Mast Cell Disorders

Abstract

Abstract Mast cell disorders are considered with the myeloproliferative disorders since mast cells are derived from pluripotential bone marrow stem cells and mast cell proliferations may be associated with both myeloproliferative and myelodysplastic disorders (Travis et al, 1988). The disorders of mast cells include systemic mastocytosis, mast cell leukemia, and the “eosinophilic fibrohistiocytic lesion,” a mast cell lesion of uncertain significance. Systemic mastocytosis is a generalized proliferative disorder of mast cells involving the bone marrow, liver, spleen, and other organs. Systemic mastocytosis is a generalized mast cell proliferation. Mast cell disease may be confined to the skin (urticaria pigmentosa) or be generalized with or without skin involvement (systemic mastocytosis). Urticaria pigmentosa is most common in childhood and is usually self-limited, but may be associated with the development of systemic mastocytosis. Adults with urticaria pigmentosa more commonly progress to systemic mastocytosis. Systemic mastocytosis is associated with an underlying hematologic disorder (most commonly a myelodysplastic syndrome or myeloproliferative disorder) in approximately one-third of cases (Travis et al, 1988). Patients with systemic mastocytosis frequently present with symptoms of hyperhistaminemia (flushing, hypotension, angioedema, dermatographism, diarrhea, abdominal pain), hepatosplenomegaly, or skeletal disease (bone pain, osteoporosis, osteosclerosis).