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Systemic mast cell disease presenting with peripheral blood eosinophilia
R N Miranda1, A R Esparza, S Sambandam
1Department of Pathology, Rhode Island Hospital, Providence 02903.
Human Pathology
|July 1, 1994
Summary
Systemic mast cell disease can present without skin lesions, sometimes manifesting initially as unexplained eosinophilia. Early diagnosis is crucial for proper patient management.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Systemic mast cell disease (SMCD) often presents with skin lesions (urticaria pigmentosa).
- A subset of SMCD patients lacks skin manifestations, complicating diagnosis.
- Hematologic abnormalities, including eosinophilia, are common in SMCD.
Observation:
- An 82-year-old woman with coronary artery disease presented with incidental severe eosinophilia, thrombocytopenia, and anemia.
- The patient had no skin lesions but exhibited left upper quadrant tenderness.
- Bone marrow biopsy revealed mast cell aggregates and eosinophils, suggesting SMCD.
Findings:
- Autopsy confirmed widespread mast cell aggregates in multiple organs.
- Bone marrow showed hyperplasia with granulocytic and eosinophilic predominance.
- Histological stains confirmed mast cell granules, supporting the SMCD diagnosis.
Implications:
- Peripheral eosinophilia can be an initial presenting sign of SMCD, even without skin lesions.
- High clinical suspicion is vital for appropriate bone marrow biopsy handling and diagnosis.
- Accurate diagnosis of SMCD is essential for effective treatment and patient outcomes.