final heme review, quiz and exam questions

What is the most stable parameter of the cbc? a. MCHC b. RBC c. MCV d. RDW

c. MCV

in adults, which of the following is the most common cause of IDA

chronic blood loss

iron recycled vs ingested in adults and infants

adults dietary- 5% adults recycled - 95 infants dietary - 30% infants recycled - 70

hgb h disease is associated with

alpha thalassemia disease

All the following are risk factors or triggers for Sickle cell crisis except: a Hypoxia b Alkalosis c Acidosis d Fever

b Alkalosis

1. An anemia that manifests as decreased marrow cellularity, pancytopenia, and a reticulocytopenia is appropriately termed: a. Megaloblastic anemia b. Aplastic anemia c. Sideroblastic anemia d. Iron deficiency anemia

b. Aplastic anemia

Match the following protein with their primary function a. Spectrin - allows iron to convert to 2+ b. Cholesterol - stimulates red cell production c. Glycophorin - Promotes attachment of red cell antigens d. Phospholipids - produce ATP

b. Cholesterol - stimulates red cell production

Which of the following statements regarding 2,3 dpg is correct? a. Catalyzes prophyrin synthesis b. Controls hemoglobin affinity for oxygen c. Prevents oxidative denaturation of hemoglobin d. COnverts methemoglobin to oxyhemoglobin

b. Controls hemoglobin affinity for oxygen

1. Individuals with Fanconi's anemia characteristically will have: a. Intravascular hemolysis b. Increased Hgb F c. Ringed sideroblasts d. Thrombocytosis

b. Increased Hgb F

The erythrocyte stage that marks the beginning of hemoglobinization /hgb synthesis is called a. Basophilic normoblast b. Polychromatiphilic normoblast c. Orthrochromic normalblast d. Pronormoblast

b. Polychromatiphilic normoblast [65%]

1. An eccentric "puddling" of a RBC's hemoglobin contents is most closely associated with: a. Stomatocytosis b. Xerocytosis c. G6PD deficiency d. Pyruvate kinase deficiency

b. Xerocytosis

A CBC with a platelet count of 1,000,000 on a first time patient is called a. normal result b. critical result c. leukemic result d. something you can ignore

b. critical result

what is also known as cooley's anemia

beta thalassemia major

what % of hgb is synthesized in the reticulocyte stage a 65 b 95 c 35 d 45

c 35

all but one of the following are characteristics of RBC in stages of development a nuclei are baseball round b immature cells are larger c distinct granulation in cytoplasm d N:C ratio decreases as cell matures

c distinct granulation in cytoplasm [never granules]

what is the major hgb in newborn a hgb F b hgb A1 c hgb gower d hgb A2

c hgb gower

all of the following are symptoms of IDA except a pallor b pica c vertigo d numbness

d numbness

the alpha thalassemia result from which of the following

gene depletion

given lab results: serum iron = 300 ug/dl, transferrin = 312 mg/dl, transferrin saturation - 122%, ferritin = 1,000 ng/dl, with hair loss and cardiac arrythmia, which of the following should be considered

hereditary hemochromatosis

majority of the hgb made in people with thalassemia major is

hgb F

in order for hgb to combine reversibly with O2, the iron must be

in ferrous [fe+2] state

which of the following can cause a decrease in hct a incomplete sealing of capillary tubes b inadequate centrifugation c buffy coat included in hct reading d delay in reading after centrifugation

incomplete sealing of capillary tubes

iron panel results in person with IDA

increased ***TIBC decreased ***serum ferritin ***transferrin ***serum iron ***iron saturation

the 1st stage of IDA is termed

iron depletion

one of the gravest clinical problems for people with thalassemia major is

iron overload

treatment for IDA

iron supplements transfusions

Extra-medullary hematopoiesis may take place in which organs?

liver and spleen

in IDA the retic count will be

low

In hemoglobin C disease, ____ is substituted for glutamic acid.

lysine

what type of inclusion is likely to be seen in a patient with HH

pappenheimer bodies

variation in RBC shape is termed

poikilocytosis

which population is most susceptible to IDA

pregnant women

transferrin receptors for iron uptake are located on the

pronormoblast

error analysis, standard protocols, and turnaround times are all part of the

quality assurance system

which of the following lab tests would be decreased at each stage of iron deficiency

serum ferritin

which of the following lab analyses determined iron stores

serum ferritin level [and hemosiderin]

which of the following electrolytes maintains its highest concentration circulating in the plasma as opposed to within the RBC cytoplasm

sodium [intracellular: extracellular Na = 1: 12, K = 25:1]

at what stage does symptoms of IDA occur

stage III

What are the main functions of the spleen?

storage [holds 1/3 WBC and platelets] immunologic [makes Ab] hematopoiesis [making WBC, RBC, platelets if needed] filtration [pittng out inclusion ]

In hemoglobin C disease, the majority of the red blood cells appear as:

target Cells

a 15-yo- lebanese boy was sent to lab and lab results are as follows: WBC 7.5 RBC 5.9 hgb 11.6 hct 36% what is presumptive condition

thalassemia trait

describe principle of microhematocrit test

to compare RBC content to the volume of whole blood

which of these analytes is an acute phase reactant

transferrin

IDA is a chronic condition that can improve in a matter of weeks

true

in the thalassemias, the deficit has nothing to do with iron

true

patients with moderate anemia [7-10 g/dl] may be asymptomatic

true

explain drawbacks of complete splenectomy

decreased memory B cells [located in marginal zone] pancytopenia

which of the following are iron rich food a tea and coffee b clams, soybeans, and oatmeal c orange juice, pickles, and alcohol d liver and tofu e B and D

e B and D

yolk sac - liver and spleen - BM -

2 weeks- 2 months 2-7 months 7 months - adult

the hgb molecule consists of

4 heme molecules and 4 globin chains

in U.S. about ___ % of anemias seen is due to IDA

50

the embden-meyerhof pathway produces what percentage of cellular ATP, through what process?

90% of cellular ATP through anaerobic metabolism

mature RBC leave the BM and enter circulation via

BM sinuses

The lungs are particularly susceptible in individuals with sickle cell disease and may be susceptible to:

Pulmonary Hypertension

In sickle cell anemia, _____is substituted for glutamine.

Valine

Patient is a 30 year old male. Which hematocrit is abnormal? a. 38 b. 42 c. 49 d. 52

a. 38 [range is 42-52%]

1. One of the least severe clinical manifestations of G6PD is: a. Acute Hemolytic Anemia b. Favism c. Neonatal Jaundice d. Congenital Non-spherocytic Hemolytic Anemia

d. Congenital Non-spherocytic Hemolytic Anemia

where is BM located in adults

iliac crest and sternum

1. Lack of Heinz bodies or bite cells in the peripheral smear is enough to rule out G6PD Deficiency. 2. There is more jaundice than hemolysis in hemolytic disease of the fetus/newborn. 3. Aplastic anemia is caused by an immune mechanism where T cells suppress stem cell proliferation. 4. The peripheral smear for Thalassemias shows a microcytosis, hypochromia, targets, and ↑nRBCs.

1. False 2. False [For Congenital Nonspherocytic Hemolytic Anemia] 3. False 4. True

list 2 sources of error when preparing a smear

1 angle of smear is >45 or <30 causing too thick/thin smear 2 using too big of a drop of blood > thick smear

A blood smear from a patient with sickle cell crisis shows 80 nucleated red blood cells per 100 leukocytes. The total leukocyte count is 18 ×109/L. The corrected white blood cell count is:

10,000 corrected WBC count = WBC x 100 / [nRBC + 100]

Case Study: A 2-year-old African American boy was seen in the sick baby clinic with vomiting, fever, and red-colored urine staining his diapers. His initial laboratory results showed hemoglobin of 5 g/dL and hematocrit of 15%. The most remarkable chemistry value was LDH of 500 IU/L (reference range, 0 to 100 IU/L), which is extremely elevated. His peripheral smear revealed polychromasia and occasional bite cells. When the mother was questioned about whether the boy had ingested anything out of the ordinary, she stated that he had been chewing on mothballs in her closet. The known active ingredient in moth balls is Naphthalene. The child was transported to the intensive care unit. What is happening to this child?

Acute Hemolytic Anemia

When the bone marrow temporarily ceases to produce cells in a sickle cell patient, a(n) ______ crisis has occurred.

Aplastic

An anemia that manifests as decreased marrow cellularity, cytopenias in two cellular elements, and a reticulocytopenia is appropriately termed:

Aplastic Anemia

What are the three abnormal hemoglobins?

Carboxyhemoglobin [hgb bound to CO bc CO has 200x O2 affinity vs normal hgb, if not tx with O2 therapy > CO poisoning] Methemoglobin [cyanosis, hgb M, chocolate blood] Sulfohemoglobin [100x lower O2 affinity vs normal hgb, from exposure to sulfonamides, tx with new methylene blue]

Case Study: A 55-year-old woman complained to her physician that her fingers and toes became blue during cold weather. When she warmed them up, her digits became painful. She also noted that she has been feeling extremely fatigued, with tachycardia and dyspnea. There was no family history of anemia or any other inherited hematologic condition. A CBC and differential were ordered with the following results: WBC, 8.0 × 109/L; RBC, 2.04 × 1012/L; Hgb, 9.0 g/dL; Hct, 24.0%; MCV, 117 fL; MCH, 44.1 pg; and MCHC 37.5%. Which anemic condition can lead to this patient's unusual symptoms?

Cold Agglutinin Syndrome

Which of the following hemolytic disorders has red blood cells that are especially sensitive to lysis by complement?

Paroxysmal Nocturnal Hemoglobinuria

The principle behind hemoglobin electrophoresis is best represented by which of the following statements?

Hemoglobins migrate to different locations depending on time, pH, and media used.

acidic hgb electrophoresis which hgb migrate together

Hgb A Hgb O, Hgb A2, Hgb DPunjab, Hgb GPhila Hgb E

alkaline hgb electrophoresis which hgb migrate together

Hgb C, Hgb E, Hgb OArab, Hgb A2 Hgb S Hgb DPunjab Hgb GPhila Lepore

The hemoglobin that cannot be separated from Hgb S through electrophoresis in buffer at pH 8.6 is:

Hgb D

The hemoglobin variant that is seen frequently in the Southeast Asian population and migrates with Hgb C at pH 8.6 is:

Hgb E

A patient with sickle cell anemia may develop cardiopulmonary disease due to:

High blood viscosity

calculate RBC indices MCV MCHC MCH

MCV - hct / RBC x 10 [fl] MCHC - hgb / hct x 100 [%] MCH - hgb / RBC x 100 [pg]

Autosplenectomy is characteristic of which disorder?

Sickle Cell Anemia

The clinical picture of a patient with Hgb S-beta-thalassemia is similar to that seen in which other hemoglobinopathy?

Sickle Cell Anemia

Which red cell precursor is the final one to be nucleated?

Orthochromic normoblast

A 26-year-old day laborer with a long history of anemia has a several months' history of dyspnea, fever, and pulmonary pain. Additionally, he complains of tightness in the chest and upper abdominal region. His hematology values are as follows: Hgb A =3% Hgb A2 = 2% Hgb S = 85% Hgb F = 10% WBC 15.9 ×109/L RBC 2.9 ×1012/L Hgb 9.5 g/dL Hct 30% MCV 103 fL MCH 31 pg MCHC 32% NRBC 15/100 WBC Platelets 275 × 109/L Reticulocytes 16.8% The patient's most likely diagnosis is:

Sickle Cell Disease

rule of three

RBC x 3 = hgb hgb x 3 = hct +/- 3

Which of the following RBC morphologies are classically associated with hemoglobinopathies and liver disease?

Targets Cells

which of the following is not a cause of iron deficiency anemia a inherited defect of globin chain b nutritional deficiency c GI bleed d excessive menses [bleeding]

a inherited defect of globin chain

Name one condition which may shift the OD curve to the left a. A high affinity hemoglobin b. Metabolic acidosis c. An anemic condition d. Increased hemoglobin concentration

a. A high affinity hemoglobin

1. One of the most severe clinical manifestations of G6PD is: a. Acute Hemolytic Anemia b. Favism c. Neonatal Jaundice d. Congenital Non-spherocytic Hemolytic Anemia

a. Acute Hemolytic Anemia

1. Pyruvate Kinase Deficiency is a defect caused by which metabolic pathway? a. Embden-Meyerhof Pathway b. Pentose Phosphogluconate Pathway c. Methemoglobin Reductase Pathway d. Complement Pathway

a. Embden-Meyerhof Pathway

1. The inability of the red blood cell to generate ATP for membrane function is most closely associated with: a. Pyruvate kinase deficiency b. G6PD deficiency c. Fanconi's anemia d. Congenital hemolytic anemia

a. Pyruvate kinase deficiency

The red cell protein that is responsible for deformability and flexibility of the red cell is a. Spectrin b. Glycophorin c. 2,3 DPG d. EPO

a. Spectrin

1. Bite cells are formed from: a. The spleen "pitting" out Heinz bodies b. Third-degree burns c. Spectrin deficiency d. G6PD deficiency

a. The spleen "pitting" out Heinz bodies

Which of the following physiologic conditions shifts the oxygen dissociation curve to the right? a. Low 2,3 DPG b. Reduced body temp c. Anemia d. Increased pH

c. Anemia

1. The deficit of which of these is most closely associated with Southeast Asian ovalocytosis? a. Spectrin b. Ankyrin c. Molecular band 3 d. G6PD

c. Molecular band 3

Which is the best test to assess the bone marrows response to anemic stress? a. Hemoglobin values b. MCV value c. Retic count d. Hematocrit

c. Retic count

1. An osmotic fragility test is performed on a patient's sample. Initial hemolysis occurs at 0.75% NaCl and complete hemolysis occurs at 0.50% NaCl. These results are indicative of which of the following disorders? a. Sickle cell anemia b. Hgb C disease c. Thalassemia major d. Hereditary spherocytosis

d. Hereditary spherocytosis