Signs, symptoms, and abnormalities of common laboratory studies in mastocytosis (cutaneous and systemic) in infants and prepubertal children

Organ system affected	Signs and symptoms attributed to acute and/or chronic mast cell mediator release	Abnormalities of routine laboratories due to organ infiltration
Systemic	Anaphylaxis, fatigue
Skin	Flushing, pruritus, urticaria Darier's sign (urticarial flaring of skin lesions) Polymorphic MPCM/UP (most common) Bullous eruptions/blistering Nodular or plaque-like lesions ranging in size from several millimeters to 10 cm Diffuse skin involvement (rare) Isolated mastocytoma (rare) Monomorphic MPCM/UP (rare)
Gastrointestinal tract	Abdominal pain, bloating, fussy eating Nausea, vomiting, diarrhea Gastroduodenal ulcers Hepatomegaly	Liver infiltration: Elevated alkaline phosphatase, transaminases, gamma-glutamyl transpeptidase, and/or bilirubin
Neurologic, behavioral	Apneic spells, cyanosis Irritability, aggressive behavior, anxiety Inability to concentrate, withdrawal behavior
Hematologic (lymph nodes, spleen, peripheral blood)	Lymphadenopathy, splenomegaly	Splenic infiltration: May have nonimmune hemolytic anemia resulting from hypersplenism Peripheral blood: Anemia (mild to moderate) Thrombocytopenia (usually asymptomatic) Eosinophilia

Signs and symptoms of both acute and chronic release of mast cell mediators are seen in infants and prepubertal children 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 the signs and symptoms described in the table. In cutaneous mastocytosis, which accounts for 90% of pediatric mastocytosis, mast cells infiltrate the skin but not other organs. In children 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.

MPCM/UP: maculopapular cutaneous mastocytosis/urticaria pigmentosa.

Graphic 121386 Version 2.0

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Signs, symptoms, and abnormalities of common laboratory studies in mastocytosis (cutaneous and systemic) in infants and prepubertal children