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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
what is seen on electrophoresis in 3 deletion alpha thalasemmia
HbH
26
describe the levels of homocysteine and methylmalonic acid in vitamin B12 deficiency
increased
27
Describe the PTT and PT of hemophilia A
Prolonged PTT normal PT
28
what test is used for diagnosis of hereditary sclerocytosis
osmotic fragility test (increased fragility in hypotonic solution)
29
what does parvovirus B19 infection
progenitor red cells
30
describe the level of methylmalonic acid in folate deficiency
normal
31
Who is hemoglobin H disease more common in
Asian populations
32
What mutation is responsible for paroxsymal nocturnal hemoglobinuria
PIGA
33
what is 4 gene deletion called in utero
hydrops fetalis
34
What disorder is associated pencil cells (elongated Red cells
Iron deficiency
35
what is macrocytic anemia most commonly due to
folate or vitamin B12 deficiency
36
what screen is used to test for any HbS cells
metabisulfite screen
37
what occurs with 3 gene deletion in alpha thalasemmia
severe anemia beta chains form tetramers that damage RBCs
38
what does indirect coombs test confirm presence of
Abs in its serum
39
what is the change made in hemoglobin C
normal glutamic acid is replaced by lysine
40
what does G6PD present as
hemoglobinuria and back pain
41
What typically causes HUS
E. coli O157:H7
42
what appears on the blood smear with beta thalasemmia minor
target cells and microcytic hypo chromic RBCs
43
Describe the marrow aspirated of aplastic anemias
Often yield little material; dry tap
44
what is absent with PNH
GPI
45
What disorder has heinz bodies and bite cells
G6PD deficiency
46
When are the signs of fetal distress with hydrophobic details evident
3rd trimester
47
Where is transferrin synthesized
Liver
48
what type of hemolysis goes with sickle cell anemia
both intra and extra
49
what test is used to confirm immune hemolytic anemia
direct coombs test
50
what chromosomes are the 2 beta genes located on
11
51
What disorder is defined as inherited deficiency of platelet membrane glycoprotein complex (receptor for vWF)
Bernard-Soulier syndrome
52
Describe the change in MCHC for hereditary spherocytosis
Increased MCHC due to dehydration because of loss of K+ and H2O
53
What is the most common complication of transfusions
Febrile nonhemolytic reaction
54
what emerge on blood smear of hereditary sclerocytosis
howell-jolly bodies
55
What GPI-linked protein issues are associated with paroxysmal nocturnal hemoglobinuria
CD55, CD59, C8 binding protein
56
describe the MCV in microcytic, normocytic and microcytic anemia
micro= less than 80; normo= 80-100; macro= greater than 100
57
what is the disorder classified as decreased synthesis of global chains of hemoglobin
thalassemia
58
what is normocytic anemia due to
increased peripheral destruction or underproduction
59
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
Franconi anemia
60
what is alpha thalasemmia usually due to
gene deletion
61
what does direct coombs test confirm presence of
presence of Ab-coated RBCs
62
What are the clinical presentations of sequestration crises of sickle cell disease
Rapid splenic enlargement, hypovolemia, sometimes shock
63
what disorder should you think of with crew cut appearance on x-ray and chipmunk-like face of skull
massive erythroid hyperplasia in beta thalasemmia major
64
how do you treat hereditary sclerocytosis
splenectomy
65
What enzyme is associated with TTP
ADAMTS13 deficiency plasma enzyme
66
What chromosome are the alpha-globin genes on
16
67
what is damaged with aplastic anemia
HSC
68
What antibodies are involved in warm antibody type of immunohemolytic anemia
IgG
69
Describe the blood smear of beta thalassemia major
Anisocytosis, poikilocytosis, microcytosis, hypochromia
70
what are carriers of thalassemia often protected against
plasmodium falciparum malaria
71
what can confirm presence and amount of HbS
Hb electrophoresis
72
Where is hepcidin synthesized
Liver
73
what is the chain with sickle cell anemia
normal glutamic acid (hydrophilic) replaced by valine (hydrophobic)
74
How is paroxsymal nocturnal hemoglobinuria diagnosed
Flow cytometry
75
What is the Pentad associated with TTP
Fever, thrombocytopenia, microangiopathic hemolytic anemia, transient neurologic deficits, renal failure
76
what appears on the blood smear for sickle cell anemia
howell-jolly bodies
77
what is the hallmark of microangiopathic hemolytic anemia
schistocytes (look like helmet)
78
What is the most specific morphological finding with hereditary spherocytosis
Spherocytosis (smears that have small, hyperchromic red cells lacking the central zone of parlow)
79
Who does sequestration crises occur in
Children with sickle cell disease that have intact spleens
80
what is the main cause of death in PNH
thrombosis
81
what are the clinical lab findings with extravascular hemolysis (in general)
hemoglo binemia, hemoglobinuria, hemosiderinuria, decreased serum haptoglobin
82
what is the presentation when 2 genes are deleted in alpha thalasemmia
mild anemia with slightly increased RBC count
83
What is the most common nutritional disorder in the world
Iron deficiency anemia
84
What is another name for alpha thalassemia with deletion of 3 genes
Hemoglobin H disease
85
What are the clinical presentations of von willebrand disease
Epistaxis, excessive bleeding from wounds, menorrhagia
86
what is shown in the biopsy of aplastic anemia
empty, fatty marrow
87
Describe the level of activity of hepcidin with hemochromatosis
Inappropriately low
88
What do the RBCs lack in beta thalassemia major
HbA
89
what does the oxidative stress with G6PD precipitate Hb as
heinz bodies

Lecom pathology year 2 (18 decks)

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