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

Signs, symptoms, and abnormalities of common laboratory studies in mastocytosis (cutaneous and systemic) in infants and pre-pubertal 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 pre-pubertal 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 1.0