Chapter 7 & 8 Hem

Front 1

Which inclusion cannot be visualized by the Wright stain peripheral smear

Back 1

Heinz bodies

Front 2

Which function most affect spherocytes as they travel through the circulation

Back 2

They are less deformable and more sensitive to the low glucose in the spleen

Front 3

Many individuals with hereditary spherocytosis are prone to jaundice because of

Back 3

Gallstone disease

Front 4

What are characteristics of hereditary pyropoikilocytosis

Back 4

Elliptocytes, spherocytes, and budding red blood cells

Front 5

What red blood cell morphology is formed as a result of Heinz bodies being pitted from the red blood cell

Back 5

Bite cells

Front 6

What hemolytic disorder has red blood cells that are especially sensitive to lysis by complement

Back 6

Paroxysmal nocturnal hemoglobinuria

Front 7

In the osmotic fragility test, normal red blood cells hemolyze at which level

Back 7

0.45%

Front 8

One of the least severe clinical manifestations of G6PD deficiency is

Back 8

Congenital nonspherocytic hemolytic anemia

Front 9

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

Back 9

Aplastic anemia

Front 10

Individuals with Fanconi's anemia characteristically show

Back 10

Increased Hgb F

Front 11

What is the amino acid substitution in patients with sickle cell anemia

Back 11

Valine for glutamic acid

Front 12

What are the factors contributes to the pathophysology of sickling

Back 12

Dehydration

Front 13

Most clinically significant hemoglobin variants

Back 13

Most are single amino acid substitutions

Front 14

What hemoglobins rank second in variant hemoglobins world wide

Back 14

Hbg E

Front 15

What shows crystals appearing like bars of gold in the peripheral smear

Back 15

Hgb CC

Front 16

What conditions is the leading cause of hospitalization for sickle cell patients

Back 16

acute chest syndrome

Front 17

Which hemoglobin separation methods is used for most newborn hemoglobin screening

Back 17

Isoelectric focusing

Front 18

Autosplenectomy is characteristic of

Back 18

Sickle cell anemia

Front 19

The Benin haplotype of sickle cell disease is prevalent in which country or countries

Back 19

West Africa

Front 20

When the bone marrow temporarily ceases to produce cells in a sickle cell patient an _______ crisis has occurred

Back 20

Aplastic

Front 21

What organ plays a vital role in red blood cell health and longevity

Back 21

Spleen

Front 22

An autosomal dominant disorder of several membrane proteins is

Back 22

HS

Front 23

What is a key protein in HS

Back 23

spectrin

Front 24

Spherocytes are _________ deformable and are _______ osmotically fragile

Back 24

less

more

Front 25

Patients with hereditary spherocytes have

Back 25

spenomegaly

jaundice

increase tendency for gallstones disease

Front 26

The ________ _________ test is a labor intensive by valuable test to assess red blood cell response in different hypotonic salt solutions

Back 26

osmotic fragility

Front 27

HE is a membrane disorder of spectrin showing _________ thermal stability

Back 27

Decreased

Front 28

What are some clinical variants of HE

Back 28

common HE

Southeast Asia ovalocytosis

spherocytic HE

Front 29

A recessive membrane disorder with a vizarre red blood cell morphology that shows hemoglobin budding

Back 29

Hereditary pyropoikilocytosis

Front 30

A membrane disorder in which red blood cells have an intrinsic defect to sodium and potassium permeability

Back 30

Hereditary stomatocytosis

Front 31

______ is an X-linked recessive disorder with more than 400 variants

Back 31

G6PD

Front 32

Drug induced or infection induced hemolysis in G6PD is

Back 32

intravascular

brisk

self limiting

Front 33

Hypoproliferative disorder in which there is cellular depletion and a reduced production of blood cells

Back 33

Aplastic anemia

Front 34

are rear hyproliferative disorders with congenital malformations

Back 34

Fanconi's anemia & Diamond Blackfan anemia

Front 35

a hemolytic anemia that is caused when nine red blood cells become increasingly sensitive to complement lysis

Back 35

PNH

Front 36

What is a disease affecting elderly individuals that is caused by an IgM auto-antibody of wide thermal range

Back 36

CAS

Front 37

is an extremely rare hemolytic disorder caused by anti P of a wide thermal wide

Back 37

Paroxysmal cold hemoglobinuria

Front 38

Most hemoglobinopathies are the result of single

Back 38

single amino acid substitution in the beta chain

Front 39

In sickle cell disorders valine is substituted for ___________ in the ________ position of the beta chain

Back 39

glutamic acid

sixth

Front 40

In Hgb C disorders __________ is substituted for glutamic acid in the ________ position of the beta chain

Back 40

lysine

sixth

Front 41

The presence of _________ affords some protection against malarial infection of red blood cells

Back 41

Hgb S

Front 42

Cells containing _________ as the majority hemoglobin are insoluble in areas of the body with low oxygen tension

Back 42

Hgb S

Front 43

Sickle cells clog small vessels during sickling crisis, causing

Back 43

extensive organ damage and pain

Front 44

_________ ________ of Hgb S produces sickle cell animia

Back 44

Homozygous inheritance Hgb SS

Front 45

_______ ________ produces sickle cell trait

Back 45

Heterozygous inheritance Hgb AS

Front 46

Predispose a patient to sickle cell

Back 46

Hypoxia

acidosis

dehydration

cold

fever

Front 47

The is an ________ % prevalence of the sickle cell gene among Africa Americans

Back 47

8-10

Front 48

______________ is a consequence of repeated infarction to the spleen in young children with sickle cell disease

Back 48

Auto splenectomy

Front 49

__________ & _________ a serious complications in sickle cell patients

Back 49

Stroke

acute chest syndrome

Front 50

During sickle cell episodes patients show

Back 50

nucleated red blood cells

sickle cells

target cells

polychromasia

Front 51

Individuals with sickle cell trait are _________ with rare abnormalities in the peripheral smear

Back 51

asymptomatic

Front 52

_______ _______ is the usual screening procedure to determine if Hgb S is present

Back 52

Dithionite solubility

Front 53

_______ & ________ provide better methods to isolate hemoglobin bands

Back 53

Acid or Alkaline electrophoresis

IEF

Front 54

is the result of inheriting 2 abnormal hemoglobins Hgb S and Hgb C

Back 54

Hgb SC

Front 55

Hgb C disease may produce Hgb crystals on

Back 55

Wright stain

Front 56

may produce abnormal crystals formation resembling the Washington Monument or fingers in a glove

Back 56

Hgb SC

Front 57

may produce condition as severe as sickle cell disease

Back 57

Hgb S beta thalassemia

Front 58

is the second most prevalent hemoglobin variant and is seen with great frequency in southeast Asia

Back 58

Hgb E

Front 59

_______ & ________ migrate with Hgb S on alkaline electrophoresis

Back 59

Hgb D & Hgb G