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23 General Pathology > 11/2 - Hematopoietic and Lymphoid System > Flashcards

11/2 - Hematopoietic and Lymphoid System Flashcards

1
Q

what is reduction of the total circulating red cell mass below normal limits

A

anemias

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2
Q

what reduce the oxygen carrying capacity of blood, leading to tissue hypoxia

A

anemias

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3
Q

what is anemia diagnosed by

A
  1. hematocrit
  2. hemoglobin concentration of the blood
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4
Q

what is the life span of RBC

A
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5
Q

what is the ratio of packed RBC to total blood volume

A

hematocrit

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6
Q

what are small red cells with decreased mean corpuscular volume (MCV)

A

microcytic

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7
Q

what are large red cells with increased MCV

A

macrocytic

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8
Q

what are pale red cels with decreased mean corpuscular hemoglobin (MCH)

A

hypochromic

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9
Q

what are oddly shaped cells

A

poikilocytosis

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10
Q

what is mixture of red cell sizes

A

anisocytosis

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11
Q

what are bulls-eye red cells

A

target cells

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12
Q

symptoms of anemia

A
  1. Fatigue, lack of exercise tolerance
  2. May have cardiac pain (angina) due to increased heart rate and decreased oxygenation (coronary artery disease)
  3. Pale skin and pale mucous membranes
  4. Atrophic glossitis; usually in severe cases only
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13
Q

classifications of anemia due to underlying mechanism

A

I. Blood loss
2. Increased red blood cell destruction (hemolysis)
3. Decreased red cell production

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14
Q

what are examples of acute and chronic blood loss that causes anemia

A
  1. acute blood loss = trauma
  2. chronic blood = GI tract lesions, gynecological disturbamces
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15
Q

mechanism and examples of increased red cell destruction and specific examples

A
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16
Q

mechanism and examples of decreased red cell production

A
17
Q

hemoglobin abnormalities that cause increased red cell destruction

A
  1. sickle cell anemia
  2. thalassemia
18
Q

what is a group of hereditary disorders characterized by the presence of a structurally abnormal hemoglobin

A

hemolytic anemia of hemoglobin abnormalities

19
Q

what result in decreased red cell survival and often acute hemolysis under certain biochemical and physiologic stress

A

hemolytic anemia of hemoglobin abnormalities

20
Q

what is single amino acid substitution, valine for glutamic acid, in the beta globin chain; worldwide, most common form of familial hemolytic anemia

A

sickle cell anemia

21
Q

8% of who is heterozygous for HbS

A

african americans

22
Q

is sickle cell traitAR or AD

A

AR

23
Q

when does HbS polymerize

A

when deoxygenated and crystallizes producing the characteristic sickle shape

24
Q

what amino acid is replaced in sickle cell anemia

A

glutamic acid becomes VALINE

25
Q

what chain is the problematic chain in sickle cell anemia

A

beta chain

26
Q

is sickle cell trait heterozygous or homozygous

A

heterozygous

27
Q

is sickle cell anemia heterozygous or homozygous

A

homozygous sickle cell disease

28
Q

what this:
One inherited copy of a genetic variant of hemoglobin S (HbS)
41% or less of Hemoglobin S
Usually, no symptoms; immense stress on body precipitates sickling of RBCs

A

sickle cell trait

29
Q

what this:

Two inherited copies of HbS
Most common form of sickle cell disease
Requires ongoing medical treatment; acute pain is so severe that it is called “crisis”

A

sickle cell anemia

30
Q

effects of sickling

A
  1. reducing life space of RBC from 120 to 20
  2. autosplenectomy
  3. may cause widespread mirovascular obstructions
  4. bone infarcts common
  5. impaired immune response
31
Q

what happens when disease (e.g., sickle cell anemia) damages the spleen to such an extent that it becomes shrunken and nonfunctional

A

autosplenectomy

32
Q

oral manifestatoins of sickle cell anemia

A
  1. orofacial and dental pain
  2. pallor of oral mucosa and delayed tooth eruption
  3. radiographic: stepladder trabecular pattern
33
Q

what disease has step ladder pattern in premolar and molar areas as an oral manifestation

A

sickle cell anemia

34
Q

how to treat oral manifestation of sickle cell anemia

A

long term penicillin prophylaxis significantly lowers caries in children with SCA

35
Q

treatment for sickle cell trait

A

no treatment required

36
Q

treatment for sickle cell anemia

A

Pain medications
Oxygen therapy
Hydroxyurea and crizanlizumab (prevents vaso- occlusive crisis)
Bone marrow transplant
Oxbryta/ Voxelotor (prevents Hb from polymerizing)
Gene therapy (LentiGlobin)- clinical trials

37
Q

what gene mutation for sickle cell disease

A

enzyme pyruvate kinase (missing) for gene PLKR

23 General Pathology (24 decks)

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