Abstract
A case of systemic mast cell disease (SMCD) was reported. A 22-year-old woman who had been suffering from pruritus at the palms and legs since 17 years old, admitted to the hospital because of developing systemic pruritus and edema on the extremities.
On admission, she had swelling of left parotid gland and systemic peripheral lymphadenopathy. Laboratory findings showed eosinophilia (WBC 15, 000/mm3, eosinophil 51%) and elevated serum LDH (1, 309IU/l). These symptoms and abnormal laboratory findings fluctuated every 20 to 40 days. In addition, the elevation of serum IgE and mild hepatosplenomegaly were seen.
The increase of mast cells were seen in the biopsied specimens of bone marrow, lymph nodes, parotid gland, skin and liver. From these findings the diagnosis of SMCD was made.
Besides of these findings, peripheral blood lymphocytes revealed about 40% of CD3 (-) CD4 (+) cells among whole lymphocytes. Three-color staining was useful for the further examination of surface markers of these abnormal lymphocytes. Lymphocyte subset abnormality like this has not been reported in SMCD until now.
SMCD is a rare disease. But eosinophilia accompanies about 15% of SMCD. If we encounter the eosinophilia of unknown origin, we should take SMCD into account.
