Anemias Part 1

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Exam Review
Josefina Gonzalez
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Josefina Gonzalez
Creado por Josefina Gonzalez hace alrededor de 9 años
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where does iron from making new RBCs come from? hemoglobin breakdown
what causes iron deficiency in males? chronic blood loss
what is the result In acute blood loss, where there is a sudden loss of 25-30% of the total blood volume most healthy patients show signs and symptoms of lightheaded-ness, hypotension and tachycardia
what is the result of a loss of 30-40% of the total blood volume? leads to shortness of breath, diaphoresis (sweating), loss of consciousness, hypotension, a rapid and weak pulse, and a decrease in total urine output
With the sudden loss of 40-50% of the total blood volume, the result is? the patient goes into severe shock, and death may ensue.
ferritin is? stored iron
define asynchronism? a red cell with a fairly mature cytoplasm and an immature nucleus.
what is intrinsic factor and what is its function? glycoprotein secreted by the parietal cells of the stomach that is necessary for binding and absorption of dietary vitamin B12.
where is B12, Folate, and iron stored? Liver and sometimes kidneys
what does peripheral blood look like in aplastic anemia? Increased nucleated red blood cells (NRBCs); Pancytopenia; some aniso
what does peripheral blood look like in renal disease? NC/NC with some burr cells and Schistocytes; Basophilic stippling
what are the signs of lead poisoning? “lead line” on the gums
who is more likely to get lead poisoning? people that work with certain chemicals or with batteries; those who live in older homes with lead based paints
what is the etiology (cause) of iron deficiency anemia? Circumstances that cause a reduction in the iron available for heme synthesis or failure to incorporate iron into heme
what is the pathophysiology (sign/symptom) of iron deficiency anemia? Fatigue, breathlessness, and dizziness; Pica; Angular stomatitis; Glossitis (inflamed tongue); spooned nails; Splenomegaly (rarely)
what does the peripheral blood look like in iron deficiency anemia? Micro/Hypo; increased platelet count
what does the bone marrow look like in iron deficiency anemia? Iron stores are severely decreased; hyperplasia
what does the iron stores look like in iron deficiency anemia? Ferritin, transferrin and iron are decreased; TIBC is increased and erythropoietin (EPO) is also increased
CBC results in iron deficiency anemia? MCV and MCHC values are low; RDW increased
what does peripheral blood look like in megaloblastic anemia Dacryocytes; neutrophils hypersegmented; ovalocytes; Up to 20% NRBCs
what does the bone marrow look like in megaloblastic anemia? Asynchronous; cellular; Most of the RBCs in the promegaloblast stage; Increased iron;
what is the etiology of megaloblastic anemia? impairment of DNA synthesis
what is the pathophysiology of megaloblastic anemia? Neurological; Fatigue; Light headedness; Shortness of breath; Pronounced pallor; Slight jaundice
CBC results of megaloblastic anemia look like? increased RDW, MCV and MCHC; deceased WBC, Hct and platelet count
What are cabot bodies? (very rare): part of the nuclear membrane; stain reddish violet with Wrights stain; seen with a patient that had a splenectomy
what are heinz bodies? broken down parts of hemoglobin and are purple with supra-vital stains, seen with hemolytic anemias, patients on antimalarial meds.
what are pappenheimer bodies? trapped excess iron seen best with prussian blue but can be seen with Wrights Stain
what are basophilic stippling? composed of precipitated ribonucleoprotein and mitochondrial remnant. Observed on Romanowsky stained blood smears as diffuse or punctate bluish black granules in toxic states such as drug (lead) exposure. It may also occur as an artifact.
what are Howell-Jolly bodies? Erythrocyte inclusion composed of nuclear remnants (DNA); Romanowsky stain; Commonly associated with megoblastic anemia and splenectomy.
what is Hemoglobin C disease? abnormal & causes hemoglobin c disease; limits carrying oxygen; genetic disorder caused by a single amino acid on the beta chain; seen with a lot of target cells
What is Hemoglobin SC disease? Both beta chains are abnormal. Hemoglobin A is absent. both sickling and crystal formation are evident. Also target cells
what is congenital dyserythropoietic anemia? (rare) inherited refractory anemia with Ineffective erythropoiesis; Hyperbilirubinemia; Many pyknotic cells present; peripheral blood: pancytopenic;
classify iron deficiency anemia? micro/hypo
classify sideroblastic anemia? micro/hypo
classify anemia of chronic disease? micro/hypo
classify lead toxicity anemia? micro/hypo
classify B12 deficiency anemia? macro/megalo
classify folate deficiency anemia? macro/megalo
classify liver disease? macro/non-megalo
classify hypthyroidism (myxedema)? macro/non-megalo
classify aplastic anemia? NC/NC or macro/non-megalo
classify dysplastic anemia? macro/non-megalo
classify refractory anemia? macro/non-megalo
classify myelophthistic anemia? macro/non-megalo
classify cytotoxic drug anemia? macro/non-megalo
classify post hemorrhagic anemia? NC/NC
classify chronic blood loss anemia? NC/NC
classify congenital dyserythropoietic anemia? NC/NC
classify renal disease anemia? NC/NC
classify endocrine disease anemia? NC/NC
what are stromatocytes? cup shaped cells; Associated with liver disease, electrolyte imbalances, hereditary stomatocytosis
what is Hyperchromasia? (use sparingly); Spherocytes are the only erythrocytes that are this abnormality. MCHC is < 36 g/DL.
what is Anisocytosis? considerable variation in erythrocyte size. Seen with sickle cell and certain leukemias
what are spherocytes? bright red color and microcytic. Removed in the spleen. High MCHC!! Seen in hemolytic anemias
when would you see microcytosis? seen with iron deficiency, severe hypochromasia
when would you see macrocytosis? with pernicious anemia
What are codocytes (target cells)? Thin, flat, bell shaped, seen in hemoglobin C disease, sickle cell anemia, hemolytic anemias, liver disease, thalassemia
what are Drepanocytes (sickle cells)? contain polymerized HbS; Fragile and have trouble carrying oxygen well. Associated with sickle cell anemia
what are Crenated cells? Mostly artifact; don’t indicate any disease
what are echinocytes (blur cells)? spiculated red cells that are smaller than RBCs with spine-like projections; can revert back to an erythrocyte. Seen in uremia (renal failure), cancer of the stomach, acute bleeding ulcers
what are acanthrocytes (spur cells)? Small spherical cells with irregular thorn like projections and contain spicules; seen in liver disease; liver disease and metabolic diseases
what are schistocytes? Fragmented cells; usually microcytic; found with intravascular fibrin formation; seen in hemolytic anemias, severe burns, DIC
what are ovalocytes/elliptocytes? Oval shaped; ovalocytosis; not specific to any certain disease/ Egg shaped; elliptocytosis: not specific to any certain disease
what are dacryocytes (tear drop)? RBCs are tear shaped. Seen in b12 and folic acid deficiency, hemolytic anemias, thalassemia, pernicious anemia
what is the etiology of inherited sideroblastic anemia? Defect in enzymes needed for heme synthesis
what is the pathophysiology of inherited sideroblastic anemia? Decreased activity within the delta-aminolevulenic acid (d-ALA) synthesis pathway
CBC results in inherited sideroblastic anemia? normal
what does the peripheral blood look like in inherited sideroblastic anemia? dimorphic; Aniso, poik, target cells & basophilic stippling; Pappenheimer bodies
what does the bone marrow look like in inherited sideroblastic anemia? hyperplasia; excess iron stores in macrophages; ringed sideroblasts; Abnormal iron “rings” in the prorubricytes, rubricytes and metarubricytes
what do the iron studies look like for sideroblastic anemia? increased: Ferritin, transferrin, and iron Normal: TIBC
CBC results in Idiopathic sideroblastic anemia (AKA refractory anemia) look like? Decreased: Hct & Hgb
what does the bone marrow look like in Idiopathic sideroblastic anemia (AKA refractory anemia)? hyperplasia; excess iron stores in macrophages; ringed sideroblasts
what do the iron studies in Idiopathic sideroblastic anemia (AKA refractory anemia) show? Increased: Ferritin, transferrin, and iron free erythrocyte protoporphyrin (FEP) levels
what is the etiology in secondary siderblastic anemia? Anemia follows exposure to certain drugs and toxins
CBC results in secondary sideroblastic anemia show? Decreased: Hgb Normal to decreased: MCV/MCHC
peripheral blood in all three sideroblastic anemias show? dimorphic; Aniso, poik, Schistocytes, target cells & basophilic stippling
bone marrow in secondary sideroblastic anemia shows? hyperplasia; excess iron stores in macrophages; ringed sideroblasts
iron studies in secondary sideroblastic anemia show? Increased: Ferritin, transferrin, and iron free erythrocyte protoporphyrin (FEP) levels
what do the iron studies in anemia of chronic disease show? Decreased: iron, TIBC, % saturated transferrin Increased: Ferritin Normal: TIR
CBC results in anemia of chronic disease show? Decreased: Hgb, Hct slightly and All indices; Retic count is normal to slightly increased
pathophysiology of anemia of chronic disease? Decreased iron
etiology of anemia of chronic disease? Block in heme synthesis due to cytokines; inhibition of EPO production and impaired erythropoiesis; block in iron release from macrophages; shortened erythrocyte survival
peripheral blood in anemia of chronic disease shows? Aniso is moderate but poik is minimal
bone marrow in anemia of chronic disease shows? Abundant iron
which erythrocyte inclusions are composed of aggregated denatured hemoglobin and stain with supravital stains but not with Romanowsky stains? heinz bodies
a patient with anemia has an RPI of 2.3 with an MCV of 103 fl. how would you classify this anemia? macrocytic
a blood smear reveals uneven distribution of blood cells. the cells appear to be stacked together like a stack of coins. how would you describe this distribution? rouleaux
which of the following tests will give information about rate of erythrocyte destruction? serum bilirubin
a patient has the following results: RBC count 2.5; Hgb 5.3; Hct 0.17; Retic count 1%. what are the absolute retic count and RPI? absolute count = 250; RPI = 0.15
what is the iron transport protein? transferrin
the term sideroblastic is most closely associated with which anemia? iron deficiency
the basic defect in sideroblastic anemia is? faulty enzymes regulating heme synthesis
describe hemosiderosis? increase in total body iron
what anemia would be expected from: MCV normal, serum iron decreased, serum ferritin normal, TIBC and % saturation decreased? anemia of chronic disease
the most common cause of macrocytosis is? alcoholism
in the majority of cases, b12 deficiency is due to a deficiency of? IF
if you saw hypersegmented neutrophils, what anemia would you suspect? megaloblastic anemia
increases in urinary excretion of formiminoglutamic acid would most likely indicate what condition? folic acid deficiency
the liver stores enough folate to meet daily requirement needs for how long? 3-6 months
what affect does pregnancy have on someones vitamin b12 daily requirements? increases requirements
a deficiency of vitamin b12 leads to impaired? DNA synthesis
which laboratory test is used to diagnose pernicious anemia? schilling test
alcoholics commonly develop a macrocytic anemia due to? folate deficiency
anemia due to liver disease is often associated with which RBC morphological form? spur cells
a lack of IF may be due to? gastrectomy
what is more typical of non-megaloblastic than megaloblastic anemia? round macrocytes
the bone marrow in aplastic anemia is typically? hypocellular
what are considered a cause of hypoproliferation in aplastic anemia? damage, depletion or inhibition of stem cells
diagnostic criteria for aplastic anemia includes a granulocyte count of? less than 0.5
what is DIC? formation of clots - it dissolves itself, releases; give them heparin to disrupt the process.
what is hemosiderin? found mainly in urine; major long term storage form of iron; slow release
what is TIBC? total iron binding capacity
serum ferritin expected result is? (M) 15-400 ng/mL; (F) 10-106 ng/mL
serum iron expected result is? 50-160 µg/dL
TIBC expected result is? 250-400 µg/dL
% ferritin saturation expected result is? 20-55%
what does the peripheral blood show in pure red cell aplasia anemia? decreased RBC count, Hct, Hgb, retic count; Increased or normal: WBC count, platelet count; bilirubin normal or low;
what physical finding is associated with iron deficiency anemia? koilonychias
what are koilonychias? spooned nails
what does yellowness of the eyes suggest? high bilirubin concentration
if a patient has a test that reveals antibodies and complement on his RBCs, what does this support? an immune hemolytic anemia
what does the presence of spherocytes indicate? a sign that the spleen has removed antibody/antigen complexes from the cell membrane
what is a typical sign of spherocytes? high MCHC
a high retic count and presence of polychromatophilic RBCs indicate what? the bone marrow is responding appropriately by increasing output of erythrocytes.
Case Study The patient is a 62 year old Mexican-American male who was seen by his physician for an annual checkup. He had a CBC and comprehensive metabolic panel, lipid panel, ferritin, TIBC, serum iron and % saturation. Results were: WBC 5.9 x 109/L Hemoglobin 16.8 g/dL Hematocrit 49.1% RBC 5.08 x 1012/L RDW 12.9 Differential: 47% neutrophils; 39% lymphocytes; 11% monocytes; 2% eosinophils; 1% basophils Platelet count 176 x 109/L Comprehensive metabolic panel: within reference range Cholesterol 202 mg/dL Serum ferritin: 390 ng/mL (12-282) TIBC: 292 mg/dL (250-450) Serum iron 166 mg/dL (35-158) Answer #1-#6
#1. Calculate the patient’s % iron saturation. Reference range at this facility is 20-50%. Saturation % = serum iron/TIBC The patient’s % saturation is 57%
#2. Interpret the patient’s iron status. The serum ferritin, serum iron and % satuation indicate an increase in total body iron levels.
#3. What condition is suggested with these results? The condition suggested by these results is hemochromatosis.
#4. The patient was sent to a hematologist who ordered genetic testing. Results showed a single heterozygous mutation at C282Y. There was no evidence of mutation of the H63D or S65C mutation. What do these genetic results mean? These results show that the man is a carrier of hereditary hemochromatosis (HH). The C282Y mutation is the most common mutation in HH. H63D and 565C mutations are less common.
#5. The patient donated a pint of blood every other week for 2 months. The physician ordered a repeat serum ferritin. The result was 218 (23-233). What was the rationale for the periodic phlebotomy? Why did the serum ferritin decrease? Phlebotomy is the usual treatment for HH. Each donation of blood removes about 250 mg of iron from the body. The serum ferritin decreases as the amount of total body iron decreases.
#6. What is the risk of iron overload in the body? When iron accumulates in the cells, the capacity of the cells to store iron by complexing with apoferitin is exceeded. The free intracellular iron accumulates and facilitates the buildup of reactive oxygen species. This causes cell injury and leads to organ failure.
MCV calculation? Hct/RBC * 10 = MCV
MCH calculation? Hbg/RBC * 10 = MCH
MCHC calculation? Hgb/Hct * 100 = MCHC
Absolute Retic Calculation? Hct/Normal * raw retic = Absolute retic
RPI calculation? absolute retic/maturation time = RPI
which maturation time correlates with a Hct of 40-45% 1.0
which maturation time correlates with a Hct of 30-39% 1.5
which maturation time correlates with a Hct of 20-29% 2.0
which maturation time correlates with a Hct of less than 20? 2.5
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