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|Year : 2013 | Volume
| Issue : 2 | Page : 161-162
Sustained paranoid psychosis in a patient with mast cell leukemia and decreased serotonin level
CA Dasanu1, LK Davis1, F Bauer2
1 Department of Hematology-Oncology, St. Francis Hospital and Medical Center, Hartford, Connecticsut, USA
2 Department of Anatomic and Clinical Pathology, St. Francis Hospital and Medical Center, Hartford, Connecticsut, USA
|Date of Web Publication||21-Jun-2013|
C A Dasanu
Department of Hematology-Oncology, St. Francis Hospital and Medical Center, Hartford, Connecticsut
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dasanu C A, Davis L K, Bauer F. Sustained paranoid psychosis in a patient with mast cell leukemia and decreased serotonin level. J Postgrad Med 2013;59:161-2
|How to cite this URL:|
Dasanu C A, Davis L K, Bauer F. Sustained paranoid psychosis in a patient with mast cell leukemia and decreased serotonin level. J Postgrad Med [serial online] 2013 [cited 2020 May 26];59:161-2. Available from: http://www.jpgmonline.com/text.asp?2013/59/2/161/113815
As there is paucity of literature, diagnosis of mast cell leukemia (MCL) presenting with psychiatric symptoms remains a diagnostic challenge and warrants clinicians to be alert of this rare possibility.
A 70-year-old male, with no antecedent history of any medical or psychiatric illness, was admitted with persistent diarrhea and disordered thinking for 4 weeks. He stated that his refrigerator had been "broken down for a while" and was convinced that this was part of a plot against him. He was not taking any medications. Physical examination revealed a man who was somewhat confused, but alert and oriented to the time, place and person. He had tachycardia and overactive bowel sounds. A neurologic examination was unremarkable. An abdominal ultrasound revealed moderate splenomegaly. Bone survey and brain magnetic resonance imaging were normal. Laboratories showed a white blood cell count of 2.1 × 10 9 /L, hemoglobin of 13.9 g/dl, a hematocrit of 42%, and a platelet count of 75 × 10 9 /L. Abnormal mononuclear cells with condensed nuclei and basophilic granules were identified in peripheral blood. Urine toxicology testing was normal. Review of the out-patient medical records showed no evidence of a pre-existing cognitive decline and documented normal blood counts 2 months prior. Bone marrow aspirate and biopsy confirmed a dominant atypical population of mast cells (MCs) and at least 8% of blasts [Figure 1]a and b. The cells were strongly CD 117 positive [Figure 1]c with aberrant co-expression of CD25, virtually confirming the diagnosis of MCL. The KIT Asp816Val mutation analysis came back positive. Serum uniCAP total tryptase was 1948 ng/ml (normal 1-15 ng/ml). Serotonin level was 37 ng/ml (normal 50-200 ng/ml). Electroencephalogram was normal; no epileptiform discharges and no seizures occurred. Psychiatric evaluation yielded the diagnosis of acute paranoid psychosis due to MCL.
|Figure 1: (a) Marrow aspirate showing increased atypical and immature mast cells (MCs) (H and E, ×100); (b) Bone biopsy showing immature MC infiltrate (H and E, ×60) and (c) Bone biopsy showing strong (bright) CD117 + MC infiltrate (×60)|
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The patient received intravenous rehydration and combined histamine receptor (HR) 1 and 2 antagonist therapy, with improvement in diarrhea. Haloperidol was used for the paranoid symptoms. However, his psychosis persisted and the disease followed a dramatic course with a rapidly fatal outcome.
MCL is a rare aggressive systemic mastocytosis (SM) featuring cytopenias, hepato/splenomegaly, osteolytic lesions and/or malabsorption resulting from gastrointestinal MC infiltrates.  Although, neurologic symptoms were shown to be present in some cases,  psychiatric manifestations have not been well-characterized. Rogers et al. conducted psychiatric testing in a cohort of patients with SM, revealing both cognitive and affective changes. However, none of the patients in their cohort had MCL or paranoid psychosis. The symptoms responded to HR antagonists and/or disodium cromoglycate in some patients. In contrast, our patient's psychiatric symptoms did not respond to these agents.
MCs were shown not only to synthesize and release serotonin, but also to express and be activated through multiple serotonin receptors.  Kushnir-Sukhov et al. determined the serotonin levels in a cohort of patients with SM. Lower serotonin values correlated with higher rates of fatigue, headaches, memory impairments, diarrhea, and abdominal pain.  Pharmacologic agents that alter blood serotonin levels may prove helpful in this context. Unfortunately, our patient's rapid demise precluded a trial of selective serotonin reuptake inhibitors. Currently, an array of investigations has been set up to explain various neuropsychiatric manifestations in SM. In the interim, the present case of a sustained paranoid psychosis in a patient with MCL and decreased serum serotonin level might offer additional insights to this widely debated and captivating topic, which in turn may yield new therapeutic strategies for SM/MCL.
| :: References|| |
|1.||Valent P, Akin C, Sperr WR, Escribano L, Arock M, Horny HP, et al. Aggressive systemic mastocytosis and related mast cell disorders: Current treatment options and proposed response criteria. Leuk Res 2003;27:635-41. |
|2.||Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, et al. Standards and standardization in mastocytosis: Consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 2007;37:435-53. |
|3.||Rogers MP, Bloomingdale K, Murawski BJ, Soter NA, Reich P, Austen KF. Mixed organic brain syndrome as a manifestation of systemic mastocytosis. Psychosom Med 1986;48:437-47. |
|4.||Kushnir-Sukhov NM, Brown JM, Wu Y, Kirshenbaum A, Metcalfe DD. Human mast cells are capable of serotonin synthesis and release. J Allergy Clin Immunol 2007;119:498-9. |
|5.||Kushnir-Sukhov NM, Brittain E, Scott L, Metcalfe DD. Clinical correlates of blood serotonin levels in patients with mastocytosis. Eur J Clin Invest 2008;38:953-8. |
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