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Professors' Posts Sideroblastic anemia
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| Terry Hamblin |
Gosh, for day 1 of my being on the list you lot are sure turning up some interesting problems. Sideroblastic anemia is a form of myelodysplastic syndrome (MDS). MDS is about as common as CLL and Trevor is about right when he says it's like CLL of a different color. Sideroblastic anaemia has been known about for about 100 years, but it was only put together with the other forms of MDS in about 1981. MDS is a group of diseases in which something goes wrong in the bone marrow with the production of red cells, white cells and platelets. They don't look right down the microscope, they don't perform right in the blood, and they don't last as long as they should. As a result the patient is anemic and may develop a low white count leaving him open to infection and a low platelet count which may cause bleeding. Then there is this tendency to turn into acute myeloblastic leukemia. there are 5 types of MDS and happily for Trevor sideroblatic anemia is the one that's least likely to turn into leukemia. In sideroblastic anemia there is an accumulation of iron in the red cell precursors. Unlike mature red cells, the precursors have nuclei, and the iron accumulates in a ring around the nucleus (that's why they are known as ring sideroblasts). There are two types of sideroblastic anemia according to the new WHO classification: those whose white cell and platelet precursors are normal and those who have dysplastic white cell and platelet precursors. It is only the latter type that has a risk of turning into leukemia. Sideroblastic anemia occurs in association with CLL in rare cases. We wrote a paper about this in 1986 Immunological abnormalities in myelodysplasia: II. Co‑existent lymphoid or plasma cell neoplasms. A report of 20 cases unrelated to chemotherapy. JA Copplestone, GJ Mufti, TJ Hamblin and DG Oscier. British Journal of Haematology 1986 63: 149‑159. I seem to remember a paper from Danny Catovsky on this topic in 1974. But sideroblastic anemia may also occur as a consequence of chemotherapy. We don't really know how common it is to have both diseases because it's hard to see the abnormalities of red cell precursors in a bone marrow stuffed full of small lymphocytes. What is likely to happen? The anemia of sideroblastic anemia is often mild and doesn't need treatment. If it does than about 50% will respond to the combination of erythropoietin and G-CSF. If this doesn't work, or if you can't get it paid for, then it's a matter of blood transfusions. |