Thirty-seven patients with
mastocytosis and unexplained elevated levels of urinary
N-methylhistamine who were undergoing bone marrow biopsy were studied with respect to the diagnosis of
mastocytosis and the manifestations of the disease. These patients were from a group of 66 patients from whom a bone marrow biopsy was obtained and urinary
N-methylhistamine levels were measured in the period 1990-1998. In seven (19%) of the 37 patients,
mastocytosis was limited to the skin. Five (14%) of the 37 patients showed accumulation of mast cells in the bone marrow without characteristic skin lesions, whereas seven (19%) of the 37 patients showed increased numbers of mast cells both in the skin and the bone marrow. Eighteen (49%) of the 37 patients with elevated
N-methylhistamine did not have mast cell accumulation in either the skin or the bone marrow biopsy. The median level of
N-methylhistamine in the urine of patients with
mastocytosis limited to the skin was 245 micro mol/mol
creatinine. The average level of
N-methylhistamine was 509 micro mol/mol
creatinine in patients with mast cell accumulation in the bone marrow and
cutaneous mastocytosis. There was a significant difference in the levels of
N-methylhistamine in patients with mast cell accumulation in the bone marrow biopsy compared with those without. The likelihood of
mastocytosis with mast cell accumulation in the bone marrow biopsy at a given level of
N-methylhistamine was calculated. It was established that an
N-methylhistamine level of 297 micro mol/mol
creatinine or higher may be considered as a threshold
indicator for obtaining a bone marrow biopsy in patients suspected of
mastocytosis with mast cell accumulation in the bone marrow. For practical purposes, we propose to consider the cut-off level of approximately 300 micro mol/mol
N-methylhistamine creatinine for this assay.