Extra Medullary Hematopoiesis in Patients with Polycythemia Vera Rare and Unusual Presentations

Abstract

Extramedullary Hematopoisis refers to the presence of hemopoietic elements in locations other than the bone marrow medullary space, it may be seen in many conditions, including chronic hemolytic anemias like thalassemia Intermedia or major. The common sites of involvement are the liver, spleen and the lymph nodes. Less common sites include the central nervous system, adrenal gland, kidney, perirenal soft tissues, breast, peritoneum and gastrointestinal tract the paraspinal region is a relatively uncommon location for these hemopoietic deposits. There are a few case reports of EMH occurring in the spineMost cases have been reported in connection with thalassemia, although it has also been described in patients with sickle cell anemia,myelodysplastic syndrome and myelofibrosis. To the best of our knowledge, there are very few documented case reports in the literature of patients with polycythemia vera and Extra medullary Hemayopoiesis.The reported cases are limited in this regard only 11 cases were reported from 1979 till 2015, Paraprasesis is the most common presenting symptom, Age between 30 to 75 year with Male to female 6:1,Thoracic region is most affected site Most cases treated by surgery ,radiotherapy or both Poorer outcome compared to MF casesOverall the mortality in EMH with polycythemia vera is higher than MF and thalassemia, some of them due to fatal location of cord compression (cervical)No definite guidelines have as yet been formulated for the treatment of such patients with EMH. Excision and decompression, radiotherapy, hypertransfusion and hydroxyurea alone or in combination have been proposed as well as lack of consensus about what is complete remission is it clinical or clinical and radiological and what is partial response is it any reduction of the mass lesion or any degree of clinical improvement in conclusion there is necessity to establish consus for treatment and definition of CR ,PR or No response in EMH with polycythemia vera. DisclosuresNo relevant conflicts of interest to declare.