Mastocytosis in adulthood and neuropsychiatric disorders

Abstract

Patients with mastocytosis can display various disabling general and neuropsychological symptoms among one third of them, including general signs such as fatigue and musculoskeletal pain, which can have a major impact on quality of life. Neurological symptoms are less frequent and mainly consist of acute or chronic headache (35%), rarely syncopes (5%), acute onset back pain (4%), and in a few cases, clinical and radiological symptoms resembling or allowing the diagnosis of multiple sclerosis (1.3%). Headaches are associated with symptoms related to mast cell activation syndrome (flushes, prurit, and so forth) and more frequently present as migraine (37.5%), with often aura (66%). Depression-anxiety like symptoms can occur in 40% to 60% of the patients and cognitive impairment is not rare (38.6%). The pathophysiology of these symptoms could be linked to tissular mast cell infiltration or to mast cell mediators release or both. The tryptophan metabolism could be involved in mast cell-induced neuroinflammation through indoleamine-2,3-dioxygenase activation. Treatments targeting mast cell may be useful to target neuropsychological features associated with mastocytosis, including tyrosine kinase inhibitors.