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HEMATOLOGY HEMATOPOIESIS > RBC Disorders part 1 > Flashcards

RBC Disorders part 1 Flashcards

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
Q

Reticulocyte count:
< 2% (hypoproliferative)

A
  • Leukemia
  • Aplastic Anemia
  • Pure red cell aplasia
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26
Q

Reticulocyte count:
> 2% (hyperproliferative)

A
  • Hemorrhage
  • Hemolytic anemia
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27
Q

Presence of megalocytes indicates what ff. conditions?

A
  • Vitamin B12 deficiency
  • Folic acid deficiency
  • Drug induced
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28
Q

Absence of megalocytes indicates what ff. conditions?

A
  • Alcohol abuse
  • Myelodysplastic syndrome
  • Liver diseases
  • Congenital bone marrow failure syndromes
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29
Q

Represent disordered hemoglobin synthesis

A

Microcytic anemia

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30
Q

microcytic anemia is caused by?

A
  • Inadequate iron
  • Abnormal globin formation
  • Deficiencies in heme & porphyrin synthesis
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31
Q

Commonest cause of microcytic anemia is _____________

A

iron deficiency

32
Q

Second most frequent type is the _________________

A

anemia of chronic disease

33
Q

What condition refers to the altered erythrocyte maturation caused by a clonal expansion of abnormal hematopoietic stem cells

A

Myelodysplastic anemia

34
Q

What condition refers with an MCV of 100 to 110, but typically without anemia; relatively caused by alcoholism

A

Macrocytic anemia

35
Q

*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

A

Normocytic anemia

36
Q

Name three (3) laboratory diagnosis for anemic diseases

A
  • Reticulocyte count
  • Corrected Reticulocyte count
  • Reticulocyte index
37
Q

Enumerate the parameters for hypoproliferative anemia

A
  • erythrocytes are usually normocytic
  • reticulocyte index < 2
  • E:M ratio is < 1:2
  • Indirect bilirubin and LDH are normal
38
Q

Enumerate the parameters for maturation defects

A
  • 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
Q

Enumerate the parameters for hemolysis

A
  • Reticulocyte index: > 3
  • E:M ratio is 1:1
  • Serum LDH & indirect bilirubin are characteristically elevated
  • Polychromatophilia is prominent
40
Q

What does NIMBLE stands for?

A

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
Q

Stage 1 of iron deficiency is characterized by?

A

progressive loss of storage iron

42
Q

Stage 2 of iron deficiency is defined by?

A

exhaustion of the storage pool of iron

43
Q

Stage 3 of iron deficiency is?

A

frank anemia
(the hemoglobin concentration and hematocrit are low relative to the reference intervals.)

44
Q

What are the S/S for anemia?

A
  • Feeling weak or tired all the time
  • Shortness of breath
  • Increased susceptibility to Infection
  • Cold hands or feet
  • Pallor
  • Pica
45
Q

Which hemoglobin would decrease in iron deficiency anemia?

A
  • Hemoglobin A2
46
Q

*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

A

Thalassemia

47
Q

Which classification of thalassemia indicates beta-chains are absent or diminished?

A

Beta-thalassemia

48
Q

Which classification of thalassemia indicates alpha-chains are affected

A

Alpha-thalassemia

49
Q

*absence (β0) or a marked decrease (β+) of β- chain production
*an excess of α-chains
*Ineffective erythropoiesis

A

B-thalassemia major (Cooley’s thalassemia)

50
Q

*caused by the β0-thalassemia gene with absent, or the β+-thalassemia gene with reduced, β-globin chain synthesis

A

Heterozygous B-Thalassemia

51
Q
  • Lepore Hemoglobins
  • an abnormal δβ-fusion chain is produced, a result of chromosome crossing-over and fusion of genetic material at the δβ-genes
A

δβ+-Thalassemia

52
Q

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

a-Thalassemia

53
Q

Give the corresponding analogy
______________: one inactive gene
_______________: two inactive a genes
Hb H disease : __________________
Hydrops fetalis w/ Hb bart: __________________

A
  • Silent carrier state
  • A-thalassemia trait
  • three inactive a genes
  • four inactive a genes
54
Q

*Complete absence of a-chain
*Incompatible w/ life
*Infants are stillborn

A

Hemoglobin Bart’s Hydrops Fetalis

55
Q

*Formed from tetrads of Beta chains
*Hemolobin H inclusion
*Low Red cell count, very low MCV, Low RDW

A

Hemoglobin H disease

56
Q

*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

A
  • Hemoglobin Constant Spring (aCSa/)
57
Q

*Hereditary recessive microcytic anemia
*Does not respond to oral iron therapy

A

Iron refractory Iron Deficiency Anemia

58
Q

*caused by subacute or chronic infections,
*impaired iron ferrokinetic
*Diminished erythropoiesis
*Shortened red blood cell life span

A

Anemia of chronic inflammation

59
Q

Which hormone is produced by hepatocytes to regulate body iron levels particularly absorption of iron in intestine & release of macrophages and hepatocytes?

60
Q

What hormone is important to prevent phagocytize bacteria from using intracellular iron for their metabolic processes

A

lactoferritin

61
Q

*Characterized by the presence of ringed sideroblasts in the bone marrow
*Associated with mitochondrial iron loading in marrow erythroid precursors (ringed sideroblast ) & ineffective erythropoiesis

A

Sideroblastic anemia

62
Q

Five or more iron granules encircling at least one third of the circumference of the nucleus

A

RIng sideroblast

63
Q

Antituberculosis drugs
*Lead poisoning
*Chloramphenicol
*Copper deficiency
*Zinc overload
*Ethanol-induced anemia most common of the reversible sideroblastic anemias
*Primary pyridoxine deficiency

A

secondary sideroblastic anemia

64
Q

Lead interferes with heme synthesis by blocking the enzymes ______,______, & _________

A

ALAS, ALA dehydratase, and heme synthase

65
Q

*a classic finding associated with lead toxicity.

A

Basophilic stippling

66
Q

*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

A

Porphyrias

67
Q

Most common acute and probably the most common inherited porphyria

A

Acute Intermittent Porphyria

68
Q

South African porphyria

A

Variegate Porphyria

69
Q

*most common of the porphyrias in the United States *Deficient enzyme is uroporphyrinogen decarboxylase (UROD)

A

Porphyria Cutanea Tarda

70
Q

*recessive disorder
*Patients present shortly after birth with red- pigmented urine, hemolytic anemia, and severe cutaneous photosensitivity
*Worst prognosis

A

Congenital Erythropoietic Porphyria

71
Q

partial deficiency of the enzyme ferrochelatase

A

Erythropoietic Protoporphyria

72
Q

Major inherited sideroblastic anemia

A

Hereditary hemochromatosis

73
Q

excessive amounts of iron that accumulate in the blood

A

Hemosiderosis

74
Q

accumulation in the parenchyma causing injury to the tissue

A

Hemochromatosia

75
Q

*Middle aged men
*Hepatomegaly
*“bronze diabetes”

A

Hereditary hemochromatosis

HEMATOLOGY HEMATOPOIESIS (7 decks)

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