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[Systemic mastocytosis. Classification, symptoms, therapy].

Doris M Kraemer1, Susanne M Grunewald, Annette Kolb-Mäurer

  • 1Medizinische Poliklinik der Universität Würzburg, Würzburg. kraemer_d@klinik.uniwuerzburg.de

Medizinische Klinik (Munich, Germany : 1983)
|March 17, 2004
PubMed
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Systemic mastocytoses involve abnormal mast cell growth in tissues. Current therapies manage symptoms as there is no known cure for this clonal disorder.

Area of Science:

  • Hematology
  • Oncology

Background:

  • Systemic mastocytoses are clonal disorders characterized by abnormal mast cell proliferation and infiltration of various organs.
  • These conditions can arise from mutations in the c-kit protooncogene, which encodes the stem cell receptor (CD117).

Observation:

  • The World Health Organization (WHO) classification (2000) categorizes mastocytoses into cutaneous and systemic types.
  • Systemic mastocytosis is further divided into indolent forms with good prognosis and aggressive subtypes with poor prognosis, including aggressive systemic mastocytosis, mast cell leukemia, mast cell sarcoma, and systemic mastocytosis with associated clonal hematologic non-mast-cell disease.

Findings:

  • Therapeutic strategies for systemic mastocytosis are symptom-dependent and do not offer a cure.
  • Management includes dietary modifications, avoidance of skin irritants, H1/H2 blockers, steroids, leukotriene receptor antagonists, and PUVA therapy.

Related Experiment Videos

  • Emergency treatment with epinephrine is recommended for systemic reactions like hypotension or syncope.
  • Implications:

    • Understanding the classification and genetic basis of systemic mastocytoses is crucial for accurate diagnosis and prognosis.
    • Tailored, multidisciplinary symptom management is essential for improving the quality of life for patients with systemic mastocytosis.