Mast cell leukemia medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]

## Overview

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The mainstay of therapy for symptomatic mast cell leukemia patients is immunochemotherapy.

## Medical Therapy

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Therapeutic approaches are limited and consist of reducing the tumor burden and conservation of organ functions.[1]

### Corticosteroids

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* Corticosteroids have been recommended as a short term use in combination with cytotoxic drugs.[2][1]
* It could also be beneficial in some situations such as:

* Malabsorption
* Ascites
* Anaphylaxis

### Interferon-α

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Interferon-α reduces the frequency of:[2][1]

* Histamine related attacks
* Decrease bone marrow infiltration by mast cells
* Decrease liver infiltration by mast cells
* Decrease skin manifestations
* Improve osteoporosis

### 2-Chloro-deoxy-adenosine

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2-Chloro-deoxy-adenosine has a potential value in the treatment of mast cell leukemia with symptomatic responses and improvement in mast cell variables.[2][1]

### Cladribine

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Cladribine has been used in rare cases of mast cell leukemia with relatively small or no efficacy.[2][1]

### Imatinib

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Imatinib does not have direct effect on the D816V KIT mutation, but it may affect other sporadic mutations.[2][1]

### Dasatinib

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Dasatinib demonstrates significant inhibitory activity against WT KIT as well as juxtamembrane domain mutant KIT. This activity has been proven in patients negative for D816V KIT.[2][1]

### Nilotinib

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Nilotinib displays also equipotent activity to imatinib in D816V c-KIT negative patients.[2][1]

## References

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1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Georgin-Lavialle, S.; Lhermitte, L.; Dubreuil, P.; Chandesris, M.-O.; Hermine, O.; Damaj, G. (2012). "Mast cell leukemia". Blood. 121 (8): 1285–1295. doi:10.1182/blood-2012-07-442400. ISSN 0006-4971.
2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Joris, Magalie; Georgin-Lavialle, Sophie; Chandesris, Marie-Olivia; Lhermitte, Ludovic; Claisse, Jean-François; Canioni, Danielle; Hanssens, Katia; Damaj, Gandhi; Hermine, Olivier; Hamidou, Mohammed (2012). "Mast Cell Leukaemia: c-KIT Mutations Are Not Always Positive". Case Reports in Hematology. 2012: 1–6. doi:10.1155/2012/517546. ISSN 2090-6560.