Term
| What are 3 examples of intrinsic hemolytic anemias? |
|
Definition
| Three examples include spherocytosis, sickle cell dz, and g6pd. |
|
|
Term
| What are 3 examples of extrinsic hemolytic anemias? |
|
Definition
| 3 examples include warm/cold ab, microangiopathic (ttp, hus, mechanical valve), infection. |
|
|
Term
| What are 3 types of microangiopathic extrinsic hemolytic anemia? |
|
Definition
| 3 examples are ttp, hus, and mechanical valve. |
|
|
Term
| What are 3 examples of anemia d/t decreased hemoglobin synthesis? |
|
Definition
| 3 examples of microcytic anemia are fe deficiency, thalassemia, and chronic dz. |
|
|
Term
| What are 2 examples of decreased DNA synthesis causing anemia? |
|
Definition
| 2 examples of megaloblastic anemia include folate and B12 deficiency. |
|
|
Term
| What are 2 examples of anemia d/t stem cell disorders? |
|
Definition
| 2 examples of nonmegaloblastic macrocytic anemias include aplastic anemia and myeloproliferative leukemia. |
|
|
Term
| What are 2 examples of anemia d/t bone marrow infiltration? |
|
Definition
| 2 examples include carcinoma and lymphoma. |
|
|
Term
| What causes reticulocytosis in hemolytic anemias? |
|
Definition
| This is caused by the attempt of bone marrow to compensate for the decreased rbc ct. |
|
|
Term
| Why does haptoglobin decrease in hemolytic anemias? |
|
Definition
| This is caused by the binding of an increased number of RBC fragments so they can be removed. |
|
|
Term
| To what does intravascular hemoglobinemia lead? |
|
Definition
| If intravascular, this leads to hemoglobinuria and urine hemosiderin. |
|
|
Term
| Does indirect hyperbilirubinemia, as seen in hemolytic anemia, come from intrahepatic or extrahepatic processes? |
|
Definition
| This comes form extraheptic processes in hemolytic anemia. |
|
|
Term
| What liver enzyme is elevated in hemolytic anemia? |
|
Definition
| In this condition, *AST*, (as opposed to ALT) is elevated. |
|
|
Term
| Elevated LDH, elevated AST, reticulocytosis, and and elevated indirect bilirubin, are all characteristic of what dz? |
|
Definition
| These are all indicative of hemolytic anemia. |
|
|
Term
| What 2 intrinsic causes of hemolytic anemias are d/t membrane defects? |
|
Definition
| 2 results are include hereditary spherocytosis and pnh. |
|
|
Term
| What 2 intrinsic causes of hemolytic anemias are d/t oxygination? |
|
Definition
| 2 results are g6pd deficiency and methemoglobinemia. |
|
|
Term
| What mediates the antibody response against RBC membranes in autoimmune hemolytic anemia? |
|
Definition
| This is mediated by IgG and IgM (as opposed to IgA or IgE), or complement (C3 or C4), directed against RBC membrane. |
|
|
Term
| Toward what part of the RBC membrane is IgG, IgM or the complement system directed in autoimmune hemolytic anemia? |
|
Definition
| This is directed against a component of the Rh system. |
|
|
Term
| In autoimmune hemolytic anemia, which end of the Ab is recognized by macrophages? |
|
Definition
| The Fc end of the Ab is recognized. |
|
|
Term
| What forms d/t the damage caused to RBC membrane in autoimmune hemolytic anemia? |
|
Definition
| This causes the formation of a spherocyte. |
|
|
Term
| In autoimmune hemolytic anemia, where are the *spherocytes* hemolyzed? |
|
Definition
| The resultant spherocytes are subject to hemolysis in liver and spleen (extravascular hemolysis). |
|
|
Term
| What is the most common cause of autoimmune hemolytic anemia? |
|
Definition
| 1/2 of all cases are idiopathic. |
|
|
Term
| How fast is the onset of autoimmune hemolytic anemia? |
|
Definition
| The onset is rapid and profound. |
|
|
Term
| What are the 2 components of Evan's syndrome? |
|
Definition
| 2 components include: 1)ITP, and 2)Autoimmune Hemolytic Anemia. |
|
|
Term
| What type of Coomb's test ms presence of IgG on RBC membrane? |
|
Definition
| This is measured by a direct coomb's test. |
|
|
Term
| What type of Coomb's test measures free IgG in human serum? |
|
Definition
| This is measured by an indirect Coomb's test. |
|
|
Term
| What type of anemia is indicated by positive Coomb's tests? |
|
Definition
| Positive direct coomb's test indicates autoimmune hemolytic anemia. Positive indirect coomb's test indicates that all the rbc sites have been filled by abs and extra abs are circulating in blood stream. |
|
|
Term
| Pt presents w/rapid onset of CHF and angina. Pt is fatigued, jaundiced, and has splenomegally. CBC reveals hct of 9% and hgb of 3. rbcs are decreased, wbc is nml, platelets are nml, mcv is nml, mch is nml, mchc is nml, rdw is increased, and retic count is increased. Smear shows nucleated rbcs and spherocytes. Indirect bilirubin is elevated, ldh is elevated, ast is elevated, alt is nml. What additional test do you order and how do you tx the anemia? |
|
Definition
| This clinical scenario indicates autoimmuune hemolytic anemia. Order a coomb's test and tx w/transfusion and prednisone. |
|
|
Term
| Does microvascular hemolytic anemia cause intravascular or extravascular hemolysis? |
|
Definition
| This results in intravascular hemolysis. |
|
|
Term
| Hemoglobinuria, shistocytes, and helmet cells on smear are all results of what type of anemia? |
|
Definition
| These are indicative of microangiopathic hemolytica anemia. |
|
|
Term
| What are 3 causes of microangiopathic hemolytica anemia? |
|
Definition
| 3 causes include TTP, HUS, and DIC. |
|
|
Term
| Coagulopathy and thrombocytopenia may coexist with what type of anemia? |
|
Definition
| These coexist with microangiopathic hemolytic anemia. |
|
|
Term
| Mechanical heart valves may cause a chronic form of what type of anemia? To what other anemia can this lead? |
|
Definition
| This causes chronic microangiopathic hemolytic anemia and can lead to Fe deficiency anemia. |
|
|
Term
| Pigment gallstones are present in what 2 types of anemia? |
|
Definition
| 2 types include hereditary spherocytosis and sickle cell dz. |
|
|
Term
| Is a coomb's test positive or negative in hereditary spherocytosis? |
|
Definition
| This test is negative as most hemolysis is occuring in the spleen. |
|
|
Term
| The osmotic fragility test would be useful for what type of anemia? |
|
Definition
| This test would demostrate cells vulnerable to osmotic swelling and is positive in heeditary spherocytosis and maybe autoimmune hemolytic anemia. |
|
|
Term
| 34 yo m pt w/family hx of anemia presents w/ruq pain, jaundice, fatigue, tachycardia, easy fatiguability, palpitations, doe, and pallor. Splenomegally is noted on exam. Cbc shows hct of 35, hgb of 11, decreased rbc, nml wbc, nml platelets, decreased mcv, increased mch, increased mchc, increased rdw, and increased reticulocyte count. Peripheral blood smear shows spherocytosis. What do you suspect is causing his ruq pain, what additional test could you order, and how do you tx the anemia? |
|
Definition
| This clinical scenario describes hereditary spherocytosis. This causes pigment gallstones which can lead to attacks of cholecystitis. Order an osmotic fragility test and tx w/uninterrupted supplementation folic acid. The tx of choice is a splenectomy. |
|
|
Term
| What type of anemia is an acquired disorder of clonal stem cells? |
|
Definition
| This is the etiology of paroxysmal nocturnal hemoglobinuria. |
|
|
Term
| How are cells lysed in paroxysmal nocturnal hemoglobinuria? |
|
Definition
| Cells are lysed by complement, *not igG*. |
|
|
Term
| To what might paroxysmal nocturnal hemoglobinuria progress? |
|
Definition
| This may progress to *aplastic anemia*, myelodysplasia, or AML. |
|
|
Term
| What cellular structures are involved w/paroxysmal nocturnal hemoglobinuria? |
|
Definition
| This involves cell membranes and complement regulating proteins (CD55 and CD59) |
|
|
Term
| *Thrombosis* and *abdominal pain* are characteristic of what type of anemia? |
|
Definition
| These are 2 key characteristics of paroxysmal nocturnal hemoglobinuria. |
|
|
Term
| Which vv are involved in paroxysmal nocturnal hemoglobinuria thrombosis? |
|
Definition
| This involves platelet activation by complement in mesenteric and hepatic vv, saggital vv, and skin. |
|
|
Term
| When does episodic hemglobinuria generally occur? |
|
Definition
| This is a manifestation of paroxysmal nocturnal hemoglobinuria that occurs in the 1st morning void. |
|
|
Term
| A 63 yo m pt c/o central abodominal pain and bloody urine when he first wakes up. Pt is fatigued, tachycardic, has palpitations, doe, and pallor. CBC reveals hct of 35, hgb of 12, decreased rbc, decreased wbc, decreased platelet, nml mcb, nml mch, nmlmchc, nml rdw, and reticulocytosis. LDH is elevated. What other test do you order and how do you tx? |
|
Definition
| Order flow cytometry to detect diminished levels of CD59 on rbcs. Rx Fe supplementation and prednisone. If condition increases in severity, definitive tx tx is allogenic bone marrow transplant. |
|
|
Term
| What enzyme catalyzes NADPH production by hexose monophosphate shunt? |
|
Definition
| This is catalyzed by G6pd. |
|
|
Term
| What causes hgb oxidation? What is caused by hgb oxidation? |
|
Definition
| Hydrogen peroxide causes hgb oxidation. HOOH is not detoxified in g6pd deficiency. Oxidized hgb ppt to form heinz bodies, which damage rbc cell membrane. |
|
|
Term
| What happens to RBC in the presence of heinz bodies? |
|
Definition
| In g6pd deficiency, heinz bodies damage rbc membrane, and damaged cells are hemolyzed and removed by spleen. |
|
|
Term
| What anemia is more common in american black males? |
|
Definition
| g6dp deficiency is an x-linked recessive trait found in american black males. Females almost universally phenotypically nml. |
|
|
Term
| What are the usual chronic signs of hemolysis in g6pd deficiency? |
|
Definition
| In this anemia, there are usually no chronic signs of hemolysis. |
|
|
Term
| What triggers hemolytic episodes in g6pd deficiency? |
|
Definition
| Severe oxidative stress caused by infections or toxic drugs like sulfonimides trigger episodes. |
|
|
Term
| How are bite cells and heinz bodies visualized on smear? |
|
Definition
| These are typical of g6pd deficiency and are visualized by cresyl violet stain. |
|
|
Term
| How does an enzyme assay (fluorescent spot test or methemoglobin reduction assay change w/g6pd deficiency acute episodes and between episodes? |
|
Definition
| This is nml during acute episodes in g6pd deficiency, but is otherwise low. |
|
|
Term
| A 21 yo m black pt w/no pmi on bactram for a sinusitis infection presents w/mild anemia. What tests do you order, what do you expect them to show, and how do you tx? |
|
Definition
| This clinical scenario is typical of g6pd deficiency. Order CBC to demonstrate anemia, and expect bite cells and heinz bodies on smear when cresyl violet stain added. d/c bactram and start non-sulfonamide abx. |
|
|
Term
| When do rbcs stick to each other? |
|
Definition
| Nml cells do not stick to each other. Sickle cells do. |
|
|
Term
| How is sickle cell dz inherited? |
|
Definition
| This is an AR dz w/single nucleic A substitution of valine for glutamine on beta globin chain. |
|
|
Term
| What is nml hgb and what do individuals w/sickl cell dz carry? |
|
Definition
| Nml hgb is hgb aa. Sickle cell pts have hbg ss. |
|
|
Term
| In sickle cell dz, which give up o2 more readily: alpha or beta chains? |
|
Definition
| Beta chains give up o2 more readily. |
|
|
Term
| What occurs in sickle cell dz when beta chains give up o2? |
|
Definition
| When this occurs, cells become sticky and bind to other hgb molecules, which polymerize into rod-like structures, deforming and stiffening the rbc. RBC membrane is also damaged. |
|
|
Term
| What are 4 factors that affect the rate of sickling? |
|
Definition
| 4 factors include RBC dehydration, Hgb S concentration, hypoxia, and acidosis. |
|
|
Term
| Parents bring in their 1 yo m black child who has jaundice and an ulcer over his right shin. CBC shows hct of 27 and hgb of 9. RBC is decreased, wbc are increased, platelets are increased. MCV is nml, MCH is nml, MCHC is nml, RDW is increased, reticulocytes are increased. What other tests do you order and what results do you expect to find? How do you tx? |
|
Definition
| This pt scenario describes sickle cell dz. Smear should demonstrate sickle cells. In older pts in whom autoinfarction of the spleen has occured, howel-jolly bodies would also be seen. Pt should be maintained on folic A supplementation and given transfusions for aplastic or hemolytic crises. Keep pt well hydrated and avoid xs exercise. |
|
|
Term
| What sickle crisis involves marrow infection? |
|
Definition
| This is an aplastic crisis. |
|
|
Term
| What sickle crisis involves splenic or hepatic sequestration or comorbid dz like g6pd? |
|
Definition
| This is a hemolytic crisis. |
|
|
Term
| What sickle crisis involves triggers that include infection, hypoxia, and dehydration? |
|
Definition
| These trigger a vasocclusive crisis. |
|
|
Term
| What vaccine is necessary for sickle cell pts? |
|
Definition
| These pts require a pneumococcal vaccination. |
|
|
Term
| What is the only dz modifying drug for sickle cell dz? |
|
Definition
| The only dz modifying drug is hydroxyurea. |
|
|
Term
| What illness involves Hgb AS? |
|
Definition
| This is the genotype for sickle cell trait. |
|
|
Term
| What is teh phenotype for sickle cell trait? |
|
Definition
| These pts are lgly nml except w/extreme exertion at high altitudes. Hematuria is more common in these pts. |
|
|
Term
| What are the 3 forms of sickle thalassemia? |
|
Definition
| Three forms include sickle alpha thalassemia, sickle beta+ (milder than ss) and beta0 (similar to ss dz) thalassemia. |
|
|
Term
| In what genotype is DNA altered at the same site as in sickle cell dz? |
|
Definition
| This occurs w/hgb c disorders. |
|
|
Term
| Which has more severe hemolytic anemia: hgb c or ss? |
|
Definition
| Hgb C is a milder anemia than SS dz. |
|
|
Term
| Is retinopathy and avascular necrosis more severe in hgb c or ss? |
|
Definition
| This is more severe in Hgb C. |
|
|
Term
| What cell abnml causes aplastic anemia and in what does this result? |
|
Definition
| Abnml of pluipotent stem cells result in pancytopenia and hypoplastic bone marrow. |
|
|
Term
| What is the most common cause of aplastic anemia? |
|
Definition
| This is most commonly caused by T-cell mediated stem cell supression. |
|
|
Term
| 35 yo f pt c/o of sob, mucosal bleeding, and petechiae. PE is (-) for organomegally and bone pain. CBC shows hct 20, hgb 7, low rbc, wbc 450 (low), platelet of 18,000, nml mch, nml mcv, nml mchc, nml rdw, reticulocytes 0.5%. How do you dx and how do you tx? |
|
Definition
| This clinical scenario suggests severe aplastic anemia. Dx w/bone marrow aspirate, which should be hypocelluar w/no abnml cells. Tx w/bone marrow transpant and transfusions. |
|
|
Term
| How do you tx older pt w/severe aplastic anemia? |
|
Definition
| These pts should receive immunosuppressive tx: antithymocyte clobulin, cyclosporine, and cyclophsophamide. |
|
|
Term
| To what may chronic aplastic anemia degenerate? |
|
Definition
| This may degenerate into pnh or myelodysplasia. |
|
|
