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Splenic hematopoiesis in polycythemia vera. A morphologic and immunohistologic study
B C Wolf1, P M Banks, R B Mann
1Department of Pathology, New England Deaconess Hospital, Boston, Massachusetts.
American Journal of Clinical Pathology
|January 1, 1988
Summary
In polycythemia vera, splenomegaly progresses with extramedullary hematopoiesis in the spleen during the spent phase. This indicates disease progression and myeloid metaplasia, distinct from early stages.
Area of Science:
- Hematology
- Pathology
- Oncology
Background:
- Polycythemia vera is a myeloproliferative neoplasm characterized by increased red blood cell production.
- Splenomegaly is a common clinical manifestation, but its pathogenesis, particularly the role of extramedullary hematopoiesis, requires further clarification.
Purpose of the Study:
- To investigate the degree of hematopoietic cellularity in spleens of polycythemia vera patients.
- To elucidate the pathogenesis of splenomegaly in polycythemia vera.
- To differentiate between uncomplicated polycythemia vera and its progression to spent phase/myeloid metaplasia.
Main Methods:
- Immunohistologic techniques were employed to study spleen tissue.
- Spleens from 42 patients with polycythemia vera were analyzed.
- Patients were categorized into erythrocytotic and spent phases.
Main Results:
- Spleens in the erythrocytotic phase showed erythrocyte congestion but minimal extramedullary hematopoiesis.
- Spleens in the spent phase exhibited significant trilinear extramedullary hematopoiesis.
- Splenomegaly and extramedullary hematopoiesis correlated with increased medullary reticulin and leukoerythroblastosis.
Conclusions:
- Splenic myeloid metaplasia signifies progression to the spent phase or postpolycythemic myeloid metaplasia, not uncomplicated polycythemia vera.
- Hematopoietic precursors in the spleen during the spent phase of polycythemia vera and agnogenic myeloid metaplasia result from peripheral blood filtration.