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Clinical Medicine 2 > Aplastic anemia > Flashcards

Aplastic anemia Flashcards

1
Q

What type of anemia is characterized by failure of the bone marrow to produce peripheral blood cells and their progrenitors?

A

aplastic anemia

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2
Q

In aplastic anemia, the (…) stem cells do not repopulate in the bone marrow, resulting in severely diminished or absent comitted (…) cells

A
  • hematopoietic stem cells
  • progenitor cells
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3
Q
  • The definition of aplastic anemia is a bone marrow failure syndrome defined by peripheral blood (….) and hypocellular bone marrow (…)
  • This is also known as (…) which is the combination of not making enough WBCs, RBCs, and platelets
  • What is the incidence of aplastic anemia?
  • What is the predominant age of patients with aplastic anemia?
A
  • pancytopenia; aplasia
  • pancytopenia
  • 2 cases per million
  • 2 peaks: 15-25 year olds, or after 60 years
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4
Q
  • Aplastic anemia can be either (…) or (…)
  • What do patients present with?
  • No cause (idiopathic) is identified in around (…)% of aplastic anemia cases
A
  • acquired or congenital
  • transfusion-dependent anemia, thrombocytopenia, and severe neutropenia with markedly reduced bone marrow cellularity (< 25% cellularity)
  • 50%
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5
Q
  • What is found in 30-80% of patients with idiopathic anemia?
  • What do many of these patients respond to?
A
  • abnormal expression of suppressor T cells
  • immunosuppressive therapy
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6
Q

What are some causes of aplastic anemia?

A
  • inherited factors
  • pregnancy
  • idiopathic cause
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7
Q

What inherited factors can lead to aplastic anemia?

A
  • Fanconi’s anemia
  • reticular dysgenesis
  • congenital dyskeratosis
  • non-hematologic syndromes (down syndrome, etc)
  • diamond-blackfan syndrome
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8
Q

What is the most common inherited form of aplastic anemia?

A

Fanconi’s anemia

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9
Q

What are some common acquired factors that can lead to (or increase risk of) aplastic anemia?

A
  • toxins
  • drugs
  • ionizing radiation
  • infections
  • nutritional deficiencies
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10
Q

What infections can lead to aplastic anemia?

A
  • hepatitis C
  • HIV
  • EBV
  • parvovirus B19
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11
Q
  • Some forms of acquired aplastic anemia are associated with an associated (…) or (…)
  • A small percentage of patients with (…) can transform into aplastic anemia
A
  • thymoma or autoimmune disorder (SLE)
  • paroxysmal nocturnal hemoglobinuria (PNH)
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12
Q

What is the differential diagnosis of pancytopenia (aplastic anemia) with hypocellular bone marrow?

A
  • acquired aplastic anemia
  • inherited aplastic anemia
  • hypoplastic MDS (myelodysplasia)
  • hypoplastic AML (acute myeloid leukemia)
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13
Q
  • Aplastic anemia is usually present with manifestations of (…)
  • What are some symptoms of aplastic anemia?
  • What are signs of aplastic anemia on a physical exam?
A
  • low blood counts
  • low platelets, anemia, neutropenia
  • low platelets, anemia
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14
Q

What symptoms are associated with low platelets, anemia, and neutropenia d/t aplastic anemia?

A
  • low platelets: epistaxis, bleeding gums, bruising w/ mild or no trauma, menorrhagia
  • anemia: fatigue, dyspnea on mild exertion
  • neutropenia: less commonly present with ongoing bacterial/fungal infection
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15
Q

What signs are associated with low platelets and anemia d/t aplastic anemia?

A
  • low platelets: petechiae (palatal or cutaneous), ecchymoses, and epistaxis
  • anemia: pallor
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16
Q

Autoimmune aplastic anemia may have (…)

A

hepatosplenomegaly

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17
Q
  • The work-up of aplastic anemia primarily consists of what?
  • This generally shows paucity or absence of (…) and (…) precursor cells
  • Patients with pure red cell aplasia demonstrate only an absence of (…) precursors in the marrow
A
  • bone marrow aspiration and biopsy
  • erythropoietic and myelopoietic precursor cells
  • RBC precursors
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18
Q

What will a CBC show if a patient has aplastic anemia?

A
  • pancytopenia
  • macrocytosis and toxic granulation of neutrophils
  • isolated cytopenias in eary stages
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19
Q

What will a reticulocyte count show if a patient has aplastic anemia?

A

reticulocytopenia; precursors are gone

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20
Q

What are some additional laboratory tests you can do if a patient has suspected aplastic anemia?

A
  • Ham test
  • peripheral blood flow cytometry to exclude PNH
  • evaluaton for Hep C
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21
Q

What test shows lysis of the patient’s RBCs in acidified fresh serum, which is specific for paroxysmal nocturnal hemoglobinuria?

A

Ham test

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22
Q

What type of imaging can you do to evaluate aplastic anemia?

A

MRI

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23
Q

What imaging technique has a spin-echo sequence that helps with the study of bone marrow disease, and the high fat content of aplastic marrow can be easily seen on this?

A

MRI

24
Q
  • What should you do if a patient is sound to have pancytopenia?
  • If these are negative, what should you do next?
A
  • examine blood smear; evaluate for signs of B12/folate deficiency
  • examine bone marrow biopsy and aspirate to see overall cellularity, cellular morphology, cytogenetics
25
Q
  • If there is no hypoceullarity on bone marrow biopsy and aspirate, what can be ruled out?
  • If there is hypocellularity, what do you look at next?
  • If this is yes, what is the diagnosis?
  • If this is no, what is the diagnosis?
A
  • aplastic anemia
  • presence of myelodysplastic morphology or clonal cytogenetic abnormalities
  • myelodysplasia
  • aplastic anemia
26
Q

What will a peripheral blood smear show in the diagnosis of aplastic anemia?

A
  • normochromic-normocytic RBCs
  • normal MCV and MCH
  • pancytopenia
  • no abnormal cells
27
Q

What will a bone marrow evaluation show in the diagnosis of aplastic anemia?

A
  • hypocellular (decreased precursor cells) bone marrow with increased fat spaces, lymph cells, plasma cells, macrophages, mast cells
28
Q

In aplastic anemia, it is important with bone marrow assessment to obtain both a (…) and (…)

A

biopsy and aspiration

29
Q
  • What best assesses overall bone marrow cellularity and provides the most sensitive evidence of some infiltrative processes in aplastic anemia?
  • What can be examined microscopically for the presence of abnormal cells but also provides cells for cytogenetic analyses (which can provide evidence supporting hypoplastic myelodysplasia and acute leukemia?
A
  • biopsy
  • aspirated sample from bone marrow
30
Q

What severity of aplastic anemia is this describing:
- bone marrow cellularity <25%
- peripheral blood neutrophil count < 500
- peripheral blood platelet count < 20,000
- peripheral blood corrected reticulocytes < 1%

A

severe

31
Q

What severity of aplastic anemia is this describing:
- peripheral blood neutrophil count < 200

A

very severe

32
Q

What severity of aplastic anemia is this describing:
- hypocellular bone marrow with peripheral blood cytopenias

A

non-severe

33
Q
  • With acute treatment of aplastic anemia, aggressive treatment of neutropenic fever is done with (…)
  • You can also administer (…) and (…) transfusions prn, but, it is important to avoid in (…) transplant candidates
A
  • parenteral broad-spectrum antibiotics
  • platelet and RBC transfusions
  • bone marrow transplant candidates
34
Q
A
35
Q

What is the acute treatment of moderate aplastic anemia?

A
  • observation/treatment prn
  • ATG: antithymocyte globulin
  • non-responders: CSA (cyclosporine A)
36
Q
  • What does ATG (antithymocyte globulin) do in acute treatment of moderate aplastic anemia?
  • How is this usually given? How long?
A
  • kills T-lymphocytes, allowing bone marrow to resupply stem cells
  • IV for 8-12 hours a day for 4 days
37
Q

What does CSA (cyclosporine A) do in acute treatment of moderate aplastic anemia?

A

prevents T lymphocytes from becoming active

38
Q

What is the acute treatment of severe aplastic anemia in children and young adults?

A
  • human leukocyte antigen (HLA) typing
  • if no sibling for bone marrow transplant, ATC and CSA (70% effective if used together)
39
Q
  • What is the acute treatment of severe disease of aplastic anemia in older adults?
  • When should you see a blood count improvement?
A
  • ATG and CSA first-line
  • at 3-6 months
40
Q

If patients are not responding to acute treatment for severe aplastic anemia, what is considered?

A
  • non-match BMT, androgens and/or 2nd immunosuppression
  • allogenic hematopoietic stem cell transplantation (HSCT)
41
Q

What is the non-pharmocological acute treatment for aplastic anemia?

A

discontinue offending agents

42
Q

What can be a curative chronic treatment for those with aplastic anemia?

A

allogenic bone marrow transplantation (ABMT) from an HLA-matches sibling donor

43
Q
  • How can patients without siblings be chronically treated for aplastic anemia?
  • What is the risk they are taking with this?
A
  • can be treated with an unrelated match (bone marrow transplant)
  • mortality rate is higher
44
Q
  • In a bone marrow transplant, what affects mortality?
  • The longer the (…) is associated with an increased 5 year survival?
A
  • telomere mutation characteristics
  • telomere length
45
Q

Pertaining to chronic treatment of aplastic anemia:
- (…) therapy with (…) is an effective alternative therapy is non-candidate for ABMT (bone marrow transplant)
- (…) also have a role in treatment
- (…) such as (…) are effective 2nd line agents in aplastic anemia treatment
- What can improve hematopoiesis in refractory severe aplastic anemia?

A
  • immunosuppressive therapy with ATG
  • ISAs (beta blockers)
  • androgens such as danazol
  • oral thrombopoietin mimetic, eltrombopag (tx for thrombocytopenia)
46
Q

What ISAs have a role in the chronic treatment of aplastic anemia?

A
  • cyclosporine
  • cyclophosphamide
  • corticosteroids
47
Q

What is the preferred treatment for children and young adults (<40) with aplastic anemia with an allele-level HLA-matched doner, especially if from a sibling donor?

A

hematopoietic cell transplantation

48
Q

(…) and (…) have been used for those patients with aplastic anemia who are unable to have a hematopoietic cell transplant

A

ATG and cyclosporine immunotherapy

49
Q
  • What is common with ATG and cyclosporine treatment with patients with aplastic anemia that receive this treatment?
  • However, long term outlook, as a result of disease (…) or evolution to (…) or (…) is poor
A
  • remission
  • relapse or evolution to oligobastic or polyblastic (acute) myelogenous leukemia
50
Q

Approximately 15% of patients with aplastic anemia treated with immunotherapy express an abnormal (…) or an overt (…)

A
  • karyotype
  • clonal myeloid neoplasm (PNH or oligoblastic/polyblastic acute myelogenous leukemia)
51
Q
  • Relapsed patients who have already taken immunotherapy for aplastic anemia may respond to a second administration of (…) or (…)
  • Often, if (…) or (…) is used initially, the alternative is used in retreatment, if available
A
  • ATG and cyclosporine
  • horse or rabbit ATG
52
Q
  • (…) alone has resulted in a favorable hematologic response in approximately 50% of patients treated who have aplastic anemia
  • In adults, 150 mg/day is administered for (…) weeks is considered appropriate; lower doses are used in (…) and children and patients of (…) descent
A
  • eltrombopag
  • 24 weeks
  • adolescents
  • Asian
53
Q
  • Up to 75% of patients with aplastic anemia response to immunosuppressive therapy with (…), (…), and (…)
  • The most recent update for bone marrow transplantation statistics reports long-term survival rate of (…)%
  • What are the major complications of ABMT?
  • All patients with aplastic anemia should be referred to (…)
A
  • antithymocyte globin (ATG), corticosteroids, and cyclosporine
  • 80%
  • graft rejection and graft-vs-host disease
  • hematology
54
Q

A person is admitted with an autoimmune disease directed against the hematopoietic stem cells. This will produce:
1. aplastic anemia
2. iron-deficiency anemia
3. sideroblastic anemia
4. fanconi anemia

A
  1. aplastic anemia
55
Q

In a patient with pancytopenia, which of the following abnormalities in the peripheral blood smear would provide a useful indicator that the cause of the pancytopenia is aplastic anemia?
1. hypersegmented polymorphononuclear leukocytes
2. nucleated red cells
3. teardrop-shaped red cells
4. giant platelets
5. none of the above

A
  1. none of the above: peripheral smear is usually normal
56
Q

Which is showing the bone parrow of a person with aplastic anemia?

A

right

57
Q

Which is showing a peripheral blood smear of a patient with aplastic anemia?

A

bottom

Clinical Medicine 2 (9 decks)

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