Term
| What is the normal range of values for RBCs, Hemoglobin, Hematocrit, and reticulocytes for men and women? |
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Definition
| RBCs - 4.4-6, 3.8-5; Hemoglobin - 13-18, 12-16; Hematocrit 40-52, 35-47; Reticulocytes - .5-1.5% |
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Term
| What is the reticulocyte index? |
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Definition
| It allows for assessment of erythrocyte kinetics in normal patients, and has one correction for degree of anemia and a second for reticulocyte life span if a certain amount of RBCs are present. |
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Term
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Definition
| An immature RBC with residual ribosomal RNA. |
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Term
| Folate deficiency can cause what type of anemia? |
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Definition
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Term
| T or F: Chronic disease and inflammation can lead to anemia because of decreased RBC production and cytokine response via the body/macrophages to withhold iron from microorganisms and also the blood. |
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Definition
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Term
| Name this type of anemia - most common type, characterized by reticulocytopenia, low or normal MCV, induced by elements that impair the production of hemoglobin, and can range from disorders of erythrocyte and hemoglobin production to the bone marrow microenvironment. |
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Definition
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Term
| Name this type of anemia - most common cause of anemia, microcytosis, targets, aniso/poikylocytosis, and negative iron stain of marrow, can be caused by chronic blood loss, infancy, lactation, or pregnancy. |
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Definition
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Term
| Which of the following could be causes of folate deficiency- nutritional, alcohol and liver disease, infancy due to boiled milk, pregnancy and lactation, drugs, or malabsorption. |
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Definition
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Term
| What molecule requires intrinsic factor from parietal cells to be absorbed, is critical to RBC synthesis, causes megaloblastic anemia in deficit, neurologic and sensory symptoms with possible mucosal changes when deficient. |
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Definition
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Term
| Which of the following anemias can be classified as mircocytic and hypochromic - iron deficiency, chronic disease, sideroblastic, or thalassemia anemia. |
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Definition
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Term
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Definition
| The proportion of blood occupied by erythrocytes after centrifugation. |
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Term
| What does it mean if the MCV or MCHC is low? |
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Definition
| Microcytosis, hypochromia |
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Term
| __________ ____________anemias usually reflect a defect in hemoglobin synthesis and can be seen with: Iron deficiency, thalassemias, certain hemoglobinopathies, lead intoxication, sideroblastic anemias, and anemias associated with chronic diseases. |
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Definition
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Term
| _________ _________ usually reflect problems or delay in nuclear maturation. Can be seen with B-12 or folic acid deficiencies; also can be seen in a group of disorders known as "refractory anemias". |
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Definition
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Term
| Why is serum ferritin a good prognostic indicator of a bodys iron levels? |
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Definition
| Because it is in equilibrium with storage ferritin |
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Term
| What molecule transports iron in the blood to the bone marrow for RBCs to bind? |
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Definition
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Term
| What cell is the storage cell of iron in the marrow? |
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Definition
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Term
| Iron builds up in the mitochondria of the developing RBC's, encircles the nucleus in a ring, this abnormality is called what? |
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Definition
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Term
| T or F: the major adult hemoglobin is Hg A with a2b2 chains and the major fetal hemoglobin is Hg F with a2y2 chains. |
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Definition
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Term
| A change in the amino acid of hemoglobin can lead to which of the following results - increased Hg precipitation, instability, inability to keep iron in the reduced form, of altered oxygen affinity. |
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Definition
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Term
| Name this hereditary anemia - decrease in the synthesis of one or more globin chains resulting in an unbalance, protects against malaria, excess globin chains precipitate and cause RBC lysis and tissue iron overload, and a decreased hemoglobin production. |
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Definition
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Term
| Which of these erythrocyte infections can cause hemolysis - malaria, babesia, bartonella, or visceral leishmaniasis? |
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Definition
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Term
| RBC membrane abnormalities either congential or acquired lead to the stereotypical morphology known as ____________? |
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Definition
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Term
| Deficiency of this enzyme is the most common RBC enzyme defects in hemolytic anemias, since RBCs depend on glycolysis for energy and are unable to protect themselves against oxidant stress or drugs. |
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Definition
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Term
| Name this hemoglobinopathy - Hg S forms due to incorrect beta globin, 10% of blacks, hemoglobin normal full of O2 but precips when deoxy, heterozygous asymptomatic, homos have vaso-oclusive findings and pain, causes low grade anemia, splenomegaly, dactylitis, and hyperbilirubinemia. |
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Definition
| Sickle cell, can cause painful bone crises or functional asplenia leaving the patient vulnerable to pneumococcal, salmonella, and parvovirus infections |
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Term
| Name this hemoglobinopathy - decreased production of Beta chains, Mediterranean heritage, homos major, heteros minor, increased Hg A2/F, decreased Hg A, clinical findings include anemia, hepatosplenomegaly, hypercellular marrow with bone changes, iron overload, and RBCs show aniso/poikylo/reticulcytosis with microcytic hypochromic RBCs and target cells. |
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Definition
| Beta- thalassemia, treated with transfusions and iron chelation; note Heinz body inclusions form causing cells to be lysed in spleen |
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Term
| Name this hemoglobinopathy - decreased production of alpha chains, seen southeast Asians, there are 2 alleles for the synthesis so if all 4 affected then hydrops fetalis, if 3 then Hg H disease with 4 beta chains, if 2 then alpha-minor with mild microcytic/hypochromic known as alpha-trait, if 1 is a silent carrier that is usually asymp. |
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Definition
| Alpha-thalassemia; can form homotetrameric Hb, like b4 which are ineffective o2 carriers |
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Term
| What are the four serine protease enzymes in the clotting cascade that are made in the liver and are Ca and Vitamin K dependent? |
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Definition
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Term
| What are the cofactors for clotting proteins 9 and 10? |
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Definition
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Term
| Why is vitamin K essential for the clotting cascade? |
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Definition
| Because it is a cofactor for a liver carboxylase enzyme that adds a carboxy group to the four proteases allowing them to bind Ca |
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Term
| How does coumadin block the clotting cascade? |
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Definition
| It inhibits the liver enzyme carboxylase that make the clotting proteases able to bind Ca |
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Term
| Neither enzymes or cofactors are consumed in clotting so impairment doesn’t occur until very low levels (<30%) or with recessive/Xlinked phenotypes, but what two proteins are consumed so that mutations can act as an autosomal dominant error? |
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Definition
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Term
| The clinical findings of petechiae, purpura, history or bruising or mild membrane bleeding indicate a dysfunction of what blood product? |
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Definition
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Term
| In thrombocythemia has both a primary (essential) or secondary cause, how do they differ? |
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Definition
| Primary is due to inborn myeloproliferative disease causing excessive platelets that are either normal or not, Secdonary is excessive normal platelets being released from marrow due to stress or steroids |
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Term
| Which of the following are possible causes of thrombocytopenia due to limited production or destruction - marrow replacement, aplasia, viral infections, drugs, congenital, prosthetic valves, hypersplenism, immune mediation, DIC. |
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Definition
| All of them, last four are destruction causes, rest are production limiters |
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Term
| T or F: the chronic immune thrombocytopenia differs from acute in that it occurs for 20-40 age, 3F:1M, gradual onset, no clear antecedent, 20-100,000 plts, lasts years, remission rare, response to immunosuppresion or splenectomy is common. |
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Definition
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Term
| T or F: testing bleeding time is only done if platelets are greater than 100k or else the time is prolonged. |
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Definition
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Term
| Describe whether the following congenital bleeding disorders are autosomal dominant, recessive, or X-linked - Von Willebrand’s Disease, Hemophilia A (F8) or B (F9), all other factors, fibrinogen. |
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Definition
| Dom, X recessive, recessive, dom or recessive |
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Term
| Von Willebrands Factor is made in endothelial cells and functions to glue platelets down in primary hemostasis but it also stabilizes what cofactor in the cascade that can affect secondary hemostasis? |
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Definition
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Term
| Of the following clinical findings in clotting factor deficiencies ID which are common or rare - bleeding in major muscles or joints, large bruises, mucosal hemorrhage, intracranial bleeds, bleeding from minor cuts and abrasions. |
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Definition
| The first two are common, rest are rare |
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Term
| Describe what the following regulators of coagulation affect in the clotting cascade - Plasmin, Protein C/S, Anti-Thrombin III. |
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Definition
| Plasmin degrades fibrin, C/S are enzyme cofactor team that degrade F8 and F5, ATIII in the presence of heparin inhibits the serine proteases except F7 |
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Term
| T or F: All thrombotic coagulation disorders such as Protein C/S/ATIII deficiency, antiphospholipid, or homocysteinemia are auto dom with incomplete penetrance. |
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Definition
| True, most symptomatic patients have more than one mutation. |
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Term
| Two blood test are prothrombin time and partial thromboplastin time, which clotting pathway does each test for? |
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Definition
| PT is extrinsic, PTT is intrinsic |
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Term
| Proteases, intravascular tissue factor, and endothelial damage can all cause a DIC resulting in both clotting and fibrinolysis, so what is the result? |
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Definition
| Microvascular thrombi leading to tissue injury and consumption of platelets and coagulation factors leading to hemorrhage. |
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Term
| List the steps in primary hemostasis. |
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Definition
| Platelet adhesion due to vWF, shape change and activation, ADP and TXA2 granule release, recruitment, aggregation, and procoag activity |
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Term
| What triggers the intrinsic and extrinsic clotting pathway? |
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Definition
| Intrinsic is exposed collagen, extrinsic is tissue factor from damage |
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Term
| The alpha hemoglobin subunit is matched to chrome ______ and the beta is on chrome ______ . |
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Definition
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Term
| The alpha hemoglobin subunit has 4 genes whereas the beta unit has 2, so how does this affect phenotypic observation of the defects? |
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Definition
| The beta subunit requires less mutations to lose functionality and alpha is apparent in the fetal stage because it is expressed from conception while beta is not noticed until after birth |
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Term
| T or F: Hereditary fetal hemoglobin is when a mutation impairs the y to beta switch in hemoglobin yielding more HbF and it is clinically very symptomatic. |
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Definition
| F, it is asymptomatic because HbF carries O2 well |
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Term
| T or F: In beta-thalassemia major b0 means no HbA is present whereas b+ means there is some formation. |
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Definition
| T, so if a person has one sickle cell gene and a b-thala gene then those determinants will either cause full sickle cell or milder |
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Term
| T or F: b-thal homozygotes who also inherit an a-thal allele may have less severe b-thalassemia, due to less imbalance or reduced excess a-globin chains. |
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Definition
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Term
| T or F: 1 unit of blood transfusion is equal to 5 g/dL of Hb raising. |
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Definition
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Term
| In blood transfusions, ________ __________ blood prevents transfusion reactions and CMV/lymphotropic transmission and becoming refractory to platelet transfusions while ________ blood prevents graft v. host disease in immune compromised patients and ______ __________ prevents CMV infections. |
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Definition
| Leukocyte reduced, irradiated, CMV seronegative |
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Term
| T or F: The level of platelets in a thrombocytopenic patient needs to be above 10,000, while a bleeding patient needs 20-50k, and a surgical patient needs 50-100k. |
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Definition
| true, one unit of platelets will raise the patients by 5-10k in a 70kg patient |
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Term
| What is the indication for fresh frozen plasma? |
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Definition
| Correction of clotting factor deficiencies where concentrates not available or when multiple clotting deficiencies are present (e.g. liver disease, Coumadin reversal, DIC) |
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Term
| What are the indications for using cryprecipitate? |
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Definition
| Correct bleeding due to fibrinogen, vWF, FVIII or FXIII deficiency or dysfunction, Bleeding due to uremia, Fibrin “glue” |
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Term
| T or F: recent studies show that pre-transfusion meds are effective at preventing transfusion reactions. |
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Definition
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Term
| List the types of blood that patients can receive RBCs from for type o, a, b, ab patients. |
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Definition
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Term
| List the types of plasma patients can receive for type O, A, B, AB blood. |
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Definition
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Term
| Name this transfusion reaction - red cell incompatibility, occurs within minutes to hours, signs are fever chills hemoglobinuria renal failure hypotension, and is managed using pressors, hemostatic components if needed and maintaining urine output. |
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Definition
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Term
| Name this transfusion reaction - antibody to donor WBC, chills fever headache with possible BP or dyspnea changes, labs rule out hemolysis, treat with antipyretics. |
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Definition
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Term
| Name this transfusion reaction - antibody IgE to donor plasma proteins, signs are urticaria pruritis, treated with antihistamines. |
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Definition
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Term
| Name this transfusion reaction - signs of dyspnea, orthopnea, cough, tachycardia, hypertension, and is treated with diuretics, upright posture, and O2. |
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Definition
| Transfusion associated circulatory overload TACO |
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Term
| Name this transfusion reaction - most common cause of transfusion related death, antibody mediated, causes acute respiratory distress, hypotension, tachycardia, fever, cyanosis, labs are against WBC antibody and treatment is supportive. |
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Definition
| Transfusion reaction associated lung injury - TRALI |
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Term
| Name this transfusion reaction - occurs in patients previously immunized to blood group antigen, hemolysis of RBCs, patients present with fever jaundice and drop in Hct/Hb, similar to hemolytic disease of blue baby syndrome where mothers antibodies destroy fetal cells. |
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Definition
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Term
| True or False: cobalamin and folate deficiency results in megaloblastic anemia. |
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Definition
| True, cobalamin is B12, if serum levels of either are borderline then high homocysteine will confirm the diagnosis |
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Term
| What is the function of hydroxyurea in treating sickle cell patients? |
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Definition
| It induces Hb F production allowing for greater oxygen delivery, used in frequent pain or symptomatic anemia |
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Term
| What are the normal white blood cell values for WBC, PMN, lymphocytes, and monos? |
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Definition
| 5-10k, 50-70%, 20-40%, 1-6%, eosinophils are 1-5%, basophils 0-2% |
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Term
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Definition
| Disease which results from decreased marrow production of red blood cells, usually hypocellular marrow and pancytopenia (WBC, RBC, Thrombo) weakness pallor infections fever bleeding peticheae |
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Term
| T or F: Plasma cell dyscrasias are neoplastic and preneoplastic disorders of plasma cells. |
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Definition
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Term
| In the development of plasma cells at which phase does VDJ rearrangement stop and somatic hypermutation begin - stem cell, pre-b cell, b cell, plasma, memory cell? |
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Definition
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Term
| What is the difference between monoclonal and polyclonal antibodies? |
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Definition
| Mono has only 1 type of Ig light chain and 1 class of heavy chain which can be seen via a spike pattern in electrophoresis, whereas poly has a mixture of both kappa and lambda light chains. |
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Term
| Since most aplastic anemias are idiopathic and can be due to drugs, infections, or immune what is the prognosis of severe aplastic anemia since there is variable severity. |
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Definition
| 90% die within 1 year with transfusions and antibiotic therapies. |
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Term
| Name this leukocyte disorder - clonal proliferation of neoplastic plasma cells, normal marrow suppression, bone lesions, monoclonal protein present in serum and or urine, renal fail due to uric acid and calcium phosphate, calcium increase, anemia, hypogamma, rouleaux formation of RBCs on smear, IgG is common spikes found in spep/iep. |
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Definition
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Term
| What is the difference between plasmacytoma and plasma cell leukemia and lymphoma? |
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Definition
| Cytoma is plasma cell tumor, leukemia is neoplastic plasma cells in the blood, lymphoma is cells in the nodes or other lymphatic tissue |
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Term
| As the size of myeloma cell mass increases from the months of original observation the rate of survival ______________. |
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Definition
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Term
| Name this leukocyte disorder - lymphoid-plasmacytoid cells in marrow or blood, clonal production of IgM, hyperviscosity, splenomegaly, no bone lesions, hyperviscosity symptoms such as fatigue, malaise, dyspnea, neurologic symptoms, headache. |
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Definition
| Waldenstroms macroglobulinemia |
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Term
| In lymphoid disorders you need to remember the ontogeny of b cells when diagnosing, so how are acute leukmias and high grade lymphomas similar versus chronic leukemias and low grade lymphomas. |
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Definition
| First group is from lymphoblasts and immature cells, the second is from intermediate to mature cells |
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Term
| Name this leukocyte disorder - lymphoproliferative disorder with lymphocytosis, lympadenopathy, organomegaly, monoclonal mature B cells, hypogammaglobulinemia, common cytogenic abnormality is deletion of 13q14.3 with good prognosis, frequent infections, co-expression of CD5 with CD19&20. |
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Definition
| chronic lymphocytic leukemia CLL |
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Term
| Name this leukocyte disorder - Lymphoproliferative disorder with abnormal blood and marrow lymphocytes with fine filamentous “hairy” projections, Usually B cells, Stain for tartrate resistant acid phosphatase (“trap”) Monoclonal surface immunoglobulin and Fc receptors; Pancytopenia, splenomegaly, infections, immune abnormalities. |
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Definition
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Term
| What clotting factor activates both the intrinsic and extrinsic activation system? |
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Definition
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Term
| The following two lists are the clinical signs for primary or secondary hemostasis dysfunction , ID them - 1. petechiae, purpura, muco bleeding, bleeding following trauma, 2. delayed bleeding, epistaxis, brusing, menorrhagia, and delayed healing in soft tissue and joints. |
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Definition
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Term
| Name this bleeding disorder - x-linked, low factor VIII, bleeding disorder of joints and soft tissue, treated with factor 8 infusion or FFP or Cryoprecipitate. |
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Definition
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Term
| Name this bleeding disorder - marked reduction in platelets, increased megakaryocytes, subtype that is virally induced with remission in kids not adults, treated with IV Ig, splenectomy, rituximab, or in case of subtype steroids. |
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Definition
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Term
| Risocetin is a coag lab test that induces platelet aggregation, it measures the activity of what protein? |
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Definition
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Term
| Name this bleeding disorder - most common inherited disorder, it inhibits platelet adhesion and carrying of factor 8, gene mutation can either cause quantitative or qualitative defect, most common symptoms are epistaxis, bruising and prolonged bleeding, its own factor levels are modified by stress and estrogen. |
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Definition
| Von Willebrands Disease, treated with desmopressin which releases endothlieum stored VWF and factor 8, or treated with VWF concentrates |
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Term
| _________ ___________ – autosomal recessive trait in the NAT2 gene that could lead to either rapid inactivation or slow inactivation of drugs such as Isoniazid (anti-tuberculosis drug) resulting in higher failure rates of treatment or increased side effects, respectively, in mostly Mediterranean and whites. |
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Definition
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Term
| ______________ is a family of antibiotics, the intake of which may be associated with hearing loss in susceptible individuals due to mitochondrial DNA mutations in gene 1555A>G. |
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Definition
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Term
| __________ ________– X-linked disorder, most common enzyme deficiency, associated with hemolytic anemia due to primaquinone (antimalarial drug), sulfonamides and fava beans; risk factor for neonatal jaundice and kernicterus (brain damage due to increased bilirubin). |
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Definition
| Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency, RBC are unable to prevent oxidative damage via NADPH reducing glutathione. |
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Term
| ________ __________– autosomal dominant disorder that is potentially lethal with symptoms including sudden high body temperature, progressive muscle rigidity, severe rhabdomyolysis (muscle breakdown), and death from cardiac arrest; associated with inhalation anesthetics and muscle relaxants |
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Definition
| Malignant Hyperthermia, is a complex trait causing increased Ca and moded RYR1 and does not cause problems at every exposure. |
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Term
| _________ __________– autosomal recessive disorder that includes apnea (inability to breathe) associated with use of succinylcholine as a muscle relaxant due to decreased breakdown via BCHE. |
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Definition
| Succinylcholine Sensitivity |
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Term
| T or F: The Amplichip tests the CYP enzymes CYP2D6 and CYP2C9, which can help with drugs like Warfarin which can have a mutation in white populations in the CYP2C9 and VKOR gene that can block its effects or cause a deficiency in Vit K clotting factors. |
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Definition
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