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Systemic mast cell disease without associated hematologic disorder: a combined retrospective and prospective study
Animesh Pardanani1, Jin-Young Baek, Chin-Yang Li
1Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.
Mayo Clinic Proceedings
|November 21, 2002
Summary
Systemic mast cell disease (SMCD) without hematologic disorders shows that bone marrow mast cell burden, eosinophilia, and serum alkaline phosphatase levels are key prognostic indicators. Other factors like angiogenesis and fibrosis are not relevant.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Systemic mast cell disease (SMCD) encompasses a spectrum of disorders.
- Identifying prognostic factors in SMCD without associated hematologic conditions is crucial for patient management.
Purpose of the Study:
- To investigate clinicopathologic correlations in SMCD patients without hematologic disorders.
- To identify variables with prognostic significance in this specific patient cohort.
Main Methods:
- Retrospective and prospective analysis of 40 adult patients with SMCD.
- Evaluation of clinical, laboratory, and bone marrow (BM) histologic findings.
- Correlation of findings with aggressive SM history and patient survival.
Main Results:
- Increased BM mast cell (MC) content, unfavorable MC infiltration, BM eosinophilia, and elevated serum alkaline phosphatase (SAP) correlated with aggressive SM.
- Elevated BM MC content and SAP level were associated with reduced survival.
- BM angiogenesis, reticulin fibrosis, and MC mediator levels did not correlate with aggressive SM or survival.
Conclusions:
- BM MC burden, BM eosinophilia, and SAP level are significant prognostic markers in SMCD without associated hematologic disorders.
- BM angiogenesis, reticulin fibrosis, and MC mediator levels lack prognostic value in this context.