Your activity: 10 p.v.

Clinical manifestations of cutaneous and systemic mastocytosis

Clinical manifestations of cutaneous and systemic mastocytosis
Organ system affected Symptoms due to acute and/or chronic mast cell-mediator release Symptoms, physical findings, and abnormalities of routine laboratories due to organ infiltration
Skin Flushing, pruritus Multiple: Urticaria pigmentosa, plaque-like or nodular lesions, diffuse skin involvement, bullous eruptions, isolated mastocytoma, or telangiectasias
Gastrointestinal tract Nausea, bloating, chronic diarrhea, hyperacidity, gastroduodenal ulcer disease, chronic abdominal pain, vomiting

Stomach and duodenal infiltration: Gastroduodenal ulcers (possibly, although more clearly related to mediator release), steatorrhea, malabsorption

Liver infiltration: Elevated transaminases, portal hypertension, and ascites (only in advanced forms of the disease)
Musculoskeletal*

Adults: Fibromyalgia-like diffuse musculoskeletal pain, pain in long bones, osteoporosis, osteopenia, pathologic fractures

In mast cell sarcoma of the bone (rare), local symptoms (eg, pain, deformity, pathologic fracture) occur at the involved site
Cardiovascular Adults: Episodes of vasodilation, tachycardia, hypotension, collapse
Infants: Apneic spells, cyanosis
 
Hematologic (lymph nodes, spleen, bone marrow)  

Lymph nodes and spleen: Lymphadenopathy, splenomegaly (with hematologic findings of hypersplenism: nonimmune hemolytic anemia)

Bone marrow: Anemia (mild-to-moderate, in approximately 50%), thrombocytopenia (usually asymptomatic), eosinophilia (approximately 25%), myeloproliferative or myelodysplastic findings, fibrosis
Neurologic, psychiatric Adults: Anxiety, depression, headache, mood changes, inability to concentrate, hypersomnolence, irritability, "mixed organic brain syndrome"
Children: Aggressive behavior
 
Systemic* Adults: Fatigue, cachexia  
Signs and symptoms of both acute and chronic release of mast cell mediators are seen in patients 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.
* Rare in children.
Graphic 91167 Version 10.0