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Aggressive systemic mastocytosis with multiple organ involvement: a case report.
Effat Iranijam1, Maryam Salimi2, Mohammad Negaresh2
1Department of Internal Medicine (Hematology Division), School of Medicine, Ardabil University of Medical Sciences, Ardabil, Iran.
Oxford Medical Case Reports
|November 30, 2023
Summary
Systemic mastocytosis, a rare cancer of abnormal mast cells, can present with non-cutaneous symptoms like back pain. Hypereosinophilia may indicate this condition, even without skin lesions.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Systemic mastocytosis (SM) is a rare clonal myeloid neoplasm characterized by mast cell proliferation.
- Diagnosis typically relies on identifying abnormal mast cell infiltrates in various organs.
Observation:
- A 63-year-old woman presented with lower back pain and hypereosinophilia.
- Bone marrow biopsy revealed increased mastocytes with positive CD117, CD25, and PDGFRA markers.
Findings:
- The patient was diagnosed with aggressive systemic mastocytosis based on clinical and pathological findings.
- Treatment with cladribine was initiated, but the patient unfortunately expired due to complications.
Implications:
- This case highlights that systemic mastocytosis can manifest with non-cutaneous symptoms.
- Hypereosinophilia warrants consideration of systemic mastocytosis in differential diagnoses, irrespective of skin involvement.
