Agnogenic myeloid metaplasia: role of splenectomy.

The splenomegaly associated with myelofibrosis and agnogenic myeloid metaplasia should not be considered a manifestation of the fundamental proliferative process, nor should it be considered as necessarily compensatory for reduced marrow haematopoiesis. In deserving cases splenectomy may cause an improvement in the patient's general and haematopoietic status. Removal of the source of functional hypersplenism, causing haemolytic episodes and thrombocytopenia, results in marked amelioration in the clinical condition with reduction in the magnitude and frequency of replacement blood transfusion. The massive size of the spleen associated with this condition may not only cause local pain and discomfort but may lead to traumatic or spontaneous rupture. Consideration of two cases studied by the authors indicates that marked clinical improvement may be associated with splenectomy in selected cases of agnogenic myeloid metaplasia.