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PCM Exam 3 > Red Cell Disorders 3 > Flashcards

Red Cell Disorders 3 Flashcards

1
Q

what is the normal function of the PIGA gene?

A

synthesizes GPI anchor which is important for the anchoring of cell surface proteins on hematpoeitic cells

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2
Q

what are the CNS symptoms seen with B12 deficiency?

A
  • paraparesis, sensory ataxia and lower limb paraesthesia

- degeneration of the dorsal and lateral tracts of the CNS

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3
Q

_____ is the most common cause of B12 deficiency

A

pernicious anemia

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4
Q

_______ hemolysis is seen with warm antibody immunohemolytic anemia

A

extravascular; antibody coated RBC’s are removed from circulation by phagocytes in the spleen

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5
Q

removal of ______ (part of the GIT) will make them at risk for B12 deficiency

A

terminal ileum

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6
Q

↑ or ↓ reticulocyte count seen in megaloblastic anemia

A

↓

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7
Q

describe the steps of B12 absorption from the diet

A
  1. haptocorrin binds to B12 and creates a complex
  2. pacreatic proteases in the duodenum releases B12 from the complex
  3. free B12 binds go intrinsic factor released by gastric parietal cells
  4. B12 is transported in the blood with transcobalamin
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8
Q

↑ serum methymalonic acid is seen in ______ deficiency

A

B12 (not seen in folate deficiency)

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9
Q

cold hemolysin involves ____ autoantibodies

A

IgG

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10
Q

most antibodies in warm antibody immunohemolytic anemia are _____ class

A

IgG

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11
Q

________ are at risk for B12 deficiency

A

vegans

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12
Q

______ tetramers are seen in fetus’s with hydrops fetalis

A

gamma

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13
Q

B12 is ultimately absorbed in the _______

A

distal ileum

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14
Q

_______ is a clinical presentation of someone with cold agglutinin type of antibody immunohemoltic anemia

A

Raynaud’s phenomenon: blue fingers

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15
Q

in the cold agglutinin type of cold antibody immunohemolytic anemia , there are ______ antibodies

A

IgM antibodies that react strongly at lower temperatures and not at normal body temperatures

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16
Q

what are the types of α thalassemia seen based on the number of genes deleted

A
  • silent carrier : 1 α gene is only deleted
  • minor: 2 α genes deleted
  • HbH disease: 3 α genes deleted
  • Hb Barts/ hydrops fetalis: all 4 α genes deleted
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17
Q

what are the three most important GPI linked proteins which are affected in someone with PNH

A
  • decay accelerating factor (CD55)
  • membrane inhibitor of reactive lysis (CD59)
  • C8 binding protein

protect the cell from being destroyed by complement (intravascular hemolysis)

18
Q

the more dangerous form of α thalassemia minor is seen in _____ populations

A

Asian: α / α, -/-

19
Q

describe the pathogenesis of extravascular hemolysis seen in cold agglutinin type

A
  • as the blood recirculates and warms IgM is released before the complement mediated hemolysis occurs but the IgM deposits C3b (an opsonin) leading to extravascular hemolysis
20
Q

B cell lymphoma is associated with ____ antibody immunohemolytic anemia

A

warm and cold

21
Q

PNH is due to mutations in _____ gene

A 
PIGA gene (phosphatidylinositol glycan class A) which synthesizes GPI anchor which links many cell surface proteins to plasma membrane on hematopoietic cells 
X linked
22
Q

nuclear/cytoplasmic asynchrony is see in _______ anemia

A

megaloblastic anemia because DNA synthesis is ↓ but normal RNA and protein synthesis

23
Q

What are some general cause of impaired RBC production

A
  • failure of proliferation and/maturation of committed RBC precursors such as DNA synthesis, heme synthesis, globin synthesis
  • failure of stem cells: aplastic anemia
  • unknown/multiple mechanism: anemia of chronic disease
24
Q

why is there ↑ hemolysis during the night for PNH ?

A
  • during the night, the ↓ of blood pH will ↑ the activity of complement leading to more hemolysis
25
Q

what are some non immune hemolytic causes of anemia

A
  • mechanical RBC damage: DIC, malignant HTN, TTP/HUS, SLE and disseminated dance r
  • infections: malaria
26
Q

the most common cause of death in patients with PNH is ______

A

thrombosis

27
Q

what is the difference between the direct and indirect Coombs test?

A
  • direct: tests for the antibodies or complements on the surface of the RBC
  • indirect: detection of antibodies in the patient’s serum
28
Q

cold agglutinins are produced in response to:

A

infections: mycoplasma, EBV, HIV, CMV, influenza

neoplastic growth: B cell lymphomas

29
Q

what drugs will increase the chance of developing folate deficiency?

A

methotrexate and trimethoprim

30
Q

cold agglutinin/hemolysin type is associated with intravascular hemolysis

A
  • hemolysis (involves IgG)

agglutinin type involves IgM and will see extravascular hemolysis

31
Q

__________ form of autoimmune hemolytic anemia is usually seen following viral infections in children

A

cold hemolysin type

32
Q

absence of ____ and _____ would be seen on flow cytometry in someone with PNH

A

CD55 and CD59

33
Q

what is the composition of hemoglobin H?

A

tetramer of β globin chains (due to excess β globin chains)

34
Q

what are some associated conditions with PNH?

A
  • thrombosis
  • aplastic anemia
  • iron deficiency
  • AML and MDS (myeloid dysplastic syndrome)
35
Q

folate is absorbed in the ______

A

proximal jejunum

36
Q

______ is the only hemolytic anemia caused by an acquired genetic defect

A

paroxysmal nocturnal hemoglobinuria

37
Q

What is the best method to diagnose PNH and what would you look for?

A
  • flow cytometry in which you will see an absence of CD55 and CD59 on blood cells
38
Q

beefy tongue is associated with _____

A

B12 deficiency due to megaloblastic changes in the oropharyngeal epithelium

39
Q

pernicious anemia is an autoimmune disease where there is destruction of _____-

A

gastric parietal cells

40
Q

hyperhsegmented neutrophils are seen in _____ anemia

A

megaloblastic anemia

PCM Exam 3 (32 decks)

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