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D2 Fall > Gen Path Exam 3 - Anemia and RBC Disorders > Flashcards

Gen Path Exam 3 - Anemia and RBC Disorders Flashcards

1
Q

Decrease in the number of erythrocytes (RBCs) or hemoglobin (Hgb) content per unit volume of peripheral blood

A

Anemia

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2
Q

Anemia hemoglobin level in men vs women

A

Men: < 13 g/dL
Women: < 12 g/dL

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3
Q

Increase in number of RBCs per unit volume of peripheral blood

A

Polycythemia

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4
Q

Myeloproliferative neoplasm of the bone marrow characterized by abnormal hematopoietic stem/progenitor cells and overproduction of RBCs

A

Primary polycythemia

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5
Q

Another name for primary polycythemia

A

Polycythemia vera

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6
Q

Occurs from increased erythropoietin (EPO) levels, chronic lung disease (e.g., emphysema), high-altitude living, obstructive sleep apnea, and cyanotic heart disease

A

Secondary polycythemia

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7
Q

Anemia results from one or more combinations of what 3 basic mechanisms?

A

Reduced/ineffective erythropoiesis
Increased RBC loss/reduced RBC life span
Dilutional anemia

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8
Q

Which basic mechanism?

Decreased marrow erythropoiesis

A

Reduced/ineffective erythropoiesis

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9
Q

Which basic mechanism?

Inadequately increased total erythropoiesis

A

Reduced/ineffective erythropoiesis

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10
Q

Which basic mechanism?

Increased ineffective erythropoiesis

A

Reduced/ineffective erythropoiesis

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11
Q

Which basic mechanism?

Acute or chronic blood loss

A

Increased RBC loss/reduced RBC life span

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12
Q

Which basic mechanism?

Increased RBC destruction

A

Increased RBC loss/reduced RBC life span

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13
Q

Which basic mechanism?

Splenic pooling and sequestration

A

Increased RBC loss/reduced RBC life span

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14
Q

Which basic mechanism?

Plasma volume expansion

A

Dilutional anemia

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15
Q

How long does each RBC live?

A

120 days

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16
Q

To maintain steady populations of RBCs, how many RBCs must be renewed each day?

A

1/120

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17
Q

What does the clinical expression of anemia result from?

A

Tissue hypoxia

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18
Q

The specific signs/symptoms of anemia represent __________________ compensatory responses to the severity/duration of tissue hypoxia

A

cardiovascular-pulonary

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19
Q

Anemia is asymptomatic when hematocrit is….

A

> 30%

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20
Q

Moderate anemia

A

Hematocrit < 30%

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21
Q

Severe anemia

A

Hematocrit < 20%

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22
Q

What type of anemia is associated with:

Weakness
Vertigo
Headache
Tinnitus
Spots before eyes
Fatigue easily
Drowsy
Irritable
Bizzare behavior

A

Moderate to severe anemia

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23
Q

What type of anemia is associated with:

Splenomegaly
Amenorrhea
Loss of libido
GI problems
Jaundice
Heart failure
Shock

A

Moderate to severe anemia

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24
Q

A convenient classification for most anemias that result from RBC production defects is based on what?

A

Changes in RBC size and MCHC

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25
Q

What type of RBCs in this anemia?

Production defect results from alterations in heme or globin synthesis (e.g., iron deficiency or iron malabsorption, thalassemia and related hemoglobin synthesis defects)

A

Microcytic-hypochromic RBCs

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26
Q

What type of RBCs in this anemia?

Hypoproliferative or hypoplastic mechanism (e.g., leukemia, metastatic cancer, cytoreductive drugs)

A

Normochromic-normocytic RBCs w/ defective production

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27
Q

What type of RBCs in this anemia?

Defect in DNA synthesis; usually due to defective vitamin B12, folate, or folic acid metabolism or dietary intake, or to an interference with DNA synthesis by cytoreductive drugs

A

Macrocytic RBCs

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28
Q

Adequate marrow response to anemia is evidenced by _______________ or _______________________

A

reticulocytosis; polychromatophilia

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29
Q

table 1 (stuff under, I don’t think we have to know the #s)

A
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30
Q

Name the 4 anemias due to reduced/ineffective erythropoiesis

A

Iron deficiency anemia
Anemia of chronic disease/inflammation
Vitamin B12 deficiency/pernicious anemia
Folate deficiency anemia

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31
Q

What type of anemia?

Due to decreased iron stores

A

Iron deficiency anemia

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32
Q

What type of anemia?

The MOST COMMON cause of anemia in the US

A

Iron deficiency anemia

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33
Q

What type of anemia?

Affects toddlers 1-2 yrs old
Affects females 12-49 yrs old

A

Iron deficiency anemia

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34
Q

Why are toddlers affected by iron deficiency anemia?

A

Inadequate intake of iron

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35
Q

Why are females affected by iron deficiency anemia?

A

Menstrual loss

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36
Q

What are the 4 causes of iron deficiency anemia?

A

Chronic blood loss
Poor iron intake
Poor iron absorption
Increased demand for iron

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37
Q

What are the reasons for chronic blood loss, which causes iron deficiency anemia?

A

GI bleeding
Menstrual bleeding
Cancers

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38
Q

What 3 things can cause GI bleeding and chronic blood loss, leading to iron deficiency anemia?

A

Ulcers
Aspirin
NSAIDs

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39
Q

What 3 types of cancers cause chronic blood loss, leading to iron deficiency anemia?

A

Esophagus
Stomach
Colon

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40
Q

What do the following cause? What does this lead to?

Gastrectomy
Gastric bypass
Resection of proximal intestine
IBD
Sprue

A

Poor iron absorption
Leads to iron deficiency anemia

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41
Q

What are 3 reasons for increased demand for iron, leading to iron deficiency anemia?

A

Infancy
Adolescence
Pregnancy

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42
Q

What type of anemia?

Onset may be acute with rapid blood loss, or chronic with poor diet or slow (chronic) blood loss

A

Iron deficiency anemia

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43
Q

T/F: Patients with mild iron deficiency anemia (HCT > ~30%) are usually asymptomatic

A

True

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44
Q

What type of anemia?

Signs/symptoms include:
Skin, mucosal, conjunctival pallor
Fatigue
Irritable
Decreased concentration
Weakness
SOB
Brittle, fragile nails and koilonychia
Headache
Decreased appetite
Pica
Dysphagia
Tachycardia
Wide pulse pressure

A

Severe iron deficiency anemia

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45
Q

What does koilonychia mean?

A

Spooning of nails

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46
Q

Clinical manifestation unique to iron deficiency

A

Pica

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47
Q

Unusual craving for certain non-nutritional substances like ice, clay, or starch

A

Pica

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48
Q

What does pagophagia mean?

A

Ice craving (pica)

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49
Q

What does geophagia mean?

A

Clay craving (pica)

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50
Q

What does amylophagia mean?

A

Starch craving (pica)

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51
Q

May rarely occur in patients due to the development of upper esophageal webs (membrane-like growths); occurs most frequently in elderly women with iron deficiency

A

Dysphagia

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52
Q

The triad of iron deficiency anemia, dysphagia, and upper esophageal webs is called what?

A

Plummer-Vinson syndrome

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53
Q

What is Plummer-Vinson syndrome a triad of?

A

Iron deficiency anemia
Dysphagia
Upper esophageal webs

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54
Q

What is associated with an increased risk for the later development of esophageal carcinoma?

A

Plummer-Vinson syndrome

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55
Q

What is Plummer-Vinson syndrome associated with an increased of developing?

A

Esophageal carcinoma

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56
Q

What are the 3 oral presentations of iron deficiency anemia?

A

Angular cheilitis
Atrophic glossitis
Generalized mucosal atrophy

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57
Q

What type of anemia has the following oral manifestations?

Angular cheilitis
Atrophic glossitis
Generalized mucosal atrophy

A

Iron deficiency anemia

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58
Q

What type of anemia?

Decreased serum ferritin

A

Iron deficiency anemia

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59
Q

Why is there decreased serum ferritin in iron deficiency anemia?

A

Iron stored in bone marrow is depleted

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60
Q

What type of anemia?

Decreased serum iron
Increased TIBC + serum transferrin

A

Iron deficiency anemia

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61
Q

What type of anemia?

Decreased ability to make Hgb
Leads to microcytic hypochromic anemia

A

Iron deficiency anemia

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62
Q

What type of anemia?

Microcytic hypochromic anemia w/ wide area of central pallor, anisocytosis, and poikilocytosis

A

Iron deficiency anemia

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63
Q

What type of anemia?

Low RBC, Hgb, HCT, MCV, MCH, MCHC

A

Iron deficiency anemia

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64
Q

What type of anemia?

Increased RDW + platelet count

A

Iron deficiency anemia

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65
Q

Why is the platelet count elevated in iron deficiency anemia?

A

Reactive thrombocytosis

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66
Q

What type of anemia?

Low serum ferritin, serum iron, MCV, MCHC
High TIBC + RDW

A

Iron deficiency anemia

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67
Q

What specific lab findings help differentiate iron deficiency anemia from other anemias?

A

Low ferritin, iron, MCV, MCHC
High TIBC + RDW

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68
Q

How is iron deficiency anemia treated?

A

Iron supplements (ferrous sulfate)

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69
Q

What type of anemia?

2nd most common anemia in the US

A

Anemia of chronic disease/inflammation

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70
Q

What type of anemia?

The MOST COMMON type of anemia in hospitalized patients

A

Anemia of chronic disease/inflammation

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71
Q

What type of anemia?

Associated with many underlying diseases w/ sustained inflammation

A

Anemia of chronic disease/inflammation

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72
Q

What are 3 categories of diseases w/ sustained inflammation that are associated with anemia of chronic disease/inflammation?

A

Neoplasms
Microbial infections
Autoimmune diseases

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73
Q

Name 2 neoplasms associated with anemia of chronic disease/inflammation

A

Hodgkin lymphoma
Carcinoma (lung/breast)

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74
Q

Name 3 microbial infections associated with anemia of chronic disease/inflammation

A

Osteomyelitis
Bacterial endocarditis
Lung abscess

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75
Q

Name 4 autoimmune diseases associated with anemia of chronic disease/inflammation

A

RA
Systemic lupus
Sarcoidosis
IBD

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76
Q

Inflammatory _____________ are associated with neoplasms/microbial infections/autoimmune diseases that stimulate the ________ synthesis and release of __________.

This sequesters iron in ____________ and suppresses the production of _____________.

What type of anemia is involved in this process?

A

cytokines; hepatic; hepcidin

macrophages; erythropoietin

Anemia of chronic disease/inflammation

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77
Q

What type of anemia?

The low availability of serum iron causes iron deficiency in the bone marrow compartment, resulting in decreased RBC production

A

Anemia of chronic disease/inflammation

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78
Q

Circulating protein that blocks ferroportin

A

Hepcidin

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79
Q

An iron channel responsible for the exit of iron from enterocytes (and GI absorption) and macrophages (which accumulate iron from engulfed senescent blood cells)

A

Ferroportin

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80
Q

T/F: The clinical presentations of anemia of chronic disease/inflammation has similarities to iron deficiency anemia including fatigue, shortness of breath, weakness, pallor and headaches

A

True

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81
Q

What type of anemia?

Normocytic, normochromic, mild to moderate anemia

A

Anemia of chronic disease/inflammation

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82
Q

What type of anemia?

Abnormally low reticulocyte count

A

Anemia of chronic disease/inflammation

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83
Q

How is anemia of chronic disease/inflammation treated?

A

Treat causative condition

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84
Q

What type of anemia?

Megaloblastic, macrocytic anemia resulting from cobalamin deficiency characterized by atrophic gastritis and achlorhydria.

A

Vitamin B12 deficiency/pernicious anemia

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85
Q

What is cobalamin?

A

Vitamin B12

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86
Q

What is Vitamin B12 deficiency/pernicious anemia associated with?

A

Autoimmune disease
H. pylori infection

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87
Q

Name 3 autoimmune diseases associated with Vitamin B12 deficiency/pernicious anemia

A

Type 1 DM
Graves disease
Addison disease

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88
Q

What age group is affected by Vitamin B12 deficiency/pernicious anemia?

A

Older (40-70 yrs)

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89
Q

What sex/race is affected more by Vitamin B12 deficiency/pernicious anemia?

A

Women, particularly black

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90
Q

T/F: A rare form of Vitamin B12 deficiency/pernicious anemia is hereditary and more frequent in patients of northern European ancestry, especially Scandinavia

A

True

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91
Q

What is the most common cause of Vitamin B12 deficiency/pernicious anemia?

A

Autoimmune destruction of gastric mucosa -> suppresses intrinsic factor

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92
Q

What antibodies are present in patients with Vitamin B12 deficiency/pernicious anemia?

A

Parietal cell ABs
Intrinsic factor ABs

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93
Q

What type of anemia is associated with the following histologic features?

Atrophy of gastric glands
Achlorhydria
Intestinal metaplasia w/ goblet cells
Loss of parietal cells

A

Vitamin B12 deficiency/pernicious anemia

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94
Q

What type of anemia?

Onset is insidious and vague; pts may be asymptomatic initially

A

Vitamin B12 deficiency/pernicious anemia

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95
Q

What is the classic triad of symptoms for Vitamin B12 deficiency/pernicious anemia?

A

Weakness
Glossitis
Paresthesias

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96
Q

What does glossitis mean?

A

Sore/burning tongue

97
Q

What type of anemia?

GI symptoms include:
Anorexia
Pyrosis
Nausea
Vomiting

A

Vitamin B12 deficiency/pernicious anemia

98
Q

What type of anemia?

Mild jaundice; lemon yellow skin from pallor and jaundice

A

Vitamin B12 deficiency/pernicious anemia

99
Q

What type of anemia?

Complex neurologic syndrome secondary to methionine deficiency due to deficiency of methionine synthase

A

Vitamin B12 deficiency/pernicious anemia

100
Q

What is methionine synthase?

A

Vitamin B12 dependent enzyme

101
Q

What is required to form the essential AA methionine and to transform folate to its active form?

A

Methionine synthase

102
Q

What nerves are affected first in the complex neurologic syndrome seen in Vitamin B12 deficiency/pernicious anemia?

A

Peripheral nerves

103
Q

What symptom of Vitamin B12 deficiency/pernicious anemia?

Pts complain of paresthesias and diminished vibratory and positional sense (proprioception)

A

Complex neurologic syndrome

104
Q

What symptom of Vitamin B12 deficiency/pernicious anemia?

Posterior spinal cord columns become impaired; pts complain of difficulty with balance and walking (unsteady gait)

A

Complex neurologic syndrome

105
Q

What symptom of Vitamin B12 deficiency/pernicious anemia?

Cerebral function may be altered; impaired memory, delirium or dementia may occur

A

Complex neurologic syndrome

106
Q

What is the oral presentation of Vitamin B12 deficiency/pernicious anemia?

A

Glossitis (tongue, lips, mucosa)
Patchy areas of oral mucosa erythema and atrophy

107
Q

T/F: pts can have non-hematologic signs and symptoms of Vitamin B12 deficiency for months before they develop a detectable anemia or any RBC abnormalities

A

True

108
Q

T/F: Anemia is absent in 20% of patients with diagnosed with vitamin B12 deficiency

A

True

109
Q

T/F: Macrocytosis is absent in > 30% of patients at the time of diagnosis for vitamin B12 deficiency

A

True

110
Q

How is the diagnosis of vitamin B12 deficiency made?

A

Low vitamin B12 (< 100 pg/mL)

111
Q

What type of anemia?

High MCV, MCH
Normal MCHC

A

Vitamin B12 deficiency/pernicious anemia

112
Q

What type of anemia?

Thrombocytopenia
Mild leukopenia

A

Vitamin B12 deficiency/pernicious anemia

113
Q

What type of anemia?

Low reticulocytes

A

Vitamin B12 deficiency/pernicious anemia

114
Q

What type of anemia?

Anisocytosis and poikilocytosis with large, egg-shaped macro-ovalocyte RBCs

A

Vitamin B12 deficiency/pernicious anemia

115
Q

What type of anemia?

Hypersegmented neutrophils (lobe count > 5)

A

Vitamin B12 deficiency/pernicious anemia

116
Q

What type of anemia?

High serum methylmalonic acid and homocytesine

A

Vitamin B12 deficiency/pernicious anemia

117
Q

What helps differentiate Vitamin B12 deficiency/pernicious anemia from other anemias?

A

High serum methylmalonic acid and homocytesine

118
Q

How is Vitamin B12 deficiency/pernicious anemia treated?

A

Intramuscular injections of vitamin B12

119
Q

How is Vitamin B12 deficiency/pernicious anemia a problem for dentistry?

A

DO NOT use nitrous oxide!!!

120
Q

Why should you not use nitrous oxide in pts with poor controlled Vitamin B12 deficiency/pernicious anemia?

A

Nitrous oxidizes cobalt atom in vitamin B12 -> methionine synthase + vitamin B12 become inactive

121
Q

How is folate obtained from the diet?

A

Green vegetables
Fruit

122
Q

What type of anemia?

Result of inadequate dietary intake, sometimes complicated by increased metabolic demands

A

Folate deficiency anemia

123
Q

What type of anemia?

May stem from problems with absorption in the jejunum or metabolism

A

Folate deficiency anemia

124
Q

What type of anemia?

Develops within months, as body stores are minimal

A

Folate deficiency anemia

125
Q

What happens if intracellular stores of folate decrease? What does this cause?

A

DNA replication is blocked -> causes megaloblastic anemia

126
Q

What type of anemia?

Onset is insidious, associated with non-specific symptoms such as weakness and easy to fatigue

A

Folate deficiency anemia

127
Q

What type of anemia?

Clinical features are very similar to vitamin B12 deficiency, but does NOT result in neurologic abnormalities

A

Folate deficiency anemia

128
Q

What type of anemia?

Megaloblastic, macrocytic anemia

A

Folate deficiency anemia

129
Q

How do you distinguish folate deficiency anemia from vitamin B12 deficiency?

A

Serum and RBC folate
Vitamin B12 levels

130
Q

What type of anemia?

High serum homocysteine
Normal methylmalonic acid

A

Folate deficiency anemia

131
Q

A prolonged low (chronic) rate of bleeding nearly always results in a deficiency of what? When severe, what is it characterized by?

A

Iron

Hypochromic anemia

132
Q

What are the 3 causes of chronic blood loss?

A

GI (ulcer, cancer, polyp)
Genitourinary (cancer, stone)
Gynecologic (menstrual)

133
Q

RBC destruction or reduced RBC life span

A

Hemolysis

134
Q

What are the 2 types of hemolysis?

A

Congenital
Acquired

135
Q

Congenital or acquired hemolysis?

Due to intrinsic RBC defect

A

Congenital hemolysis

136
Q

Congenital or acquired hemolysis?

Effects are manifested in childhood; a family history of anemia is common

A

Congenital hemolysis

137
Q

Congenital or acquired hemolysis?

Abnormal marrow or systemic abnormality/disease; RBC itself is perfect

A

Acquired hemolysis

138
Q

Congenital or acquired hemolysis?

Family history is normal; the onset of anemia can be pinpointed

A

Acquired hemolysis

139
Q

These disorders can result from a congenital defect in hemoglobin structure, in RBC metabolism, or in hemoglobin synthesis

A

Congenital hemolytic anemias

140
Q

Complications include jaundice, bilirubinate
gallstones, iron overload (hemosiderosis), and the effects of chronic anemia (fatigue, weakness, and cardiac enlargement and failure)

A

Congenital hemolytic anemias

141
Q

What are hemoglobinopathies and hemolytic anemias caused by?

A

Congenital, hereditary abnormality in the Hgb molecule

142
Q

Hemoglobinopathies and hemolytic anemias can be demonstrated by electrophoresis in the __________ portion of the molecule

A

globin

143
Q

What is the normal predominant hemoglobin in adults?

A

Hemoglobin A (HbA)

144
Q

What does Hemoglobin A (HbA) consist of?

A

2 alpha chains
2 beta chains

(a2b2)

145
Q

Which hemoglobins do adults usually have small amounts of?

A

Hemoglobin A2 (HbA2)
Hemoglobin F (HbF)

146
Q

When abnormal hemoglobin occurs in the _____________ state along with normal adult hemoglobin, a nonsymptomatic “_________” condition usually results

A

heterozygous; trait

147
Q

When abnormal hemoglobin is present in the _____________ state (or when a person is heterozygous for two abnormal hemoglobin types), a clinical “__________” characterized by hemolysis usually occurs

A

homozygous; disease

148
Q

What are the congenital hemolytic anemias?

A

Sickle cell
Thalassemia

149
Q

A congenital hemoglobin synthesis disorder characterized by the production of an abnormal
hemoglobin molecule and a resulting chronic hemolytic anemia

A

Sickle cell

150
Q

What is the abnormal hemoglobin molecule in sick cell anemia?

A

Hemoglobin S (HbS)

151
Q

What is the MOST COMMON hereditary hemolytic anemia?

A

Sickle cell anemia

152
Q

Which gene is transmitted as an autosomal codominant trait in sickle cell anemia?

A

HbS gene

153
Q

Sickle cell anemia predominantly affects which group of people?

A

People of African descent

154
Q

What is sickle cell anemia caused by?

A

Mutation in the Beta chain of hemoglobin

155
Q

In sickle cell anemia, a mutation in the Beta chain of hemoglobin results from what?

A

Single AA substitution -> valine for normal glutamic acid at position 6

Result = abnormal hemoglobin (HbS)

156
Q

HbS is ___________ and polymerizes into long strands when exposed to various stressors, including _____________ and _________.

This results in affected RBCs to deform into a characteristic _______ shape

A

unstable; hypoxemia (deoxygenation); acidosis

sickle

157
Q

Chronic RBC membrane damage locks cells into an abnormal sickle shape; along with abnormal ___________ to vascular endothelium, this leads to obstruction in the _____________ and causes
painful ____________ crises and ___________ of the affected RBCs

A

adherence; microcirculation; vaso-occlusive; hemolysis

158
Q

What is the life span of a RBC in sick cell anemia?

A

25 days

159
Q

Hemolysis in sickle cell disease (SCD) is mainly ____________

A

extravascular (ex: spleen)

note: some degree of intravascular hemolysis occurs too

160
Q

Severe, life-threatening condition with over 85 to 95% of normal hemoglobin being replaced by HbS

A

Sickle cell disease (SCD)

161
Q

In patients with sickle cell trait (the heterozygous HbS state), each RBC contains ~ 30 to 45% HbS.

Is this enough to alter RBC morphology?

A

No! Therefore, sickle cell trait carriers are asymptomatic and have normal life expectancy

162
Q

What is the MOST COMMON clinical manifestation of SCD?

A

Vaso-occlusive crises

163
Q

Characterized by acute, intermittent episodes of unremitting pain and tenderness that can occur in any part of the body but most often occurs in the chest, abdomen, or extremities, often accompanied by fever, tachycardia, and anxiety

A

Vaso-occlusive crises

164
Q

Severity of pain varies from trifling to agonizing; duration ranges from a few hours to 2 weeks (average is a few days)

A

Vaso-occlusive crises

165
Q

What are vaso-occlusive crises in SCD due to?

A

Sickled RBCs disrupt blood flow in microvasculature -> tissue ischemia

166
Q

How do most vaso-occlusive crises occur?

A

Spontaneously

167
Q

Can be precipitated by:

Hypoxia
Dehydration
Infection
Systemic acidosis
Fever
Stress/anxiety
Cold or abrupt changes in temp
Menses
Excessive exercise
Alcohol consumption

A

Vaso-occlusive crises

168
Q

How many painful vaso-occlusive crises that require medical attention happen per year in a pt with SCD?

A

1

169
Q

What are the following clinical presentations/complications associated with?

Vaso-occlusive crises
Splenic sequestration
Dactylitis
Stroke
Infection
Renal failure
Bone/joint ischemia

A

Sickle cell disease

170
Q

In children with SCD, there is moderate ____________ due to red pulp ___________ caused by sudden entrapment of sickled RBC

A

splenomegaly; congestion

171
Q

In SCD, chronic splenic ___________ produces hypoxic damage and infarcts, which over time
reduces the spleen to a useless lump of fibrous tissue. This process, referred to as _______________, is complete by adulthood

A

erythrostasis; autosplenectomy

172
Q

Acute, painful swelling or inflammation of fingers or toes

A

Dactylitis

173
Q

Which clinical presentation of SCD?

Affects the bones of the hands and feet and is
accompanied by fever and leukocytosis

A

Dactylitis

174
Q

What is often the first acute symptomatic event in young children with SCD?

A

Dactylitis

175
Q

The risk of _________ ________ in patients with SCD increases with age

A

ischemic stroke

176
Q

What is the following describing in SCD?

Large artery arteriopathy, secondary to hyperplasia of the intima from repeated endothelial injury

A

Most common mechanism underlying stroke

177
Q

What is the leading cause of death in adults with SCD?

A

Acute chest syndrome

178
Q

Which clinical presentation of SCD?

Fever, tachypnea, wheezing, cough, chest pain, and leukocytosis, often with a sudden drop in hemoglobin

A

Acute chest syndrome

179
Q

Which clinical presentation of SCD?

Can create a vicious circle of worsening pulmonary and systemic hypoxemia, sickling, and vaso-occlusion

A

Acute chest syndrome

180
Q

Which clinical presentation of SCD?

Common precipitating factors = infection, pulmonary infarction, fat embolism from bone marrow

A

Acute chest syndrome

181
Q

What is the most common cause of death in children with SCD?

A

Strep pneumoniae infection

182
Q

What are 2 other common causes of infections in SCD?

A

H influenzae
Salmonella

183
Q

_____________ begins early in life in pts with SCD, and renal damage/dysfunction progress with age

A

Glomerulopathy

184
Q

In pts with SCD, 30% have renal _________ and some progress to __________ __________ __________ requiring tx with dialysis

A

insufficiency; chronic renal failure

185
Q

What can lead to aseptic (avascular) necrosis of bones, chronic arthropathy, and increased susceptibility to osteomyelitis in pts with SCD?

A

Bone and joint ischemia

186
Q

Where does aseptic (avascular) necrosis of the bones usually occur?

A

Femoral or humeral heads

187
Q

What disease is frequently caused by S aureus and salmonella?

A

Osteomyelitis

188
Q

What disease?

Pallor and evidence of jaundice in oral mucosa

A

SCD

189
Q

What disease?

Erythropoietic activity is increased, and dental X-Rays associated with the bone marrow hyperplasia include increased widening and decreased numbers of trabeculations and generalized osteoporosis

A

SCD

190
Q

How do the trabeculae btwn teeth appear in SCD? What is this due to?

A

Horizontal rows (step ladder)

Due to compensatory marrow expansion

191
Q

How does the lamina dura appear in SCD?

A

More dense/distinct

192
Q

Areas of __________ bone have been reported in pts with SCD

A

sclerotic

193
Q

What disease?

Pts often have delayed eruption of teeth and dental hypoplasia

A

SCD

194
Q

In SCD, vaso-occlusive events can promote ischemic necrosis within the ___________ and
___________ ____________

A

mandible; peripheral neuropathy

195
Q

What disease?

Moderate to severe anemia
Marked reticulocytosis

A

SCD

196
Q

What disease?

WBC count fluctuates substantially and unpredictably during and between vaso-occlusive crises, infectious episodes, and other intercurrent illnesses

A

SCD

197
Q

In SCD, irreversibly sickled cell comprise what % of RBCs?

A

5-50%

198
Q

What disease?

Reticulocytes
Nucleated RBCs (normoblasts)
Target/bullseye cells (codocytes)

A

SCD

199
Q

What disease?

High serum lactic dehydrogenase

A

SCD

200
Q

Why do pts with SCD have high serum lactic dehydrogenase?

A

RBC hemolysis

201
Q

Inherited disorders of hemoglobin caused by genetic mutations leading to decreased synthesis of either the alpha or beta globin chain of hemoglobin A.

A

Thalassemias

202
Q

Caused by defective synthesis of the alpha globin chain

A

Alpha thalassemia

203
Q

Caused by defective synthesis of the beta globin chain

A

Beta thalassemia

204
Q

What disease?

Occurs predominantly in patients from the Mediterranean region

A

Beta thalassemia

205
Q

What disease?

Transmitted as an autosomal codominant trait

A

Beta thalassemia

206
Q

What disease?

Caused by > 200 point mutations that lead to defects in the transcription, splicing, or translation of beta globin mRNA, resulting in defective beta globin chain synthesis.

A

Beta thalassemia

207
Q

What are the 2 mechanisms that defective synthesis of beta globin contributes to anemia?

A
  1. Inadequate HbA formation
  2. Accumulation of unpaired alpha globin chains -> damage to RBCs
208
Q

Which mechanism that defective synthesis of beta globin contributes to anemia?

Results in microcytic, poorly hemoglobinized (hypochromic) RBCs

A

Inadquate HbA formation

209
Q

Which mechanism that defective synthesis of beta globin contributes to anemia?

Form toxic precipitates that severely damage the membranes of RBCs and erythroid
precursors resulting in hemolysis

A

Accumulation of unpaired alpha globin chains

210
Q

What are the 3 forms of beta thalassemia?

A

Beta thalassemia minor
Beta thalassemia major
Beta thalassemia intermedia

211
Q

Which form of beta thalassemia?

Heterozygous form

A

Beta thalassemia minor

212
Q

Which form of beta thalassemia?

Mild, asymptomatic anemia and microcytosis

A

Beta thalassemia minor

213
Q

Which form of beta thalassemia?

Low/normal Hgb

A

Beta thalassemia minor

214
Q

Which form of beta thalassemia?

No significant clinical manifestations, no organ failure; compatible with normal life span

A

Beta thalassemia minor

215
Q

Which form of beta thalassemia?

Homozygous form

A

Beta thalassemia major

216
Q

Which form of beta thalassemia?

Severe anemia, blood transfusion independence

A

Beta thalassemia major

217
Q

Which form of beta thalassemia?

Severe complications related to bone marrow expansion and iron overload

A

Beta thalassemia major

218
Q

Which form of beta thalassemia?

Clinical severity of disease is somewhere between beta thalassemia minor and major

A

Beta thalassemia intermedia

219
Q

Which form of beta thalassemia?

Pts are either homozygous or compound heterozygous

A

Beta thalassemia intermedia

220
Q

Which form of beta thalassemia?

Pt is able to maintain Hgb level compatible with survival (7-11 g/dL) without blood transfusion dependence

A

Beta thalassemia intermedia

221
Q

Severe anemia begins 6 months after birth in infants with beta thalassemia ________

A

major

222
Q

What disease?

Growth retardation, short stature, delayed puberty

A

Beta thalassemia major

223
Q

What disease?

Skeletal deformities due to massive bone marrow expansion and hyperplasia from extramedullary hematopoiesis

A

Beta thalassemia major

224
Q

What disease?

Abnormal facial structure (“chipmunk facies”) and skeletal bone fractures

A

Beta thalassemia major

225
Q

What disease?

Splenomegaly
Hepatomegaly w/ jaundice
Lymphadenopathy

A

Beta thalassemia major

226
Q

Why do splenomegaly, hepatomegaly w/ jaundice, and lymphadenopathy occur in pts with beta thalassemia major?

A

Extramedullary hematopoiesis
Gallstones
Cirrhosis

227
Q

The clinical course of beta thalassemia major is improved significantly by what?

A

Blood transfusions

228
Q

What is used to treat beta thalassemia major but leads to hemosiderosis with heart failure, cardiac arrhythmias, cirrhosis, endocrinopathies, and pseudoxanthoma elasticum?

A

Blood transfusions

229
Q

Transfusional iron overload

A

Hemosiderosis

230
Q

Calcification and fragmentation of the elastic fibers of skin, retina, and cardiovascular system

A

Pseudoxanthoma elasticum

231
Q

What disease?

Massive bone hyperplasia affects the jaws, producing painless enlargement of mandible + maxilla

A

Beta thalassemia

232
Q

What disease?

Chipmunk “facies” with frontal bossing (prominent forehead), maxillary hypertrophy, depression of nasal bridge, Class II malocclusion

A

Beta thalassemia

233
Q

What disease?

Skull radiograph shows “hair on end” or “crew cut” appearance of the calvaria

A

Beta thalassemia

234
Q

What disease?

Severe anemia
Low RBCs, MCV, MCHC
High RDW

A

Beta thalassemia major

235
Q

What disease?

Nucleated RBCs (normoblasts)
Target cells
Anisocytosis
Poikilocytosis
Polychromatophilia

A

Beta thalassemia major

236
Q

What disease?

RBCs = deformed, microcytic, hypochromic

A

Beta thalassemia major

237
Q

What disease?

HbA is absent
HbF and HBA2 are high

A

Beta thalassemia major

238
Q

What disease?

High indirect bilirubin
High lactate dehydrogenase
Low haptoglobin

A

Beta thalassemia major

239
Q

Binds with high affinity to free hemoglobin

A

Haptoglobin

D2 Fall (45 decks)

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