Term
| What stage of erythropoesis is a large nucleated cell that does not contain Hb? |
|
Definition
|
|
Term
| What stage of erythropoesis is characterized by increased Hb synthesis and in which organelles are lost and the nucleus is ejected? |
|
Definition
|
|
Term
| What is the cell called in erythropoesis once the nucleus has been ejected? |
|
Definition
|
|
Term
| Name the 4 erythroid stages of erythropoesis. |
|
Definition
| 1) Proerythroblast 2) Erythroblast 3) Reticulocyte 4) Mature erythrocyte |
|
|
Term
| Name 3 types of erythroblasts. |
|
Definition
| 1) Basophilic 2) Polychromatic 3) Acidophilic erythroblast |
|
|
Term
| What does too few RBC's lead to? |
|
Definition
|
|
Term
| What does too many RBC's cause? |
|
Definition
| undesirable blood viscosity |
|
|
Term
| What type of control is erythropoesis under? |
|
Definition
|
|
Term
| What organ monitors oxygen levels and produces erytropoeitin in response to hypoxia? |
|
Definition
| Kidney - erythropeoetin produced by renal interstitial cells if renal tissue is hypoxia |
|
|
Term
| What nutrients are required for erythropoesis? |
|
Definition
| Iron, vitamins B12, C, and B6, folic acid, and AA |
|
|
Term
| Name 3 conditions which trigger erythropoesis. |
|
Definition
| 1) Hypoxia d/t low RBC 2) Decreased O2 availability 3) Increased tissue demand for O2 |
|
|
Term
| Name 2 things caused by enhanced erythropoesis. |
|
Definition
| 1) Inc. RBC in blood 2) Inc. O2 carrying ability of the blood |
|
|
Term
| What organ to a lesser extent, also produces erythropoetin? |
|
Definition
|
|
Term
| Name 4 places the body stores iron. |
|
Definition
| 1) Hb (65%) 2) Liver 3) Spleen 4) Bone marrow |
|
|
Term
| Intracellular iron is stored in protein-iron complexes such as ______. |
|
Definition
|
|
Term
| Circulating iron is loosely bound to the transport protein _________. |
|
Definition
|
|
Term
| What is the life span of an erythrocyte? |
|
Definition
|
|
Term
| What engulfs dying erythrocytes? |
|
Definition
|
|
Term
| What is heme degraded into? |
|
Definition
| a yellow pigment called bilirubin |
|
|
Term
| The liver secretes bilirubin into the ________ as _______. |
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Definition
|
|
Term
| What do the intestines metabolize bile into to be excreted in feces? |
|
Definition
|
|
Term
| Globin is metabolized into _____ and is released into the ___________. |
|
Definition
|
|
Term
|
Definition
| Redution in # of circ. RBC's in the peripheral blood w/ abnormally low O2- carrying capacity |
|
|
Term
| Name 4 manifestations of anemia. |
|
Definition
| 1) BM d/o 2) Abn. of RBC's 3) Nutritional def. 4) Sys. dz. that result in anemia |
|
|
Term
| Is anemia considered a sx or a dz? |
|
Definition
|
|
Term
| Differentitate b/t absolute and relative anemia. |
|
Definition
| 1) Relative - normal RBC mass/excess plasma volume (pregnancy) 2) Absolute - actual dec. in RBC # |
|
|
Term
| Name 3 types of anemia caused by impaired production of RBCs by the BM. |
|
Definition
| 1) Normocytic 2) Macrocytic 3) Microcytic |
|
|
Term
| What is the anemia d/t erythropoetin def. or def. of hematopoetic cells? |
|
Definition
|
|
Term
| What is the anemia d/t impaired erythroid precursor cell division/DNA synthesis? |
|
Definition
|
|
Term
| What is the anemia d/t impaired heme or globin synthesis? |
|
Definition
|
|
Term
| What 3 things does anemia caused by accelerated destruction or loss of circulating erythrocytes? |
|
Definition
| 1) Increased EPO prod. 2) Increased # of erythroid precursors in the BM 3) Release of inc. newly formed reticulocytes in the blood |
|
|
Term
| Name 4 things that result in hemolytic anemia. |
|
Definition
| 1) intrinsic RBC membrane defects 2) intrinsic RNBC enzymopathies 3) Hgb variants w/ red. solubility or protein instability 4) Abn. extrinsic to RBC |
|
|
Term
| Describe the clinical presentation of anemia w/ rapid development/loss of blood? |
|
Definition
|
|
Term
| What is the clinical presentation of anemia that is acute/chronic resulting in tissue/organ hypoxia? |
|
Definition
| Hypotension and tachycardia |
|
|
Term
| What is the clinical presentation of hemolytic anemia? |
|
Definition
| toxicity of products of lysed RBCs |
|
|
Term
| What sx are reported in mild-to-moderate anemia? |
|
Definition
| fatigue, gen. weakness, and loss of stamina followed by tachycardia and exertional dyspnea |
|
|
Term
| When do young, healthy individuals typ. begin to experience sx of anemia? |
|
Definition
|
|
Term
| What s/sx occur in an anemic pt w/ h/o MI? |
|
Definition
| pronounced edema, dyspnea, orthopnea, fatigue, and loss of stamina |
|
|
Term
| What occurs in anemic pts w/ CAD? |
|
Definition
| onset or exac. of angina or may precipitate an MI |
|
|
Term
| What sx occur in an anemic pt w/ PAD? |
|
Definition
|
|
Term
| What occurs in anemic pts w/ h/o CVA? |
|
Definition
| frequent or severe TIAs or strokes |
|
|
Term
| What personal and family hx may be a clue to a pt having anemia? |
|
Definition
| anemia, splenectomy, cholecystectomy, gallstones, jaundice |
|
|
Term
| What physical signs may be present in a pt w/ anemia? |
|
Definition
| pallor in skin, palmar creases, or mucus membranes (conjunctiva, and pitting of fingernails |
|
|
Term
| What cardiac signd may be present in a pt w/ anemia? |
|
Definition
| orthostatic hypotension, tachycardia, systolic ejection murmur, bounding pulses |
|
|
Term
| What is the primary lab test to check for the presence of anemia? |
|
Definition
|
|
Term
| Name 6 other tests useful to dx type of anemia. |
|
Definition
| 1) Reticulocyte count 2) Peripheral blood smear 3) Iron panel 4) Combs test 5) Bilirubun 6) Hgb electrophoresis |
|
|
Term
| What is the most common nutritional def. and the most common cause of anemia? |
|
Definition
|
|
Term
| Name 5 conditions that cause iron def. anemia? |
|
Definition
| 1) Low intake 2) Dim. absorption 3) Inc. in req. 4) Excessive iron loss 5) CRF |
|
|
Term
| What behavior can alert a provider to the presence of iron def. anemia? |
|
Definition
|
|
Term
| Name 4 sx of severe iron def. anemia? |
|
Definition
| 1) Glossitis 2) Dysphagia 3) Erosions at corners of mouth 4) Gastritis |
|
|
Term
| What is koilonychia? What type of anemia is it most common in? |
|
Definition
| Spoon-shaped nails, iron-def. |
|
|
Term
| What lab tests should be done to check for iron-def. anemia? |
|
Definition
| CBC (H&H, RBC indices, RDW) and iron panel (serum ferritin, TIBC) |
|
|
Term
| What is the tx for iron-def. anemia? |
|
Definition
| Oral ferrous sulfate w/ Vit. C, tx underlying cause |
|
|
Term
| Improvement of Hb w/ iron supplementation in iron-def. anemia is not seen until 6-8 wks, what test could be performed to monitor efficacy of tx /p only 2 wks? |
|
Definition
|
|
Term
| What is the name of the classic B12 def.? |
|
Definition
|
|
Term
| what physiologic deficiency results in B12 def.? |
|
Definition
|
|
Term
| What social hx results in B12 def. ? |
|
Definition
|
|
Term
| what is the name given to the effect of B12 def.? Name the sx. |
|
Definition
| "megaloblastic anemia" - paranoid ideation, dementia, cognitive dysfxn, delusions, hallucinations, prorioception disturbances, parasthesia of hands and feet |
|
|
Term
| Name 4 social hxs that may result in folate def.? |
|
Definition
| 1) Poor or fad diet 2) Malabsorption 3) Etohism 4) Pregnancy |
|
|
Term
|
Definition
| 1) Depression 2) Sleep dep. 3) Irritability 4) Memory loss 5) Personality changes |
|
|
Term
| Name 8 signs of Vit B12 and folate def.? |
|
Definition
| 1) Pedal edema 2) Nocturia 3) Tachypnea 4) Dyspnea 5) Tachycardia 6) Glossitis 7) Wt loss 8) Steatorrhea |
|
|
Term
| Name 4 MS sx of B12 and folate def. |
|
Definition
| 1) Arthralgia 2) Arthritis 3) Nocturnal pain 4) Cramping in UE & LE |
|
|
Term
| What are the dermatologic signs of B12 and folate def? |
|
Definition
| blotchy brown skin pigmentation of nail beds and skin creases |
|
|
Term
| What labs tests should be performed to check for B12 def? |
|
Definition
| CBC (H&H, RBC indices, RDW) and peripheral blood smear |
|
|
Term
| Is it rec. to perform a Schilling's test to check for B12 def.? |
|
Definition
|
|
Term
| What is the tx for B12/folate def. in critically ill pts? |
|
Definition
| IM B12 (1mg/d) and PO folate (1-5mg/d) |
|
|
Term
| What other nutrient should be monitored in pts on B12 tx? |
|
Definition
| Potassium, manage w/ K supplementation |
|
|
Term
| Can supplementation reverse s/sx in pts w/ neurologic damage d/t Bqw/folate def.? |
|
Definition
| slow reversal, up to 6 mths. <3mths sx may have complete reversal. |
|
|
Term
| What does anemia of chronic disease occur from? |
|
Definition
| failure of renal/endocrine fxn (impaired erythropoetin prod. and BM compensation) or of renal excretory fxn (leads to hemolysis, BM cell depression and blood loss) |
|
|
Term
| Other than gen. affects of anemia, what are other clinical manifestations of anemia of chronic dz/renal failure? |
|
Definition
| Pericardial effusion, dec. Hct in proportion to renal insuff., uremia (BUN in urine) when GFR <40 |
|
|
Term
| what might you see in a peripheral blood smear of a pt w/ anemia of chronic dz? |
|
Definition
|
|
Term
| What values in a CBC are normal in a pt w/ anemia of chronic dz? |
|
Definition
| WBC, differential cell count, platelets, RBC indices |
|
|
Term
| What is the tx for anemia of chronic dz? what is the goal for tx? |
|
Definition
| erythropeotin - target Hct 33-36% and Hb 11-12 |
|
|
Term
| what other supplements are given to pts w/ anemia of chronic dz? |
|
Definition
|
|
Term
| If pts do not repond to tx for anemia of chronic dz, what would you want to check for? |
|
Definition
| adequate iron supply, infection, or excessive splenic hemolysis |
|
|
Term
| What type of anemia is caused by dec. RBC survival d/t the presence of mutant genes that suppress the syn. of globin chains? |
|
Definition
|
|
Term
| Why does thalassemia cause such a severe anemia? |
|
Definition
|
|
Term
| Differentiate b/t thalassemia major and minor. |
|
Definition
| Major - severe form, homozygous, minor - heterozygous carrier |
|
|
Term
| What ethnic group is alpha-thalassemia most common? |
|
Definition
| Primarily Asian, some Mediterranean/African descent |
|
|
Term
| What is alpha-thalassemia in which 3 genes are deficient called? what signs are present? |
|
Definition
| Hemoglobin H dz - typical facies and hepato-splenomegaly |
|
|
Term
| What occurs in alpha-thalassemia in which 4 genes are affected? |
|
Definition
| Barts Hb - Hydrops fetalis (pale, edematous, hepato-splenomegaly, ascites) |
|
|
Term
| What ethnic group is beta-thalassemia most common? |
|
Definition
| Mediterranean, some Middle East, part of India, SE Asia |
|
|
Term
| What occurs in major beta-thalassemia? (6) |
|
Definition
| skull deformities, mongoloid faces, bowing of long bones, icterus, heptosplenomegaly, endocrinopathies (DM) |
|
|
Term
| What is seen on CBC in major thalassemia? |
|
Definition
| Hb <7, nucleated RBCs, inc. WBC, normal platelets |
|
|
Term
| What is seen in the bone marrow of a pt w/ thalassemia? |
|
Definition
| hypercellular w/ profound erythroblastic hyperplasia |
|
|
Term
| What tx is given to childern w/ thalassemia to ensure normal growth and development? |
|
Definition
| Blood tranfusion to maintain Hb 9.5 to 11.5 |
|
|
Term
| Should pts w/ thalassemia be given iron supplements? |
|
Definition
|
|
Term
| What can be done if ferritin level is >1000mcg/dl? |
|
Definition
| Chelation therapy w/ deferoxamine |
|
|
Term
| Why is Vitamin C given to pts w/ thalasemia? |
|
Definition
| to increase iron excretion |
|
|
Term
| When might a BMT be performed for thalassemia? |
|
Definition
| for severe beta-thalassemia |
|
|
Term
| What is the prognosis of infants w/ Bart's Hb hydrops fetalis? Hb H dz? |
|
Definition
| Hydrops - stillborn or death shortly after birth, Hb H - live a full life |
|
|
Term
| What is the prognosis of pts w/ beta-thalassemia intermedia? |
|
Definition
| Live through middle-age w/ iron overload and crippling bone dz in 3rd or 4th decades |
|
|
Term
| Defferentiate b/t thalassemia and sickle-cell anemia. |
|
Definition
| Thalassemia - too few globins syn., Sickle - incorrectly fxn globin |
|
|
Term
| In Hemoglobin S, _____ is substituted for __________ in the ______ position of the beta-chain. |
|
Definition
| valine, glutamic acid, 6th |
|
|
Term
| What type of Hb is present in homozygous sickle-cell pts? heterozygous w/ sickle-cell trait? |
|
Definition
| Homo - only Hb S, Trait - both Hb A and Hb S |
|
|
Term
| What ethnic group is sickle-cell anemia and trait almost entirely found in? |
|
Definition
|
|
Term
| What occurs during a sickle-cell crisis? |
|
Definition
| vasooclussion from sickle-shaped RBCS resulting in capillary stasis, venous thrombosis, and arteriol emboli (occlusion occurs in sm. capillaries and venules) |
|
|
Term
| what are clinical manifestations of sickle-cell in children? |
|
Definition
| Short stature, delayed puberty, necrosis of metacarpals and metatarsals |
|
|
Term
| What conditions of organs occur in sickle cell anemia? |
|
Definition
| hepatomegaly, jaundice, gallstones |
|
|
Term
| What cardiac sx occur in sickle cell anemia? |
|
Definition
| tachycardia, acute chest syndrome (fever, CP, inc. WBC, pulmonary infiltrates) |
|
|
Term
| Name 4 other clinical manifestations of sickle-cell anemia. |
|
Definition
| 1) Priapism w/ impotence 2) Retinal vessel obs. 3) CVA 4) Infections |
|
|
Term
| Name 5 clinical manifestations of sickle-cell anemia. |
|
Definition
| 1) Pyelonephritis 2) Pna 3) Antepartum hemorrhage 4) Premature fetal delivery 5) Fetal death |
|
|
Term
| What is seen in labs in a hemolytic crisis? |
|
Definition
| leukocytosis, normoblastosis, urinalysis (diffuse intravascular coagulation) |
|
|
Term
| What is a curative tx of sickle call anemia? |
|
Definition
| stem cell transplantation |
|
|
Term
| what prophylactic measures should be done in pts w/ sickle cell anemia? |
|
Definition
| 1) Pneumococcal pna vaccine (b/f 2yo and booster 3-5yrs later) 2) Hep B vaccination 3) H influ. vaccination 4) Penicillin prophylaxis for strep pneumo |
|
|
Term
| What is sequestration syndrome? |
|
Definition
| Rapid accumulation of RBCs in the spleen - should do splenectomy |
|
|
Term
| Name 4 common causes of death from complications of sickle cell anemia. |
|
Definition
| 1) HF 2) Bone marrow and fat emboli 3) Organ failure 4) Shock |
|
|
Term
| What is the most common metabolic disease of the RBC. |
|
Definition
|
|
Term
| What type of genetic inheritance is seen in G6PD deficiency? What ethnic group is it seen in? |
|
Definition
| X-linked recessive, Blacks and Sephardic Jews |
|
|
Term
| Do pts w/ G6PD deficiency have hemolytic anemia? |
|
Definition
| not unless challenged by drugs, infxn, DKA, or fava beans |
|
|
Term
| What drugs should be avoided in pts w/ G6PD deficiency? (7) |
|
Definition
| Asprin, Vit C and K, anti-malaria, Chlorophenicol, Procainamide, Quinidine, Sulfa-drugs |
|
|
Term
| What foods should be avoided in pts w/ G6PD deficiency? |
|
Definition
| Fava beans, red wine, legumes, blueberries, soy foods |
|
|
Term
| What does G6PD def. require tx? What is the tx? |
|
Definition
| Presence of life-threatening hemolysis. Blood transfusion |
|
|
Term
| What are the sx of G6PD def. if hemolytic anemia is not present? |
|
Definition
|
|
Term
| What is the most common hereditary hemolytic anemia? Who is it most common in? |
|
Definition
| Hereditary spherocytosis. Northern European background |
|
|
Term
| Describe the genetic transmission of hereditary spherocytosis. |
|
Definition
|
|
Term
| Name 5 clinical signs of hereditary spherocytosis. |
|
Definition
| 1) Anemia 2) Jaundice 3) Splenomegaly 4) Gallstones 5) Chronic leg ulcers |
|
|
Term
| What are sx of aplastic crisis precipitated by infxn in pts w/ hereditary spherocytosis? (6) |
|
Definition
| 1) Fever 2) Weakness 3) Pallor 4) Tachycardia 5) Hypotension 6) Shock |
|
|
Term
| What is seen on peripheral blood smear in hereditary spherocytes? |
|
Definition
|
|
Term
| What is the curative tx of hereditary spherocytosis? what preventative measures should be taken? |
|
Definition
| Splenectomy. Influenza and pna vaccine d/t risk for aquiring serious infxn. |
|
|
Term
| What supplentation should be given to pts w/ hereditary spherocytosis? Tx for aplastic crisis? |
|
Definition
|
|
Term
| What is the principle cellular defect in hereditary spherocytosis? |
|
Definition
| loss of membrane surface area of RBC d/t defects of sev. membrane proteins. |
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