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[Primary myelofibrosis transforming into multiple subcutaneous monoblastoma--a case report]
K Hamamoto1, H Taniguchi, S Ohga
1First Department of Internal Medicine, Kansai Medical University.
[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|November 1, 1991
Summary
A patient with primary myelofibrosis developed multiple subcutaneous tumors. Biopsies revealed these tumors originated from monocytes, indicating a rare extramedullary manifestation of the disease.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Primary myelofibrosis is a bone marrow disorder characterized by fibrosis and extramedullary hematopoiesis.
- Patients may present with constitutional symptoms, splenomegaly, and cytopenias.
Observation:
- An 83-year-old man with primary myelofibrosis presented with multiple subcutaneous tumors.
- Tumor biopsy revealed diffuse infiltration of monocytes and promonocytes.
- Immunohistochemical and ultrastructural analysis confirmed monocytic origin.
Findings:
- The subcutaneous tumors were identified as extramedullary monocytic infiltrates.
- Monocytic origin was supported by positive alpha-naphtyl butyrate esterase staining and specific cell surface markers (CD11b, CD13, CD14, CD33, HLA-DR).
- The patient also exhibited leukoerythroblastosis, marked bone marrow fibrosis, and elevated lysozyme levels.
Implications:
- This case highlights a rare extramedullary manifestation of primary myelofibrosis.
- Subcutaneous tumors in myelofibrosis patients may represent monocytic differentiation.
- Further research is needed to understand the pathogenesis and clinical significance of such presentations.