TAILIEUCHUNG - Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes

The hypoproliferative anemias are normochromic, normocytic or macrocytic and are characterized by a low reticulocyte count. Deficient production of RBCs occurs with marrow damage and dysfunction, which may be secondary to infection, inflammation, and cancer. Hypoproliferative anemia is also a prominent feature of hematologic diseases that are described as bone marrow failure states; these include aplastic anemia, myelodysplasia (MDS), pure red cell aplasia (PRCA), and myelophthisis. | Chapter 102. Aplastic Anemia Myelodysplasia and Related Bone Marrow Failure Syndromes The hypoproliferative anemias are normochromic normocytic or macrocytic and are characterized by a low reticulocyte count. Deficient production of RBCs occurs with marrow damage and dysfunction which may be secondary to infection inflammation and cancer. Hypoproliferative anemia is also a prominent feature of hematologic diseases that are described as bone marrow failure states these include aplastic anemia myelodysplasia MDS pure red cell aplasia PRCA and myelophthisis. Anemia in these disorders is often not a solitary or even the major hematologic finding. More frequent in bone marrow failure is pancytopenia anemia leukopenia and thrombocytopenia. Low blood counts in the marrow failure diseases result from deficient hematopoiesis as distinguished from blood count depression due to peripheral destruction of red cells hemolytic anemias platelets idiopathic thrombocytopenic purpura or due to splenomegaly and granulocytes as in the immune leukopenias . Hematopoietic failure syndromes are classified by dominant morphologic features of the bone marrow Table 102-1 . While practical distinction among these syndromes usually is clear they can occur secondary to other diseases and some processes are so closely related that the diagnosis may be complex. Patients may seem to suffer from two or three related diseases simultaneously or one diagnosis may appear to evolve into another. Many of these syndromes share an immune-mediated mechanism of marrow destruction and some element of genomic instability resulting in a higher rate of malignant transformation. Table 102-1 Differential Diagnosis of Pancytopenia Pancytopenia with Hypocellular Bone Marrow Acquired aplastic anemia Constitutional aplastic anemia Fanconi s anemia dyskeratosis congenita Some myelodysplasia Rare aleukemic leukemia AML Some acute lymphoid leukemia Some lymphomas of bone marrow Pancytopenia with Cellular Bone Marrow .

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