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Essential Haematology > Aplastic anemias &Secondary Anemias > Flashcards

Aplastic anemias &Secondary Anemias Flashcards

1
Q

What is Aplastic Anemia

A

Defined as pancytopenia resulting from aplasia of the bone marrow

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2
Q

Describe the marrow in Aplastic Anemia

A

Hypo cellular Marrow in which normal haemopoietic marrow replaced by fat cells

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3
Q

What is the pathophysiology of Aplastic Anemia

A

A reduction in the number of hematopoietic pluripotential stem cells

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4
Q

What is pancytopenia

A

Describes a reduction in the blood count of all major cell lines- red cells, white cells and platelets

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5
Q

Aplastic Anemia is classified into which two types

A

Primary (congenital or acquired)

Secondary

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6
Q

What are the two types of primary causes of Aplastic Anemia

A

Congenital

Idiopathic acquired

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7
Q

What are the two types of Congenital Aplastic Anemia

A

Fanconi and Non Fanconi Types

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8
Q

What are the four main secondary causes of Aplastic Anemia

A

Ionizing radiation
Chemicals
Drugs
Viruses

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9
Q

List examples of exposure to Ionizing Radiation that may cause Aplastic Anemia

A

Radiotherapy
Radioactive isotopes
Nuclear power stations

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10
Q

Exposure to which drugs/ chemicals may lead to Aplastic Anemia

A

Cytotoxic drugs:
Benzene
Organophosphates
DDT and other pesticides

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11
Q

True or False

Exposure to Cytotoxic drugs that causes Aplastic Anemia is usually reversible

A

True

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12
Q

Which drugs occasionally cause bone marrow aplasia

A

Chloramphenicol
NSAID’s
Insectisdes
Gold

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13
Q

List four drugs that regulary cause marrow depression

A

Busulfan
Cyclophosphamide
Anthracyclines
Nitrosoureas

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14
Q

List four viruses that cause Aplastic Anemia

A

Hepatitis non A non B
Parvovirus B19
EBV (rare)
HIV

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15
Q

Hepatitis non A non B can cause Aplastic Anemia about how many weeks after infection?

A

6-12 weeks

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16
Q

Parvovirus leads to transient aplasia of which blood line

A

Red cell

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17
Q

What fraction of Aplastic Anemia cases are no underlying cause found

A

2/3

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18
Q

What is the pattern of inheritance of Fanconi caused Aplastic Anemia

A

Autosomal recessive

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19
Q

When is the onset of Aplastic Anemia

A

Any age

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20
Q

When is the peak incidence for aplastic anemia

A

30 yrs

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21
Q

Which gender is slightly more susceptible to Aplastic Anemia

A

Male

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22
Q

List some clinical features of Aplastic Anemia

A

Non specific, the symptoms are due to the pancytopenia effect

  • infections
  • bruising/ bleeding gums, epistaxis(nose bleed), menorrhagia
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23
Q

True or false

Lymph nodes, liver and spleen are enlarged in Aplastic Anemia

A

False

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24
Q

Describe the colour and size of RBCs in Aplastic Anemia

A

Normochromic

Normocytic or
Macrocytic (MCV 95-110fl)

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25
Q

Is the reticulocyte count high or low in Aplastic Anemia

A

Low

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26
Q

The range in fall of granulocytes in Aplastic Anemia is usually below what value

A

1.5 x 10^9 / L

:

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27
Q

True or false

Neutrophil appears normal in Aplastic Anemia

A

True

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28
Q

In severe cases of Thrombocytopenia in Aplastic Anemia the values fall below what number

A

20 x 10^9 / L

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29
Q

Does Aplastic Anemia present with abnormal cells in peripheral blood

A

Nope!

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30
Q

In Aplastic Anemia the bone marrow shows hypoplasia, with loss of haemopoietic tissue which is replaced with what

A

Fat cells

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31
Q

What is the importance of the Trephine Biopsy

A

May show patchy cellular areas in a hypocellular background

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32
Q

What is the type of biopsy done to examine bone marrow and test for Aplastic Anemia

A

Trephine Biopsy

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33
Q

In the trephine biopsy of a pt with Aplastic Anemia, which cells are mainly present

A

Lymphocytes

Plasma cells

34
Q

In the trephine biopsy of a pt with Aplastic Anemia, which cells are severely reduced or absent

A

Megakaryocytes

35
Q

Severe cases of Aplastic Anemia show what percentage of Marrow cellularity

A

<25%

36
Q

What is the peripheral blood criteria for Severe Aplastic Anemia

A

Granulocytes <0.5 x 10^9
Platelets < 20 x 10^9/l
Corrected retic count <1%

37
Q

What is the criteria for Severe Aplastic Anemia based on Bone marrow trephine

A

Marked hypocellularity <25%

Moderate hypocellularity 25% -50%

38
Q

List five tests used to help diagnose aplastic anemia

A 
Liver function test to rule out hepatitis
Urine hemosiderin
Hams test
Human leukocyte antigen
Cytogenetics studies
39
Q

Why is a liver function test done when diagnosing Aplastic anemia

A

To rule out hepatitis as the cause

40
Q

Why is urine hemosiderin test done when diagnosing a plastic anemia

A

To rule out chronic intravascular hemolysis as the cause

41
Q

What is Ham’s test

A

This is a blood test used to diagnose paroxysmal nocturnal hemoglobinuria PNH

42
Q

What is the name of the blood test used to diagnose paroxysmal nocturnal hemoglobinuria

A

Ham’s test

43
Q

Why is Ham’s test done when diagnosing aplastic anemia

A

To rule out paroxysmal nocturnal hemoglobinuria

44
Q

What is the name of the blood test used to identify the best donor for bone marrow transplant

A

Human leukocyte antigen (HLA) typing

45
Q

Why is human leukocyte antigen typing done when diagnosing aplastic anemia

A

To determine the best donor for a bone marrow transplant

46
Q

List three examples of supportive management of patients with a plastic anemia

A

Packed red blood cell transfusion (ideally WBC depleted)
Platelet transfusion
Prevention/management of infections

47
Q

List five examples of specific management for a plastic anemia

A

Stem cell transplant
Immunosuppressive therapy
Anabolic steroids
High dose methylprednisolone (immunosuppressive agent)
Granulocyte macrophage colony stimulating factor GM- CSF

48
Q

Define stem cell transplant

A

It is the process by which normal haematopoiesis is establish by infusion of pluripotent stem cells to reestablish marrow function in a patient with defective or damaged bone marrow

49
Q

List five types of stem cell transplant

A 
Syngeneic
Allogeneic related match
Allogeneic unrelated match
Partially mismatched related
Autologous
50
Q

Who is a Syngeneic blood donor

A

Identical twin

51
Q

Who is an autologous blood donor

A

Self

52
Q

What are three sources of stem cells

A

Peripheral blood stem cells PBSC
Bone marrow
Cord blood

53
Q

List seven early complications of stem cell transplant

A 
Nausea and vomiting
Mucositis
Diarrhea
Aplasia 
Hemorrhagic cystitis
Alopecia
Hepatic Veno-occlusive disease
54
Q

List four late complications of stem cell transplant

A

Stability
Growth disturbances
Cataracts
Second-degree malignancies

55
Q

List three complications of stem cell transplant

A

Graft rejection
Graft versus host disease
Leukemic relapse

56
Q

List seven differential diagnosis of pancytopenia

A 
Aplastic anemia
Megaloblastic anemia
Bone marrow infiltration
Myelofibrosis
Hypersplenism
Proximal nocturnal hemoglobinuria
Myelodysplastic syndrome
57
Q

Define Myelofibrosis

A

It is a rare type of blood cancer in which the bone marrow is replaced by fibrous scar tissue

58
Q

Define pure red blood cell aplasia PRCA

A

It is a rare disorder of blood production in which the bone marrow fails to function in an adequate manner resulting in anemia

59
Q

List one congenital cause of pure red blood cell aplasia PRCA

A

Diamond Blackfan Anemia

60
Q

List four cause of Acquired Pure Red Cell Aplasia PRCA

A

Infection - parvovirus B 19
Thymoma
Hematological malignancies
Idiopathic

61
Q

What is another name for a secondary anemia

A

Anemia of chronic disorder

62
Q

List three causes of secondary anemia

A

Chronic infections
Inflammatory disorders
Malignancy

63
Q

List five characteristic features of Pure Red Cell Aplasia PRCA
Interms of : colour, reticulocyte level, bone marrow invasion, severity

A

Normochromic normocytic or mildly hypochromic indices
Reticulocytes normal or decreased mild non-progressive anemia
Does not require bone marrow invasion
Severity depends on the disease activity

64
Q

What is the morphology of the red blood cells in PRCA

A

Normochromic
Normocytic
Mildly hypochromic

65
Q

What is the level of reticulocytes in PRCA

A

Normal or slightly decreased

66
Q

When does PRCA usually develop

A

In the first few months of illness

67
Q

What is the pathogenesis of PRCA

A 
Cytokine mediated process
Shortened RBC survival
Impaired marrow response
Abnormal iron metabolism
TNF, IL-1 interferons
68
Q

What is the treatment for PRCA

A

Treat the underlying cause

Erythropoietin can be used in some cases

69
Q

What percentage of erythropoietin is produced in the renal cortex

A

90%

70
Q

90% of erythropoietin is produced where

A

Renal cortex

71
Q

What percent of erythropoeitin is produced in the liver

A

10%

72
Q

10% of erythropoietin is produced in which organ

A

The liver

73
Q

What is the stimulus for erythropoietin production in the kidney

A

Oxygen tension

74
Q

What is the role of Erythropoietin

A

Increases the number of progenitor cells committed to erythropoiesis

75
Q

What are the normal blood levels of erythropoietin

A

11-48 mU/mL ( milliunits per millilitre)

76
Q

List seven indications for treatment of PRCA with Erythropoietin

A 
Chronic renal failure
Multiple myeloma
Myelodysplastic syndrome
Cancer chemotherapy
Chronic diseases
HIV
AZT antiretroviral drug treatment
77
Q

What is the dose of Erythropoietin administered per week To treat PRCA

A

150 units/kilograms three times per week

78
Q

The maximum effect of PRCA with erythropoietin occurs in which week of treatment

A

4-8 week

79
Q

What is the half time of erythropoietin

A

4 to 13 hours

80
Q

Where is erythropoietin metabolized

A

By the liver

81
Q

List five effects of treatment with erythropoietin

A 
Depletion of iron stores
Hypertension, edema
Congestive cardiac failure
Thrombosis, myocardial infarction, stroke
Seizures, headaches

Essential Haematology (14 decks)

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