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[Systemic mastocytosis. Study of 14 cases]
J A García-Erce1, P Giraldo, P de Vicente
1Servicio de Hematología y Hemoterapia, Hospital Miguel Servet, Zaragoza.
Medicina Clinica
|December 22, 1998
Summary
Systemic mastocytosis (SM) often presents with skin lesions like urticaria pigmentosa. Bone marrow involvement is constant in SM, making its study crucial for diagnosis.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Systemic mastocytosis (SM) is a rare myeloproliferative neoplasm characterized by abnormal mast cell accumulation.
- Accurate diagnosis and classification are essential for appropriate patient management.
Observation:
- A retrospective analysis of 14 systemic mastocytosis cases diagnosed between 1991-1996 was conducted.
- Patients were classified into four groups based on Metcalfe's criteria, ranging from indolent mastocytosis to mastocytic leukemia.
- Common initial complaints included urticaria pigmentosa (71.4%), with skin lesions and digestive symptoms being predominant clinical features.
Findings:
- The average age at diagnosis was 52.4 years, with 64% of patients being female.
- Osteopenia was a significant radiological finding, present in 50% of patients.
- Bone marrow involvement was universal (100%), with myelodysplastic features noted in two cases.
Implications:
- Diagnosis of SM can be challenging without skin lesions, highlighting the importance of considering bone marrow evaluation.
- Constant bone marrow involvement underscores its high diagnostic yield in suspected systemic mastocytosis.
- This study contributes to understanding the clinical, analytical, and evolutive spectrum of systemic mastocytosis.