Li Liu, MD
Last Modified: November 1, 2001
Dear OncoLink "Ask the Experts,"
My wife has had polycythemia vera which was diagnosed in 1964. She was treated initially with frequent phlebotomies during the first decade of her illness. She developed splenomegaly and underwent surgical splenectomy in 1984. In the early 1990s she went into post polycythemic myeloid metaplasia with bone marrow fibrosis. She has been on hydroxyurea and has been receiving Epogen for anemia. She has developed mesenteric mass, which has been followed by CT scan. It is the foci of extramedullary hematopoiesis, although no biopsy was taken because of her coagulopathy. It has enlarged to the size of a full term pregnancy during the past several months. Her doctor is suggesting radiation therapy, hoping to reduce the abdominal mass. He states that he has had experience in radiation therapy for the splenomegaly, but not for the mesenteric mass.
My question to you is: do you have any experience in radiating erythropoietic mesenteric mass secondary to post-polycythemic myeloid metaplasia in order to reduce its size? I would appreciate your response.
Li Liu, MD, OncoLink Editorial Assistant, responds:
Dear Dr. K:
Thank you for your interest and question.
It is not uncommon to see patients with polycythemia vera presenting with extramedullary hematopoiesis. Radiation therapy can be used, but under certaincircumstances. First of all, your wife should have a very thorough physical examination and radiographic studies to fully evaluate the status of her disease. Tissue diagnosis prior to treatment to ensure correct diagnosis and rule out other causes for an abdominal mass may also be considered.
In addition, as you mentioned in your letter, your wife's bone marrow is fibrotic. If her bone marrow is not functioning at all, then this mesenteric mass could be the only focus of extramedullary hematopoiesis to keep her alive. Therefore irradiating this mass could cause fatal consequence. One physician at the University of Pennsylvania has treated several cases for pain due to these masses, with good results. Only one or two cases were treated to reduce the size of the mass, and these seemed to respond partially to somewhat higher doses.
I would recommend you and your wife consult a radiation oncologist to discuss the pros and cons of radiation treatment and to explore other treatment options as well.