis.