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Information

Although the diagnosis of mastocytosis may be suspected on the basis of clinical and laboratory findings, it can be established only by tissue biopsy (usually bone marrow biopsy). The diagnostic criteria for systemic mastocytosis are shown in Table 156-2. Laboratory studies that can help support a diagnosis of systemic mastocytosis include measurement of urinary or blood levels of mast cell products such as histamine, histamine metabolites, prostaglandin D2 (PGD2) metabolites, or mast cell tryptase. Other studies including bone scan, skeletal survey, and GI contrast studies may be helpful. Other flushing disorders (e.g., carcinoid syndrome, pheochromocytoma) should be excluded.

Treatment: Systemic Mastocystosis

  • H1 and H2 antihistamines.
  • Proton pump inhibitors for gastric hypersecretion.
  • Oral cromolyn sodium for diarrhea and abdominal pain.
  • Aspirin for severe flushing to block PGD2.
  • Systemic glucocorticoids may help, but frequently are associated with complications.
  • Mast cell cytoreductive therapy with interferon α (IFN-α) or cladribine is generally reserved for advanced, nonindolent variants of systemic mastocytosis.
  • Chemotherapy for frank leukemias.

For a more detailed discussion, see Boyce JA, Austen KF: Allergies, Anaphylaxis, and Systemic Mastocytosis, Chap. 376, p. 2113, in HPIM-19.

Outline

Section 12. Allergy, Clinical Immunology, and Rheumatology