Aleukemic leukemia presenting with paranoid psychosis.

Area of Science:

• Hematology
• Neuroscience
• Psychiatry

Background:

• Mast cells synthesize and release serotonin, and express serotonin receptors.
• Systemic mastocytosis (SM) is a rare disorder characterized by mast cell proliferation.
• Mast cell leukemia (MCL) is an aggressive subtype of SM with poorly understood psychiatric manifestations.

Observation:

• A patient with KIT D816V positive MCL and gastrointestinal involvement presented with severe paranoid delusional illness.
• Gastrointestinal symptoms improved with H1 and H2 antagonists.
• Psychosis persisted despite treatment, and the patient had a rapidly fatal outcome.

Findings:

• This case illustrates a unique presentation of MCL with severe psychiatric symptoms.
• The study highlights the potential link between mast cell biology, serotonin, and neuropsychiatric disorders.
• Diagnosis of MCL with psychiatric symptoms is challenging due to limited literature.

Implications:

• Clinicians should consider MCL in patients with unexplained severe psychiatric symptoms, especially with gastrointestinal complaints.
• Further research is needed to understand the neurobiological mechanisms underlying psychiatric manifestations in MCL.
• Early recognition and multidisciplinary management may be crucial for improving outcomes in rare cases of MCL.