title: Aleukemic leukemia presenting with paranoid psychosis.
abstract: Mast cells not only synthesize and release serotonin, but also express and are activated through multiple serotonin receptors. Low blood serotonin level might define a specific subset of patients with systemic mastocytosis (SM) who are more likely to present with neurologic and gastrointestinal complaints. Mast cell leukemia (MCL) is a rare and aggressive type of systemic mastocytosis, and psychiatric manifestations in its course have not been well-characterized. We describe herein a unique patient with a KIT D816V mutation positive a leukemic leukemia variant of systemic mastocytosis with gastrointestinal involvement, presenting with a severe and sustained paranoid delusional illness. While diarrhea improved with the use of histamine H1 and H2 receptor antagonists, the psychosis did not, and the disease followed a dramatic course with a rapidly fatal outcome. As there is paucity of literature, diagnosis of MCL presenting with psychiatric symptoms remains a diagnostic challenge and warrants clinicians to be alert of this rare possibility.
authors.affiliation: Department of Hematology-Oncology, St. Francis Hospital and Medical Center, Hartford, USA. c_dasanu@yahoo.com
authors.forename: Constantin A
authors.initials: CA
authors.lastname: Dasanu
authors.forename: Lynn K
authors.initials: LK
authors.lastname: Davis
authors.forename: Frank
authors.initials: F
authors.lastname: Bauer
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D003967:Diarrhea / Q000209:etiology; D006801:Humans; D007946:Leukemia, Mast-Cell / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D007958:Leukocyte Count; D008297:Male; D010259:Paranoid Disorders / Q000503:physiopathology*; D012701:Serotonin / Q000097:blood
chemical_list: D012701:Serotonin