Mast cell leukemia C94.3

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

Mast cell leukemia; Mast cell reticulosis

Definition
This section has been translated automatically.

Mastocytosis with systemic malignant mast cell proliferation and leaching of atypical mast cells into the peripheral blood

Manifestation
This section has been translated automatically.

Especially patients with systemic mastocytosis without cutaneous skin involvement are at risk (approx. 30%).

Clinical features
This section has been translated automatically.

Corresponding to mastocytosis.

Diagnosis
This section has been translated automatically.

>20% mast cells in the bone marrow aspirate, mast cells usually immature, often blastar. >10% mast cells in blood smear.

Therapy
This section has been translated automatically.

Both aggressive systemic mastocytosis (ASM) and mast cell leukemia require cytoreductive therapy because they are life-threatening.

Cytostatic drugs such as cyclophosphamide, chlorambucil and others can be tried. However, the chances of success are low.

Progression/forecast
This section has been translated automatically.

Infaust, usually rapidly progressive course. In mastocytosis with skin involvement malignancy development is very rare.

Literature
This section has been translated automatically.

  1. Arber DA et al (2016) The 2016 revision to the World Health Organization classification of myeloidneoplasms
    and acute leukemia. Blood 127:2391-405.
  2. Torrey E et al (1990) Malignant mastocytosis with circulating mast cells. Am J Hematol 34: 283-286

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020