Extramedullary Hematopoiesis in Patients with Primary Myelofibrosis Rare and Serious Complications

Abstract

Abstract Extra Medullary Hematopoiesis 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, polycythemia vera ,myelodysplastic syndrome and myelofibrosis. To the best of our knowledge, there are very few documented case reports in the literature of patients with myelofibrosis who developed cord compressive symptoms due to EMH in the spine from 1958 till 2016 only 15 cases were reported, In reported cases male to female ratio 7:1 with age range between 30 and 75 year old Paraprasesis are the most common presenting symptoms Thoracic region is the most affected site The degree of anemia and bone marrow fibrosis does not correlate with the occurrence of EMH since it was reported in cases with early fibrosis or mild anemia. Correlation between molecular markers like JAK2 and CALR, MPL is not described 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 consensus for treatment and definition of CR, PR or No response in EMH with MF. Disclosures No relevant conflicts of interest to declare.