Haematology / Abnormal Red Cell Count and Bleeding Flashcards

1
Q

What is anemia?

A

Reduced mass of circulating red blood cells (RBCs) demonstrated by decreased hemoglobin and hematocrit levels

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2
Q

What labs are included in the initial evaluation of anemia? (3)

A

FBC, RBC indices, and peripheral smear

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3
Q

What are the typical signs and symptoms of anemia?(7)

A
  • conjunctival and skin pallor
  • postural dizziness
  • fatigue
  • dyspnea (at rest or exertional)
  • palpitations
  • tachycardia
  • high output congestive heart failure (CHF)
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4
Q

How can you determine whether or not the bone marrow is responding appropriately to anemia?

A

The reticulocyte index (RI) should be elevated.

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5
Q

What are reticulocytes?

A

Immature RBCs that normally account for 1%-2% of circulating RBCs

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6
Q

How do you calculate the RI?

A

Percent reticulocytes × (actual hematocrit/ideal hematocrit)

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7
Q

What does an RI <2% indicate?

A

Decreased RBC production (bone marrow is not responding)

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8
Q

What does an RI >2% indicate?

A

Increased RBC production: bone marrow is responding appropriately to RBC loss or destruction

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9
Q

What is mean corpuscular volume (MCV)?

A

The average volume of RBCs

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10
Q

What is the most common cause of normocytic (MCV = 80-100) anemia from decreased RBC production?

A

Anemia of chronic disease (ACD)

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11
Q

Describe the pathogenesis of ACD. (3)

A

A chronic infectious, inflammatory, or neoplastic disease causes anemia by any one or a combination of the following:

  • decreased response to or production of erythropoietin
  • bone marrow hypoactivity
  • shortened RBC life span.
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12
Q

What is erythropoietin?

A

A glycoprotein hormone, primarily made by the kidney, which regulates red blood cell production

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13
Q

Besides ACD, what are other causes of normocytic anemia from decreased RBC production? (4)

A
  • bone marrow infiltration
  • aplastic anemia
  • chronic renal failure and other chronic disease
  • endocrine dysfunction (eg, hypopituitarism, hypothyroidism)
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14
Q

What is aplastic anemia?

A

Failure of bone marrow to make all blood cell types because the bone marrow stem cells are damaged. It can be idiopathic, genetic, or acquired.

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15
Q

What are the causes of normocytic anemia from increased RBC loss? (3)

A
  • acute blood loss
  • genetic and acquired hemolytic anemias
  • sequestration from hypersplenism
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16
Q

In hemolytic anemia, Reticulocyte count typically increased or decreased?

A
  • Reticulocyte count is Increased
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17
Q

In hemolytic anemia, LDH typically increased or decreased?

A

Lactate dehydrogenase Increased

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18
Q

In hemolytic anemia, Uncojugated bilirubin typically increased or decreased?

A

Unconjugated bilirubin Increased

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19
Q

In hemolytic anemia, Haptoglobin bilirubin typically increased or decreased?

A

Haptoglobin is decreased

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20
Q

In hemolytic anemia, urine hemosiderin typically increased or decreased?

A

Urine hemosiderin is increased

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21
Q

Sickle cell disease, hereditary spherocytosis, hereditary elliptocytosis, glucose-6-phosphate dehydrogenase deficiency, and pyruvate kinase deficiency are all genetic causes of what type of anemia?

A

Hemolytic anemia

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22
Q

Paroxysmal nocturnal hemoglobinuria causes what type of hemolytic anemia?

A

Acquired hemolytic anemia

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23
Q

What type of acquired hemolytic anemi is caused by Warm/cold agglutinin disease?

A

Autoimmune hemolytic anemia

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24
Q

What type of acquired hemolytic anemia is caused by HELLP syndrome in preeclampsia?

A

Mechanical

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25
Q

What type of acquired hemolytic anaemia is caused by Blood transfusion with incompatible blood type?

A

Alloimmune

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26
Q

What type of acquired hemolytic anaemia is caused by Penicillin/Quinine/Levodopa?

A

Drug-induced

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27
Q

A Direct Coombs test for the presence of antibodies against RBCs is positive in what type of anemia?

A

Autoimmune hemolytic anemia

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28
Q

What diseases increase the risk of developing autoimmune hemolytic anemia?(8)

A
  • Chronic lymphocytic leukemia
  • non-Hodgkin lymphoma
  • mycoplasma pneumonia
  • Epstein-Barr infection (mononucleosis)
  • cytomegalovirus infection
  • hepatitis
  • HIV
  • autoimmune diseases (eg, lupus)
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29
Q

What is microcytosis?

A

RBCs are too small (MCV <80)

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30
Q

What is sideroblastic anemia?

A

It is a genetic or acquired microcytic anemia in which iron cannot be correctly used to synthesize hemoglobin and is characterized by the presence of ringed sideroblasts (erythroblasts with abnormal mitochondrial iron deposits) in the bone marrow.

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31
Q

What is the most common cause of acquired sideroblastic anemia?

A

Ethanol abuse

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32
Q

Overexposure to what heavy metal commonly found in soil, batteries, and cable covers can cause poisoning with several toxic effects, including brain damage (learning disabilities, seizures, coma, death) and sideroblastic anemia?

A

Lead

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33
Q

What is the treatment of lead poisoning?

A

Deferoxamine

34
Q

What is basophilic stippling? (2)

A
  • It is a classic peripheral smear finding seen in (but not unique to) lead poisoning.
  • Accumulated ribosomal ribonucleic acid appears as spots on the periphery of RBCs.
35
Q

What are the three main causes of microcytic anemia?

A
  1. Iron deficiency anemia (IDA)
  2. Anemia of chronic disease (normal or low MCV)
  3. Thalassemia
36
Q

Worldwide, what is the most common cause of IDA?

A

Parasitic infection

37
Q

In the United States, what is the most common cause of anemia in children?

A

IDA from inadequate dietary iron

38
Q

In the United States, what is the most common cause of IDA in adults?

A

Chronic blood loss

39
Q

What is the most common cause of chronic blood loss leading to IDA in men and postmenopausal women?

A

Gastrointestinal (GI) bleeding

40
Q

Besides a GI workup, what additional procedure is needed in the workup of IDA in a postmenopausal woman?

A

Endometrial biopsy

41
Q

What is the most common cause of chronic blood loss leading to IDA in reproductive-age women?

A

Menometrorrhagia

42
Q

What is the treatment for IDA?

A

Treat underlying cause of blood loss and give ferrous sulfate supplement

43
Q

How long should you give a ferrous sulfate supplement in order to adequately replenish iron stores?

A

Continue treatment for 2 months after the hemoglobin corrects itself

44
Q

In a person with IDA, effective iron supplementation should increase hemoglobin at what rate?

A

2-4 g/dL every 3 weeks

45
Q

What is pica?

A

A craving for substances not fit as food (such as dirt, paper products, and ice), which is sometimes seen in IDA

46
Q

What syndrome is associated with dysphagia and IDA?

A

Plummer-Vinson

47
Q

What is the triad that characterizes Plummer-Vinson syndrome?

A
  1. Esophageal webs (causing dysphagia)
  2. IDA
  3. Atrophic glossitis
48
Q

Ferritin is an indicator of what mineral stores?

A

Iron

49
Q

What is transferrin?

A

An iron-transporting protein

50
Q

What organ produces transferrin?

A

Liver

51
Q

What is TIBC?

A

An indirect measure of the amount of transferrin in the blood

52
Q

How do you calculate percent TIBC saturation?

A

Serum iron divided by TIBC

53
Q

What is a common iron panel profile for a patient with IDA?(4)

A
  • Low ferritin (<40)
  • high TIBC
  • low percent TIBC
  • saturation (<20%)
54
Q

What is a common iron panel profile for a patient with ACD (clue: iron is present but not available for use)?

A
  • Normal or high ferritin
  • low TIBC
  • normal or low percent TIBC saturation
55
Q

What is thalassemia?

A

Anemia caused by reduced alpha (alpha-thalassemia) or beta (beta-thalassemia) globin protein production leading to decreased hemoglobin production

56
Q

Thalassemia is more common amongst which ethnic groups?

A

Asian, Mediterranean, and African American

57
Q

In a patient with thalassemia, what causes hepatosplenomegaly?

A

Extramedullary hematopoiesis

58
Q

What lab test is used to diagnose thalassemia?

A

Hemoglobin electrophoresis

59
Q

Why is it important not to use iron to treat a patient with thalassemia?

A
  • Patients with thalassemia already have a chronic state of iron overload
60
Q

What causes the chronic state of iron overload in thalassemia?

A
  • increased GI iron absorption secondary to accelerated iron turnover
  • multiple transfusions, or both
61
Q

What is the treatment for thalassemia?

A

Transfusions and iron chelators

62
Q

What is macrocytosis?

A

RBCs are too large (MCV >100)

63
Q

What are the causes of macrocytosis?(6)

A
  • folate deficiency
  • vitamin B 12 deficiency
  • drugs such as hydroxyurea and zidovudine (AZT)
  • increased lipid deposition (liver disease, hypothyroidism, hyperlipidemia)
  • alcohol abuse
  • myelodysplastic disorders
64
Q

Folate and vitamin B 12 deficiencies produce what kind of cells on a peripheral smear?

A

Megaloblasts (large immature RBCs) and/or hypersegmented neutrophils (at least five lobes)

65
Q

Why does it take months for folate deficiency to develop?

A

Folate body stores (10,000 mcg) are small compared to the daily requirement (400 mcg).

66
Q

What is the most common cause of folate deficiency?

A

Poor diet (classically associated with alcoholism)

67
Q

What are other causes of folate deficiency?

A
  • pregnancy/lactation (increased daily folate demand)
  • drugs that interfere with folic acid metabolism (trimethoprim, methotrexate, phenytoin)
68
Q

What is the treatment of folic acid deficiency?

A

Folic acid 1 mg PO daily for 1-4 months

69
Q

Are neurological signs seen in folate or B 12 deficiency, or both?

A
  • Vitamin B 12 deficiency, since B 12 is needed for myelin synthesis
70
Q

What specific neurological signs and symptoms may be seen with B 12 deficiency?

A
  • loss of vibration and position sense
  • weakness
  • spasticity
  • ataxia
  • dementia
71
Q

What causes the loss of vibration and position sense in Vitamin B12 deficiency?

A

Degeneration of dorsal column tracts

72
Q

What causes the weakness and spasticity of Vitamin B12 deficiency?

A

Degeneration of corticospinal (upper motor neuron) tracts

73
Q

Why does it take years for vitamin B 12 deficiency to develop?

A

B 12 body stores (5000 mcg) greatly outweigh the daily requirement (9 mcg).

74
Q

What foods contain B 12 ?

A

Meat and dairy products

75
Q

Where is B 12 absorbed?

A

Terminal ileum

76
Q

What is the most common cause of vitamin B 12 deficiency in adults?

A

Pernicious anemia

77
Q

What is pernicious anemia?

A

Impaired absorption of B 12 secondary to antibodies to gastric intrinsic factor (IF)

78
Q

What are causes of vitamin B 12 deficiency?

A
  • Pernicious anaemia
  • ther causes of impaired absorptione.g
    • gastrectomy or
    • terminal ileal resection
    • competitive bacteria or parasites (tapeworm)
    • pancreatic insufficiency
  • poor dietary intake of B 12 , such as in a strict vegan
  • impaired ability to use B 12
79
Q

What are the treatment options for vitamin B 12 deficiency?

A
  • Vitamin B 12 IM
    • 1 mg daily for 1 week—
    • then 1 mg weekly for 4 weeks
  • vitamin B 12 PO
    • 2 mg PO daily for 4 months
  • vitamin B 12 nasal spray (but comparative studies are lacking)
80
Q

What is polycythemia?

A
  • Increased plasma concentration of red blood cells resulting in increased viscosity of blood (can eventually lead to thrombosis).
  • Primary polycythemia (polycythemia vera) is a myeloproliferative disease.
81
Q

What are the causes of secondary polycythemia?(4)

A
  • body senses relative hypoxia (high altitude, heart or lung disease, chronic smoking, etc)
  • decreased plasma volume with subsequently increased relative red cell concentration (dehydration)
  • erythropoietin or steroid secreting tumors
  • rare genetic causes
82
Q
A