Term
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Definition
| disroder that is decreased red cells in marrow, without effect on other heompatoietic cell types. Causes; autoimmune. Secondary Tgamma disease- wherem alignancy of cd8 t cells attacks rbcs, parvovirus infection, thymoma- tumors of thymus gland- self reactive cd8 t cells from this. |
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Term
| nutritional deficiencies of anemia |
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Definition
| vitamin b12 , folic acid deficiency |
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Term
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Definition
| most common cause of anemia Cause: bleeding - GI tract, reproductive tract,pregnancy. |
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Term
| anemia due to increased destruction |
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Definition
| blood loss, hemolysis, splenic sequestration. |
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Term
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Definition
| average rbc size (between 80 and 100 normally). Microcytic anemia- <80, Macrocytic anemia>100, normocytic - 80-100 |
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Term
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Definition
| variation in size of red cells - anemia of eelevated rdw means bone marrow is spitting out cells of various sizes (Anisocytosis)- evidence of pathological process. |
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Term
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Definition
| reticulcytes- rbcs that are young, haave not degraded RER. When stress upon erythron happens, huge stimaultion of rbc produciton results in graeter numbers of reticulocytes- this will happen in blood loss or hemolysis for example, clues you in that it is on BM disorder. |
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Term
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Definition
| when rbcs are degraded, they release heme ( hemoglobin is toxic to renal tubules) which is eventually converted to biliribuin, conjugated byliver and excrted. Therefore high levels of bilirubin are signs of hemolysis- severy hemolysis results in rise of unconjugated, as the liver cant keep up |
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Term
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Definition
| rbcs rich in LDH, so when lyse, LdH will be increased- need tofracitonate ldh to be sure it is rb. |
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Term
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Definition
| number of reticulocytes/100 wbc. |
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Term
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Definition
| RC X measured hematocrit/45. Accounts for level of anemia- and the higher level of response from reticulocytes that should be happening. |
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Term
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Definition
| produced by liver ,free protein which sopps u free hemoglobin sincei ti is toic to renal tubule, depresed levelsm eans it is being used up and is probably icnraesedh emolsyis unless hve liver fialure. |
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Term
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Definition
| urine exam- iron pigment in urine , iron from hemoglobin that was lysed from red cells and incorporated into plasma cell - seen in INTRAVAsCULAR HEMOLYSIS |
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Term
| intravascular vs. extravascular |
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Definition
| intravascular is within the vessels, extravascular- is within liver and spleen - hemolsyis extravascularly mediated by macrophages - this is due to autoimmune hemolysis which opsonates for macrophages- if only partially get destroyed and are able to heal rest, rbcs become spherocytes. Intravascullarly- they are known as shistocytes (sheared up) |
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Term
| causes o intravascular hemolysis |
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Definition
| Sickle ceel anemia, microangiopathy. |
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Term
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Definition
| gi blood, gu blood loss, preganncy, respiratory blood loss, trauma blood loss, inadequate dietary iron, growth, itnestianl malabsorption |
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Term
| histology of iron deficiency |
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Definition
| hypochoroia ( greater central pallor than normal), schistocytes, cigar cells (pathegnemonic for iron deficiency). |
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Term
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Definition
| iron deficiency, anemica of crhonci infalmamtory disease, lead poisoning, thalassemias, siderobalstic anemia , myelodyspaltic syndromes. |
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Term
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Definition
| apoferretin plus iron boudn to it, good measurement ofiron stores; may be elevated in acute inflammation. Will remain normal/high. Serum iron levels may also be meaured hoeverhtey may lasobe depressed due to chronic inflammation. |
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Term
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Definition
| levels go up in iron deficiency because ryign to grab as much as possible, also will go up if injected with erythropoetin. . Transferrin levels are normal in anemai of chronci disease |
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Term
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Definition
| go up when iron stores getting depleted. |
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Term
| MCV during irondefifcieicny |
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Definition
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Term
| anemia of chronic inflammatory disease |
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Definition
| rhematoid artiritis, SLE, Chronic infections (tuberculosis bacterial abscesses, deep fungal diseases, disseminated malignancies hiv) |
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Term
| MOA of chronic inflammatory disease anemia |
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Definition
| Th1 (inflammatory): Il-1 and tnfa stimulate hypoferritenmia and anemia . Th2- antiinflammatory cytokines :il-4 and il-10 block ifngamma activation of IRP, thery providing a sink for iron. |
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Term
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Definition
| regulation of iron aborption. Higher hepcidin , less iron absorped in gut. Hepcidin levels elevated in chronic inflammatory anemia. |
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Term
| differences in lab values between anemia of chronic dsiease and iron deficency anemia (fe, transferretin, saturation, ferritin, transerretin receptor) |
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Definition
| both low iron levels, plasma transferrin is high in iron deficiency, it is low or normal in chronic disease, tansferretin saturation is low in iron deficiency , low/normal in chronic disease, ferritin is low in iron deficiency, normal/high in anemia of chronic disease, transferretin receptor is high in iron deficiency, nromal in chronic disease. |
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Term
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Definition
| megaloblastic anemia. Causes: autoimmune- antibodies to parietal cells, this inhibits intrinsic factor release, -this is associatedwith gastric neoplasia. Secondary to surgical resectin of the stomach (often accompanied by iron deficiency, secondary to small bowel disease (i.e. IBD, Crohns disease), diphyllobhtrium latum (gobbles up b-12), genetic deficienis of colbamaine transporters. |
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Term
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Definition
| megaloblastic anemia. Causes: inadequate diet (rare), increased need due to hemolysis, increased loss due to dialysis (dilating concentration that is in body), malabsorption due to bowel disease, drug toxicity. |
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Term
| cytoplasm-nuclear mistmach |
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Definition
| sign of either b12, or folate deficiency, both are requrired for creation of T bases, so cytoplasm will mature normally in rbcs, with delayed maturation of nucleus , resulting in large cells with a lot of cytoplasmw and no real nucleus. |
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Term
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Definition
| sign of b12 deficiency, as iti s required to metabolize homocysteine. |
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Term
| neutrophil hypersegmentation (histologY0 |
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Definition
| If neutrophil has 6-7 nucleations, this is suggestive of b12/folate deficiency. Should know this for exam. |
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Term
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Definition
| anemia of acute blood loss (before marrow has respodned to produce reticulocytes, anemia of chronic inflammatory disease (in about half the cases) , anemia of renal insufficiency, anemia due to myelodysplasia, sideroblastic anemia. |
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Term
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Definition
| rigned sideroblasts in bone marrow (at least fifteen percent of erythrocytes). Nucleated immature proerythroblasts with a ring of iron pigmentation around nucleus. Needs prussian blue stain. |
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Term
| Sideroblastic anemia causes |
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Definition
| isoniazid treatment, heavy alcohol use, myelodysplasia, familial, idiopathic. |
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Term
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Definition
| RBC membane defects: spherocytosis (due to lack of spectrin), elliptocytosis (spectrin or band 4.1 defects), herditary pryopoikilcytosis. Globin chain defects: Alpha thalassemias, beta Thalassemias. Red cell enzimopathies: G6PD defiicney, pyruvate kianse deficiency Hemolytic anemia due to hemoglobin mutations: i.e. sickle cell anemia, Hemolytic anemia due to autoimmune proceeses : medication induced (hapten, direct anti-RBC, antibodies formed as a result of plasma protein-drug complex inblood), autoimmune disease, or lymphoproliferative disease (CLL/ALL lymphocytes created anti-rbc antibodies) |
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Term
| Hemolysis: confirmatory studies |
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Definition
| abnormal cells in peripheral smeas (disadvantage: may be masked by transfusion which always appears as spherocytes), haptoglobin: depressed (but always low in liver disease) , LDH elevated (in low grade lysis might be normal), Retic count elevated ( in production disorder will remain low), bilirubin elevated (in low grade hemolysis normal), urine hemosiderin present (normal levels in extravascular hemolysis). |
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Term
| nucleated Rbc histology (look at slide) |
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Definition
| nucleated RBCS are almost always evidence of something pathological, definetly not something normal. |
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Term
| spherocytosis Histology (slide) |
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Definition
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Term
| Thalassemia histology (sldie) |
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Definition
| hypochromia and target cells (can also be seen in iron deficiency) |
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Term
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Definition
| Hemoglobin A: a2b2, Hemoglobin a2: a2delta2, Hemoglobin fetal: a2gamma2 |
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Term
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Definition
| usually huge chunks of DNA are dleted- corresponds to . Apositive- a2 mutation, one gene out of four parent copies deleted (silent muatation). Homozygous alpha plus (a1) - corresponds to a trans mutatino- one gene from each parent deleted (mild anemia0, microcytic ) also a0 trait (two from one parent deleted). if Have 3 delted leads to HbH hemoglobin with mroe severe anemia and microcytosis |
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Term
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Definition
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Term
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Definition
| Formed due to A- Thallasemia, due to two gene deletion, less alpha chain is formed, so more b-chain is formed. They form tetramers and end up gathering in cytoplasm, forming inclusion bodies. Macropahges interpret this as foreign so try to gobble them up, hwoever not all of them die, adn simpyly reseal themselves- forming microcytic cells . |
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Term
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Definition
| thalasemia major- doenst matter whether a or b, so severe that are transfusion depepndant, thalassemia minor- asymoptomatic. Intermedia- very symptomatic may require transfusion from tiem to tie but do not depend on it to live. For all three- will see microcytosis, but red blood count may actually be normal or mildly anemic for first two, despite the fact that the rbcs are microcytic. |
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Term
| Thalassemia complciations |
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Definition
| mostly from blood transfusion; iron overlaodaffecting a bunch of organs- such as heart and liver. Splenomegaly from extramedullary hematopoeisis seen in major na d intermedia, endocrine complciatioans (diabetesmellitus), bone deformities from exuberant hematopoeisis in bone marrow |
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Term
| look at SS histology: mutation |
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Definition
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Term
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Definition
| dexogyenated rbcs change shape, when change shape start stacking, they puncutre eachother and the cells becoem dehydrated, continue to stack and eventually plug up blood vessels , causing organ damage everywhere. |
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Term
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Definition
| look at chart: anemia, proliferation of BM leading ot boneabnormalities, impaired mental function, orgn dysfunction (brain ifnarcts, spleen infarct, heart failure, muslce and joint dmage), enlargement of splee and eventual fibrosis. |
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Term
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Definition
| Rabbit antibody to our antibodies. If there are antibodies on RBC , coombs reagant willlbind to FC portion of these antibodies and join them together, resulting in RBC clumping. Direct coombs; identifes antibodies on patietns rbcs. Indirect: identifies natibodies on patients serum. |
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Term
| Thermal amplitude of hemoglobin : |
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Definition
| optimal temperature at which antibodies lyse hemoglobin. Warm is mostly IgG, Cold is mostly IgM |
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Term
| Intravscular hemolysis histology |
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Definition
| shcistocyte, look at slide |
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Term
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Definition
| any process yielding schistoyctes (resulting in itnravascualr hemolysis) |
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Term
| Hemolytic anemia due to parasitemia |
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Definition
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Term
| Myelodysplastic syndromes definition: |
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Definition
| clonal myeloid disroders that result in cytopenias, qualitative disorders of blood cells/ and precursors, and some prediliction to going on toacute myelodi luekemia (has nothing to do with lympohoid lineage). Also results in qualititative defects. |
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Term
| myelodysplasia clinical features |
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Definition
| Suspect myelodysplasia with anemia that is otherwise unexlpainable. With a hsitory of expoure to aromatic hydrocarbons or toxins, older age, pancytopenia and dyssplatic features in neutrophils (somtimes looks like b12/folate deficiency, even though patient doesnt ahve this defiicency) , must always look at marrow and see funny looking bone marow , also do cytogenetics (deletion in chrosome 7 is bad) and immunophenotype. sometimes have myeloproliferative/myelodysplastic disroder together (rare). |
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Term
| Myelodysplastic syndromes histology |
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Definition
| (slides)- nuclear cytoplasmic mismatch (rbcs that aere this pink and small shouldn’t haven uclei at all, instead here nuclcei are appearing in these rbcs that would fit a cel wlith more cytoplasm that is actually blue. (slide2)- hypersegmented neutrophiles, more than 2 or 3 segments, normally characeristic of b12/folate defiicency- this is a megaloblastoid cell. also hypogranular cell. (slide 3)- refractory anemia with excess blasts. (slide 4) = ringed sideroblasts- erythroblasts in BM. (slide 5) - myelomonocytic luekemia- several monocytes and myeloid cells- cell in the middle is apromyelocyte. -all stages of maturation present. |
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Term
| Refractory anemia with multilineage dysplasia |
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Definition
| patients anemic, funny looking megakarcyotics and precurors ( less than five percent blasts)- multilineage dysplasia . Don’t do as well as refractory anemia patients |
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Term
| Sideroblastic anemia myelodysplastic. |
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Definition
| not familial, not drug related or acquired. Primary sideroblastic anemia (mutationally)- do reasonably well. |
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Term
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Definition
| higher percentaage of blasts than normal (blasts 5-10 percent), has possibly of going into AML. |
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Term
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Definition
| blasts 11-20 percent. Higher risk ofAML- a worse survival - . year or so. Once over 20 percent, haveaml. |
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Term
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Definition
mutation in ribosomal protein (rps14) critical in rna and dna metabolism. Deletions in chrosome 5. Predominantly women,elderly, anemia, but have normal to elevated platelet count . Drug available that improves blood counts for this condition.
female preponderance, severe anemia, prononouncd macrocytosis, moderatlye decreased luekocyte count and normal, noraml or moderatly increased platelet count rare acute myelodi transformation is rare, good survival. Bone marow features;: large monolobulated megakaryocytes, very little blast excess. RPS |
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Term
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Definition
| overmethylatio of genes, silencing them. Polymorphisms in variable methylatiosn explaisn the heterogeniety of myelodysplasia. |
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Term
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Definition
| empty bone amrrow and pancytopenia (look at slide). Types: fanconis anemia ,dyskeratosis congenita. Can be cuased by enviornmental toxins, medications, viruses and autoimmune idsease. |
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Term
| aplastic anemia major cause |
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Definition
| aplastic anemia: abnormal t cells- if can immunosuppress the mmay be able to get normal hematopoeisis. |
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Term
| paroxymal nocturnal hemagolbinuria cause |
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Definition
| mutation in clone of cels impacts adversley with way you anchorp roteins . Increased sensitivty to hemolysis- because missing cd55 and 69 |
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Term
| anemia due to decreased production |
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Definition
| aplastic anemia luekemias and myeloproliferative, myelodysplastic , myelophtisic anemias- marrow infiltraiton by cancers form otehr organs . can also be speciifco to erythron: prue red cell aplasia, nutritioan ldeficeinces, iron deficiency . |
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Term
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Definition
| Fatigue dyspnea, palpitations ,pica , neurolgooci deficits, vit b12 deficiency. early satiety (due to splenmegaly). tea colored urine. Pallor ofskin and conjunctiva, tachypnea on extertion, tachycardia, nail deformities and glossitis neruologic deicits palbable spleen jaundice |
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Term
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Definition
| Fatigue dyspnea, palpitations ,pica , neurolgooci deficits, vit b12 deficiency. early satiety (due to splenmegaly). tea colored urine. Pallor ofskin and conjunctiva, tachypnea on extertion, tachycardia, nail deformities and glossitis neruologic deicits palbable spleen jaundice |
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Term
| table showign what to do iwth v rrious retic counts |
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Definition
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Term
| basophicllic stipplign (slide) |
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Definition
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Term
| high retic and normal bilirubin |
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Definition
| reposnding adeuqately to blood loss, or simply just responding to therapy (i.e. high EPO). |
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Term
| causes of intravascular anemia |
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Definition
Dysfunctional mechanical heart valve
“March” hemoglobinemia
Hyperthermia
Thrombotic Thrombocytopenic Purpura
Hemolytic-Uremic Syndrome
Disseminated Intravascular Coagulation
Drug induced microangiopathic hemolysis (Cyclosporin A, Mitomycin C,
Ticlopidine) |
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