RBC disorders Flashcards

1
Q

Anemia due to underproduction is caused by

A

Parvovirus B19

Aplastic anemia

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2
Q

Autosplenectomy leads to increased suseptability to

A

Capsulated organism:

  1. S. pneumoniae
  2. H. influenzae
  3. Salmonella
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3
Q

Cis deletion in two gene α-Thalassemia found in

A

Asians

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4
Q

Clinical features of Iron deficiency

A
  1. Anemia
  2. Koilonychia
  3. Pica
  4. Plummer-Vinson
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5
Q

Clinical findings of Folate deficiency

A

Glossitis

Macrocytic RBC and hypersegmented PMN

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6
Q

Clinical in Hemolytic anemia

A
  1. Anemia with splenomegaly
  2. Jaundice due to unconjungated bilirubin
  3. Marroy hyperplasia with corrected RC
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7
Q

Hb Bart see in

A

Four gene deletion in α-Thalassemia

Usually leads to hydrops fetalis

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8
Q

HbH is seen in

A

Three gene deleted α-Thalassemia

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9
Q

Heinz bodies seen in

A

G6PD deficiency

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10
Q

IgG-mediated IHA chrx

A

Occurs extravascular

Occurs in warm temperatures

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11
Q

IgM-mediated IHA chx

A

Occurs intravascular

Occurs in cold temperatures

Associated with Mycoplasma pneumoniae

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12
Q

Immune hemolytic anemia (IHA) is

A

AB mediated IgG or IgM destruction of RBCs

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13
Q

Irreversible sickling leads to

A
  1. Dactylitis: swollen hands and feet
  2. Autosplenectomy: Howell-jolly bodies on blood smear
  3. Acute chest syndrome
  4. Pain
  5. Renal papillary necrosis
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14
Q

Lab findings in Anemia of Chronic disease

A
  1. Increase ferritin
  2. Decrese TIBC
  3. Decrease serum iron
  4. Decrease % saturation
  5. Increase Free erythrocyte protoprophyrin (FEP)
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15
Q

Lab findings in folate deficiency

A

Increase serum homocysteine

Decrese serum folate

Normal methylmalonic acid

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16
Q

Lab findings in Iron deficiency

A
  1. Microcytic, hypochromic RBC
  2. Decrease ferritin
  3. Increase TIBC
  4. Decrease serum iron
  5. Decrease % saturation
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17
Q

Lab resuts of β-Thalassemia minor

A

Microcytic, hypochromaic RBC with target cells

18
Q

Macrocytic anemia is most commonly due to

A

Folate or Vit B12 deficiency

19
Q

MCV < 80

A

Microcytic anemia

20
Q

Microcytic anemias are due to

A

Decreased produciton of hemoglobin

Extra devision in BM to maintain hemoglobin concentration

21
Q

Microcytic anemias include

A
  1. Iron deficiency anemia
  2. Anemia of chronic disease
  3. Sideroblastic anemia
  4. Thalassemia
22
Q

Paroxysmal nocturnal hemogloninuria (PNH) is due to

A

acquired defect in myeloid stem cells resulting in absent GPI, renders cells susceptible to destruction by complements

23
Q

Parvovirus B19 does what

A

Infects progenitor red cells and temporarily halts erythropoiesis

24
Q

Reticulocytes are

A

young RBCs released from the BM

Identified as larger cells with bluish cytoplasm

25
Q

Serum ferritin reflects

A

Iron stores in macrophages and liver

26
Q

Sickle cell is due to

A

replacement of normal glutamic acid with valine

27
Q

Sideroblastic anemia is due to

A

Defective protoporphyrin synthesis

28
Q

Sideroblastic anemia lab findings

A
  1. Increase ferritin
  2. Decrease TIBC
  3. Increase serum iron
  4. Increase % saturation
29
Q

Thalassemia protective against

A

Plasmodium flaciparum

30
Q

Total iron-binding capacity (TIBC) measures

A

Transferring molecules in the blood

31
Q

Trans deletion in two gene α-Thalassemia found in

A

Africans

32
Q

Vit. B12 clinical

A

Glossitis

Subacute combind degeneration of SC due to increased methylmalonic acid (peripheral neuropathy)

33
Q

What causes IgG-mediated IHA

A
  1. SLE
  2. Drugs:
    1. Penicillin
    2. Cephalosporin
    3. Methyldopa
34
Q

What complications arise from PNH

A
  1. Iron deficiency anemia
  2. AML
35
Q

What increases risk of sickling in HbS

A
  1. Hypoxemia
  2. Dehydration
  3. Acidosis
36
Q

Where are β genes pressent in β-Thalassemia

A

chromosome 11

37
Q

α-Thalassemia is due to

A

Gene deletion of alpha gene on chromosome 16

38
Q

β-Thalassemia major clinical

A

Massive erythroid hyperplasia causing:

  1. Expansion of hematopoiesis into skull (crewcut appearance)
  2. Expansion of hematopoiesis into facial bone (chipmunk face)
  3. Extramedullary hematopoiesis with hepatosplenomegaly
  4. Risk of aplastic crisis with B19 infection
39
Q

β-Thalassemia major (β00) is

A
  1. Most severe β-Thalassemia that presents with severe anemia a few months after brith
  2. α tetramers aggregate and damage RBC resulting in ineffective erythropoiesis and extravascular hemolysis
40
Q

β-Thalassemia major smear

A

Microcytic, hypochrmic RBC with target cells and nucleated RBC

41
Q

β-Thalassemia minor (β/β+) is

A

Mildest form of β-Thalassemia usually asymptomatic with an increased RBC count

42
Q

β-Thalassemia usually due to

A

gene mutation