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Aplastic anemia is a disorder that effects what cells? What is the end result? pluripotential stem cells that leads to bone marrow failure
The disorder of aplastic anemia features what? hypocellular bone marrow and pancytopenia (decreased circulating levels of all formed elements in the blood).
What is pancytopenia? bicytopenia? monocytopenia? when there is a reduction in all three marrow lineages (granulocytes, platelets and red blood cells); reduction of two lineages, one lineage; Note: aplastic anemia does not necessitate pancytopenia
What are the three etiologic groups of aplastic anemia? idiopathic (most common), acquired (drugs, chemicals,viral infections) ,inherited
Bone marrow failure may result from what two mechanisms? damage to or defects in the stem and progenitor cells in bone marrow,damage to or defects in the bone marrow stromal cells.
A consistent laboratory finding in aplastic anemia is the very low numbers of assayable bone marrow progenitor and stem cells
The survival and proliferation of hematopioetic cells are dependent on what cells? stromal marrow cells.
Are Stromal cells defective in aplastic anemia? No, and they produce normal quantities of hematopoietic growth factors.
What are the two major mechanisms invoked for aplastic anemia? Direct hemapoietic injury and immune-mediated bone failure
Describe direct hematopoietic injury as it applies to aplastic anemia? chemical(namely cancer chemo-cisplatin, mercaptopurine, vincristine) or physical agents (even viruses) injury directly impact both proliferating and quiescent hematopoietic cells. Such agents cause significant damage to DNA to result in apoptosis.
The term “idiosyncrasy” means... an unusual individual reaction to a drug or a food. Most marrow failure leading to aplastic anemia arise from complications from these drugs, which occur after a few weeks of initiation of drug use.
Describe immune-mediated bone marrow failure of aplastic anemia? stem cells are antigenically altered by exposure to drugs, infectious agents, or other unidentified environmental insults evoking cellular immune response, during which act. T cells produce cytokines (IF-γ and TNF) prevent normal stem cell growth/dev
In immune mediated aplastic anemia, blood of aplastic anemia contains... elevated levels of cyto T-cells (IFN and TNF which inhibit hematopoietic proliferation of progenitor and stem cells through cell death by apoptosis.)
Aplastic anemia is most frequent in what race? Oriental, Asian
What are pathological signs of aplastic anemia? There is a decrease in the number of cells of myeloid, erythroid, and megakaryocytic lineage, with a relative increase in lymphocytes and plasma cells and there is an increase in bone marrow fat
What characterizes aplastic anemia? Anemia, leukopenia (primarily granulocytopenia) and thrombocytopenia. Red cells have normal shape, but mildly macrocytic. Elevated EPO levels, reticulocytosis is not present. Lymphocytosis observed.
In aplastic anemia, what is seen in the bone marrow aspiration and biopsy specimen? hypocellular or acellular, loss of hematopoietic elements. (Considered severe if the bone marrow cellularity is reduced less than 30%.) Pathological examination of bone marrow biopsy specimen shows fat cell replacement of the bone marrow.
In aplastic anemia, what is seen in the peripheral blood smear? hypocellularity of the marrow is reflected in the peripheral blood smear, which shows granulocytopenia, and leukopenia as well as ample space between red cells, a reflection of this patient's anemia, and few, if any, platelets.
What are clinical features of aplastic anemia? similar to all anemia symptoms, pallor, dyspnea, fatigue, gingival bleeding, or skin bruises, recurrent infection
What are the tx for aplastic anemia? Pts req transfusion support until diagnosis. Tx infections with broad-based empirical antibiotic therapy. Bone marrow transplantation from a HLA-matched sibling donor is the treatment of choice for pts < 60 yrs. Immunosuppress if older than 60
Which drug is most likely to cause aplastic anemia? Phenylbutazone, a nonsteroidal antiinflammatory drug (NSAID) used for the treatment of pain of osteoarthritis
Anemia of chronic renal insufficiency reflects... decreased production of erythropoietin by the damaged kidneys.
What is the pathology of anemia of chronic renal disease? Normocytic and normochromic. In some cases, echinocytes (crenated cells, burr cells) with scalloped cell membranes can be seen. If renal insufficiency secondary to malignant hypertension, red cell fragmentation with formation of schistocytes may be seen.
What is the tx of choice for anemia of chronic renal disease? Administration of recombinant erythropoietin is the treatment of choice.
Anemia of renal failure results directly from... a deficiency in erythropoietin production, making it unique among the many types of anemias
Anemia of chronic disease arises from...And results from the release of what? chronic inflammatory and malignant conditions;of inflammatory cytokines (IL-1, TNF, IFN-gamma
What are some useful markers of anemia of chronic disease? in addtion to presence of cytokines...increased serum ferritin in conjunction with decreased serum iron due the cytokines is characteristic of anemia of chronic disease.
What is the pathology of the anemia of chronic disease? serum iron is reduced, and in contrast to iron deficiency anemia, total iron binding capacity also tends to be decreased (as is the serum albumin level).
What is myelophthisic anemia? refers to hypo proliferative anemia associated with infiltration of the bone marrow by a variety of processes.
What is seen with Myelophthisic Anemia? moderate to severe normocytic anemia, with anisopoikilocytosis and teardrop cells. Circulating immature granulocytes and nucleated red blood cells (leukoerythroblastosis). Extramedullary EPO may be seen.
Created by: jpop