Signs, symptoms, and abnormalities of common laboratory studies in mastocytosis (cutaneous and systemic) in post-pubescent adolescents and adults

Organ system affected	Signs and symptoms attributed to acute and/or chronic mast cell mediator release or organ infiltration	Abnormalities of routine laboratories due to organ infiltration
Systemic	Anaphylaxis, fatigue, cachexia.
Skin	Flushing, pruritus, urticaria. Darier's sign (urticarial flaring of skin lesions). Skin findings: Monomorphic MPCM/UP (most common). Diffuse skin involvement.
Gastrointestinal tract	Nausea, vomiting, bloating, chronic diarrhea, steatorrhea, chronic abdominal pain. Gastroduodenal ulcers. Gastroesophageal reflux disease. Hepatomegaly.	Liver infiltration: Elevated alkaline phosphatase, elevated transaminases, gamma-glutamyl transpeptidase, portal hypertension, and ascites (only in advanced forms of systemic mastocytosis).
Neurologic, psychiatric	Anxiety, depression, headache, mood changes, inability to concentrate, hypersomnolence, irritability, short memory span, "mixed organic brain syndrome."
Hematologic (lymph nodes, spleen, peripheral blood)	Lymphadenopathy, splenomegaly.	Splenic infiltration: May have nonimmune hemolytic anemia resulting from hypersplenism. Peripheral blood: Anemia (mild-to-moderate, in approximately 50%). Thrombocytopenia (usually asymptomatic). Eosinophilia (in approximately 25%).
Cardiovascular	Episodes of vasodilation, tachycardia, hypotension, and/or collapse.
Musculoskeletal system	Fibromyalgia-like diffuse musculoskeletal pain, pain in long bones, osteoporosis, osteopenia, pathologic fractures.

Signs and symptoms of both acute and chronic release of mast cell mediators are seen in post-pubescent adolescents and adults with cutaneous and systemic forms of mastocytosis. Episodic symptoms tend to occur in patterns that are characteristic for a given patient, but not all patients demonstrate all of the signs and symptoms described in the table. In patients with cutaneous mastocytosis, mast cells infiltrate the skin. In patients with systemic mastocytosis, mast cells may infiltrate the skin, gastrointestinal tract and liver, lymph nodes, spleen, and bone marrow. The signs and symptoms shown in bold are only seen with systemic forms of the disease.

MCPM/UP: maculopapular cutaneous mastocytosis/urticaria pigmentosa.

Graphic 121385 Version 2.0

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Signs, symptoms, and abnormalities of common laboratory studies in mastocytosis (cutaneous and systemic) in post-pubescent adolescents and adults