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Lecom pathology year 2 > RBC Disorders- chapter 14 > Flashcards

RBC Disorders- chapter 14 Flashcards

1
Q

what does G6PD deficiency have protective role against

A

falciparum malaria

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2
Q

what measurements are used as surrogates for RBC mass

A

Hb, Hct, RBC count

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3
Q

what is the screening test for PNH

A

sucrose test

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4
Q

what is beta thalasemmia due to

A

gene mutations

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5
Q

what is the disorder described as inherited defect of RBC cytoskeleton- membrane tethering proteins

A

hereditary sclerocytosis

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6
Q

what is the definition of anemia

A

decrease in circulating RBC mass

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7
Q

describe the blood smear with normocytic anemia

A

larger cells with blush cytoplasm (due to residual RNA in cell)

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8
Q

What antibodies are involved in cold hemolysis type of immunohemolytic anemia

A

IgG

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9
Q

what is the characteristic of the RBCs in hemoglobin C

A

HbC crystals

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10
Q

what is acute chest syndrome a complication of

A

sickle cell anemia

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11
Q

what causes sickle cell anemia

A

AR mutation in beta chain of hemoglobin

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12
Q

what does G6PD deficiency render cells susceptible to

A

oxidative stress

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13
Q

who is trans 2 gene deletion alpha thalasemmia most common in

A

africa

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14
Q

What type of anemia is a primary marrow disorder in which only erythroid progenitors are suppressed

A

Pure red cell aplasia

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15
Q

what is the most common cause of death in adults with sickle cell anemia

A

acute chest syndrome

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16
Q

what is seen on electrophoresis with 4 gene deletion alpha thalasemmia

A

Hb barts (gamma chains formed into tetramers)

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17
Q

what are the lab findings with folate deficiency

A

macrocytic RBCs and hyperhsegmented neutrophils; glossitis; decreased serum folate and increased homocysteine

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18
Q

What is defined as ratio of packed RBC to total blood volume

A

Hematocrit

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19
Q

What antibodies are involved in cold agglutinin type of immunohemolytic anemia

A

IgM

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20
Q

What usually triggers the aplastic crises sometimes seen with hereditary spherocytosis

A

Acute parvovirus infection

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21
Q

when does the intravascular hemolysis with PNH mostly occur

A

often at night

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22
Q

where is folate absorbed

A

jejunum

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23
Q

describe the blood smear with beta thalasemmia major

A

microcytic, hypo chromic target cells and nucleated RBCs

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24
Q

what is the most common cause of vitamin B 12 deficiency

A

pernicious anemia

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25
Q

what is seen on electrophoresis in 3 deletion alpha thalasemmia

A

HbH

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26
Q

describe the levels of homocysteine and methylmalonic acid in vitamin B12 deficiency

A

increased

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27
Q

Describe the PTT and PT of hemophilia A

A

Prolonged PTT normal PT

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28
Q

what test is used for diagnosis of hereditary sclerocytosis

A

osmotic fragility test (increased fragility in hypotonic solution)

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29
Q

what does parvovirus B19 infection

A

progenitor red cells

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30
Q

describe the level of methylmalonic acid in folate deficiency

A

normal

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31
Q

Who is hemoglobin H disease more common in

A

Asian populations

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32
Q

What mutation is responsible for paroxsymal nocturnal hemoglobinuria

A

PIGA

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33
Q

what is 4 gene deletion called in utero

A

hydrops fetalis

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34
Q

What disorder is associated pencil cells (elongated Red cells

A

Iron deficiency

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35
Q

what is macrocytic anemia most commonly due to

A

folate or vitamin B12 deficiency

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36
Q

what screen is used to test for any HbS cells

A

metabisulfite screen

37
Q

what occurs with 3 gene deletion in alpha thalasemmia

A

severe anemia beta chains form tetramers that damage RBCs

38
Q

what does indirect coombs test confirm presence of

A

Abs in its serum

39
Q

what is the change made in hemoglobin C

A

normal glutamic acid is replaced by lysine

40
Q

what does G6PD present as

A

hemoglobinuria and back pain

41
Q

What typically causes HUS

A

E. coli O157:H7

42
Q

what appears on the blood smear with beta thalasemmia minor

A

target cells and microcytic hypo chromic RBCs

43
Q

Describe the marrow aspirated of aplastic anemias

A

Often yield little material; dry tap

44
Q

what is absent with PNH

A

GPI

45
Q

What disorder has heinz bodies and bite cells

A

G6PD deficiency

46
Q

When are the signs of fetal distress with hydrophobic details evident

A

3rd trimester

47
Q

Where is transferrin synthesized

A

Liver

48
Q

what type of hemolysis goes with sickle cell anemia

A

both intra and extra

49
Q

what test is used to confirm immune hemolytic anemia

A

direct coombs test

50
Q

what chromosomes are the 2 beta genes located on

A

11

51
Q

What disorder is defined as inherited deficiency of platelet membrane glycoprotein complex (receptor for vWF)

A

Bernard-Soulier syndrome

52
Q

Describe the change in MCHC for hereditary spherocytosis

A

Increased MCHC due to dehydration because of loss of K+ and H2O

53
Q

What is the most common complication of transfusions

A

Febrile nonhemolytic reaction

54
Q

what emerge on blood smear of hereditary sclerocytosis

A

howell-jolly bodies

55
Q

What GPI-linked protein issues are associated with paroxysmal nocturnal hemoglobinuria

A

CD55, CD59, C8 binding protein

56
Q

describe the MCV in microcytic, normocytic and microcytic anemia

A

micro= less than 80; normo= 80-100; macro= greater than 100

57
Q

what is the disorder classified as decreased synthesis of global chains of hemoglobin

A

thalassemia

58
Q

what is normocytic anemia due to

A

increased peripheral destruction or underproduction

59
Q

What type of anemia is a rare autosomal recessive disorder caused by defects in multiprotein complex that is required for DNA replace that leads to aplastic anemia

A

Franconi anemia

60
Q

what is alpha thalasemmia usually due to

A

gene deletion

61
Q

what does direct coombs test confirm presence of

A

presence of Ab-coated RBCs

62
Q

What are the clinical presentations of sequestration crises of sickle cell disease

A

Rapid splenic enlargement, hypovolemia, sometimes shock

63
Q

what disorder should you think of with crew cut appearance on x-ray and chipmunk-like face of skull

A

massive erythroid hyperplasia in beta thalasemmia major

64
Q

how do you treat hereditary sclerocytosis

A

splenectomy

65
Q

What enzyme is associated with TTP

A

ADAMTS13 deficiency plasma enzyme

66
Q

What chromosome are the alpha-globin genes on

A

16

67
Q

what is damaged with aplastic anemia

A

HSC

68
Q

What antibodies are involved in warm antibody type of immunohemolytic anemia

A

IgG

69
Q

Describe the blood smear of beta thalassemia major

A

Anisocytosis, poikilocytosis, microcytosis, hypochromia

70
Q

what are carriers of thalassemia often protected against

A

plasmodium falciparum malaria

71
Q

what can confirm presence and amount of HbS

A

Hb electrophoresis

72
Q

Where is hepcidin synthesized

A

Liver

73
Q

what is the chain with sickle cell anemia

A

normal glutamic acid (hydrophilic) replaced by valine (hydrophobic)

74
Q

How is paroxsymal nocturnal hemoglobinuria diagnosed

A

Flow cytometry

75
Q

What is the Pentad associated with TTP

A

Fever, thrombocytopenia, microangiopathic hemolytic anemia, transient neurologic deficits, renal failure

76
Q

what appears on the blood smear for sickle cell anemia

A

howell-jolly bodies

77
Q

what is the hallmark of microangiopathic hemolytic anemia

A

schistocytes (look like helmet)

78
Q

What is the most specific morphological finding with hereditary spherocytosis

A

Spherocytosis (smears that have small, hyperchromic red cells lacking the central zone of parlow)

79
Q

Who does sequestration crises occur in

A

Children with sickle cell disease that have intact spleens

80
Q

what is the main cause of death in PNH

A

thrombosis

81
Q

what are the clinical lab findings with extravascular hemolysis (in general)

A

hemoglo binemia, hemoglobinuria, hemosiderinuria, decreased serum haptoglobin

82
Q

what is the presentation when 2 genes are deleted in alpha thalasemmia

A

mild anemia with slightly increased RBC count

83
Q

What is the most common nutritional disorder in the world

A

Iron deficiency anemia

84
Q

What is another name for alpha thalassemia with deletion of 3 genes

A

Hemoglobin H disease

85
Q

What are the clinical presentations of von willebrand disease

A

Epistaxis, excessive bleeding from wounds, menorrhagia

86
Q

what is shown in the biopsy of aplastic anemia

A

empty, fatty marrow

87
Q

Describe the level of activity of hepcidin with hemochromatosis

A

Inappropriately low

88
Q

What do the RBCs lack in beta thalassemia major

A

HbA

89
Q

what does the oxidative stress with G6PD precipitate Hb as

A

heinz bodies

Lecom pathology year 2 (18 decks)

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