RBC Disorders part 1 Flashcards

(75 cards)

1
Q

How do we consider a person is anemic? (give the Hgb and Hct values)

A

<13 g/dL of Hgb in males (Hct of about 39%)
< 12 g/dL of Hgb in females (Hct about 36%)

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2
Q

State the three (3) causes of Anemia:

A
  1. Blood loss
  2. Decreased or impaired blood cell production
  3. Increased blood cell destruction (hemolytic anemia)
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3
Q

Enumerate the type of anemia according to their respective classifications:

A. Bone marrow Damage, infiltration Atrophy

A
  • Leukemia
  • Leukoerythroblastosis
  • Aplastic anemia
  • Multiple myeloma
  • Myelofibrosis
  • lymphomas
  • Pure red cell aplasia
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4
Q

Enumerate the type of anemia according to their respective classifications:

B. Decreased Erythropoietin

A
  • Anemia of chronic diseases
  • Chronic renal diseases
  • Hypothyroidism
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5
Q

Enumerate the type of anemia according to their respective classifications:

C. Vitamin & Mineral deficiency

A
  • iron
  • Vitamin B12 & Folic acid deficiency
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6
Q

Enumerate the type of anemia according to their respective classifications:

D. Defect in globin synthesis

A
  • Thalassemia
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7
Q

Enumerate the type of anemia according to their respective classifications:

E. Increased iron overload

A
  • Sideroblastic anemia
  • Hemochromatosis
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8
Q

Enumerate the type of anemia according to their respective classifications:

F. Ineffective erythropoiesis

A

Congenital dyserythropoiesis

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9
Q

Enumerate the type of anemia according to their respective classifications:

Intrinsic: Hereditary (membrane)

A
  • Spherocytosis
  • Elliptocytosis
  • Acanthocytosis
  • Stomatocytosis
  • Rh null disease
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10
Q

Enumerate the type of anemia according to their respective classifications:

Intrinsic: Acquired

A
  • Paroxysmal Nocturnal Hemoglobinuria (PNH)
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11
Q

Enumerate the type of anemia according to their respective classifications:

Intrinsic: Hereditary (enzyme)

A
  • Glucose-6-phosphate-dehydrogenase (G6PD)
  • Pyruvate kinase (PK)
  • Glutathione reductase
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12
Q

Enumerate the type of anemia according to their respective classifications:

Intrinsic: Hereditary (hemoglobinopathies)

A
  • Sickle Cell disease
  • Hemoglobin C disease
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13
Q

Enumerate the type of anemia according to their respective classifications:

Intrinsic: unstable

A
  • Hemoglobin E
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14
Q

Enumerate the type of anemia according to their respective classifications:

Intrinsic: Hereditary (defect in globin synthesis)

A
  • Thalassemia
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15
Q

Enumerate the type of anemia according to their respective classifications:

Extrinsic: Immune (Isoimmune)

A
  • Incompatible blood transfusion
  • Hemolytic disease of Newborns
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16
Q

Enumerate the type of anemia according to their respective classifications:

Extrinsic: Non-immune

A
  • Chemical
  • Toxins
  • Venoms
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17
Q

Enumerate the type of anemia according to their respective classifications:

Extrinsic: Immune (autoimmune)

A
  • Warm reacting
  • Cold reacting
  • Drug induced
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18
Q

Enumerate the type of anemia according to their respective classifications:

Extrinsic: Non-immune (physical)

A
  • Burns
  • Cardiac valves
  • Microangiopathic Hemolytic Anemia (MAHA)
  • Hemolytic uremia syndrome (HUS)
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19
Q

Enumerate the type of anemia according to their respective classifications:

Extrinsic: Miscellaneous

A
  • Anemia
  • Sulfhemoglobin
  • Porphyrias
  • Methemoglobin
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20
Q

MCV that is < 80 is classified as?

A

Microcytic anemia

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21
Q

MCV that is > 100 is classified as?

A

Macrocytic anemia

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22
Q

MCV that is 80 - 100 is classified as?

A

Normocytic anemia

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23
Q

Serum iron studies:
low iron and ferritin but high TiBC indicates?

A
  • Iron deficiency Anemia
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24
Q

Serum iron studies:
low iron and ferritin and low TiBC indicates?

A
  • Anemia of chronic diseases
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25
Reticulocyte count: < 2% (hypoproliferative)
- Leukemia - Aplastic Anemia - Pure red cell aplasia
26
Reticulocyte count: > 2% (hyperproliferative)
- Hemorrhage - Hemolytic anemia
27
Presence of megalocytes indicates what ff. conditions?
- Vitamin B12 deficiency - Folic acid deficiency - Drug induced
28
Absence of megalocytes indicates what ff. conditions?
- Alcohol abuse - Myelodysplastic syndrome - Liver diseases - Congenital bone marrow failure syndromes
29
Represent disordered hemoglobin synthesis
Microcytic anemia
30
microcytic anemia is caused by?
- Inadequate iron - Abnormal globin formation - Deficiencies in heme & porphyrin synthesis
31
Commonest cause of microcytic anemia is _____________
iron deficiency
32
Second most frequent type is the _________________
anemia of chronic disease
33
What condition refers to the altered erythrocyte maturation caused by a clonal expansion of abnormal hematopoietic stem cells
Myelodysplastic anemia
34
What condition refers with an MCV of 100 to 110, but typically without anemia; relatively caused by alcoholism
Macrocytic anemia
35
*Bone marrow responds maximally by increasing red cell production and releasing young erythrocytes prematurely *Bone marrow response is reduced because of intrinsic bone marrow disease, insufficient iron, or inadequate erythropoietin effect
Normocytic anemia
36
Name three (3) laboratory diagnosis for anemic diseases
- Reticulocyte count - Corrected Reticulocyte count - Reticulocyte index
37
Enumerate the parameters for hypoproliferative anemia
- erythrocytes are usually normocytic - reticulocyte index < 2 - E:M ratio is < 1:2 - Indirect bilirubin and LDH are normal
38
Enumerate the parameters for maturation defects
- reticulocyte index is < 2 - E:M ration is > 1:1 with severe anemias - serum LDH and the indirect bilirubin are elevated (except in iron deficiency) = polychromasia is present
39
Enumerate the parameters for hemolysis
- Reticulocyte index: > 3 - E:M ratio is 1:1 - Serum LDH & indirect bilirubin are characteristically elevated - Polychromatophilia is prominent
40
What does NIMBLE stands for?
N - i increased as in need for pregnancy, children during rapid stages of growth I - intake is low e.g. maturation M - malabsorption B - blood L - loss i.e. Bleeding E - excessive donation i.e. blood donors
41
Stage 1 of iron deficiency is characterized by?
progressive loss of storage iron
42
Stage 2 of iron deficiency is defined by?
exhaustion of the storage pool of iron
43
Stage 3 of iron deficiency is?
frank anemia (the hemoglobin concentration and hematocrit are low relative to the reference intervals.)
44
What are the S/S for anemia?
- Feeling weak or tired all the time - Shortness of breath - Increased susceptibility to Infection - Cold hands or feet - Pallor - Pica
45
Which hemoglobin would decrease in iron deficiency anemia?
- Hemoglobin A2
46
*Inherited disorders with reduced or absent synthesis of one or more globin chains *Leads to reduce production of functioning hemoglobin leading to hypochromic microcytic erythrocytes
Thalassemia
47
Which classification of thalassemia indicates beta-chains are absent or diminished?
Beta-thalassemia
48
Which classification of thalassemia indicates alpha-chains are affected
Alpha-thalassemia
49
*absence (β0) or a marked decrease (β+) of β- chain production *an excess of α-chains *Ineffective erythropoiesis
B-thalassemia major (Cooley's thalassemia)
50
*caused by the β0-thalassemia gene with absent, or the β+-thalassemia gene with reduced, β-globin chain synthesis
Heterozygous B-Thalassemia
51
- Lepore Hemoglobins - an abnormal δβ-fusion chain is produced, a result of chromosome crossing-over and fusion of genetic material at the δβ-genes
δβ+-Thalassemia
52
most common single-gene disorder in humans. Its distribution is largely limited to tropical and subtropical regions of Asia and Africa and the Mediterranean
a-Thalassemia
53
Give the corresponding analogy ______________: one inactive gene _______________: two inactive a genes Hb H disease : __________________ Hydrops fetalis w/ Hb bart: __________________
- Silent carrier state - A-thalassemia trait - three inactive a genes - four inactive a genes
54
*Complete absence of a-chain *Incompatible w/ life *Infants are stillborn
Hemoglobin Bart's Hydrops Fetalis
55
*Formed from tetrads of Beta chains *Hemolobin H inclusion *Low Red cell count, very low MCV, Low RDW
Hemoglobin H disease
56
*most common of the elongated α-chain variants *common in Southeast Asia, where it is found in about 50% of cases of Hb H disease (αCSα/− −) *Normal CV, low RBC count
- Hemoglobin Constant Spring (aCSa/)
57
*Hereditary recessive microcytic anemia *Does not respond to oral iron therapy
Iron refractory Iron Deficiency Anemia
58
*caused by subacute or chronic infections, *impaired iron ferrokinetic *Diminished erythropoiesis *Shortened red blood cell life span
Anemia of chronic inflammation
59
Which hormone is produced by hepatocytes to regulate body iron levels particularly absorption of iron in intestine & release of macrophages and hepatocytes?
Hepcidin
60
What hormone is important to prevent phagocytize bacteria from using intracellular iron for their metabolic processes
lactoferritin
61
*Characterized by the presence of ringed sideroblasts in the bone marrow *Associated with mitochondrial iron loading in marrow erythroid precursors (ringed sideroblast ) & ineffective erythropoiesis
Sideroblastic anemia
62
Five or more iron granules encircling at least one third of the circumference of the nucleus
RIng sideroblast
63
Antituberculosis drugs *Lead poisoning *Chloramphenicol *Copper deficiency *Zinc overload *Ethanol-induced anemia most common of the reversible sideroblastic anemias *Primary pyridoxine deficiency
secondary sideroblastic anemia
64
Lead interferes with heme synthesis by blocking the enzymes ______,______, & _________
ALAS, ALA dehydratase, and heme synthase
65
*a classic finding associated with lead toxicity.
Basophilic stippling
66
*Make up a group of inherited and acquired disorders of heme biosynthesis caused by a deficiency of a specific enzyme in the biosynthetic pathway, culminating in the excess production and increased excretion of precursors formed in the steps before the enzyme defect
Porphyrias
67
Most common acute and probably the most common inherited porphyria
Acute Intermittent Porphyria
68
South African porphyria
Variegate Porphyria
69
*most common of the porphyrias in the United States *Deficient enzyme is uroporphyrinogen decarboxylase (UROD)
Porphyria Cutanea Tarda
70
*recessive disorder *Patients present shortly after birth with red- pigmented urine, hemolytic anemia, and severe cutaneous photosensitivity *Worst prognosis
Congenital Erythropoietic Porphyria
71
partial deficiency of the enzyme ferrochelatase
Erythropoietic Protoporphyria
72
Major inherited sideroblastic anemia
Hereditary hemochromatosis
73
excessive amounts of iron that accumulate in the blood
Hemosiderosis
74
accumulation in the parenchyma causing injury to the tissue
Hemochromatosia
75
*Middle aged men *Hepatomegaly *“bronze diabetes”
Hereditary hemochromatosis