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

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

Medical Therapy

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

Corticosteroids

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:

Interferon-α

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

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

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

Imatinib

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

Dasatinib

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

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

References

↑ ^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.
↑ ^2.0 ^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.

[Georgin-LavialleLhermitte2012-1] 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.

[JorisGeorgin-Lavialle2012-2] 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.

[1]

[2]