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Heme > Bone Marrow Failure/HCST > Flashcards

Bone Marrow Failure/HCST Flashcards

1
Q

What is pancytopenia?

A

Anemia, leukopenia, and thrombocytopenia.

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2
Q

What is the definition of bone marrow failure?

A

Pancytopenia due to failure of bone marrow to produce blood cells.

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3
Q

What are the symptoms of anemia?

A

Dyspnea, chest pain, and fatigue.

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4
Q

What are the symptoms of leukopenia/neutropenia?

A

Fever, infection, and mouth sores.

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5
Q

What are the symptoms of thrombocytopenia?

A

Bleeding.

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6
Q

What is the differential diagnosis of pancytopenia?

A
  1. Increased destruction: immune destruction, sepsis
  2. Sequestration: hypersplenism
  3. Decreased production: myelodysplasia, marrow infiltrate, B12 def, aplastic anemia, drugs, viruses, radiation
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7
Q

In simplest terms, what are the two causes of bone marrow failure?

A

Hypercellular or hypocellular marrow.

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8
Q

A hypercellular marrow is the result of what?

A

Infiltration.

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9
Q

What are the 5 causes of bone marrow infiltration?

A
  1. Hematological Malignancies (leukemias, myeloma, lymphoma)
  2. Carcinoma
  3. Storage disorders
  4. Myelodysplastic syndromes
  5. B12 or folate def
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10
Q

What is the cause of a hypocellular marrow?

A

Aplastic anemia.

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11
Q

What are the 2 forms of aplastic anemia?

A
  1. Congenital: Fanconi’s anemia

2. Acquired: idiopathic, myelodysplastic syndrome, drugs/chemicals, radiation, viruses

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12
Q

Define aplastic anemia.

A

Severe, life-threatening syndrome in which production of erythrocytes, WBCs, and platelets has failed.

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13
Q

What patient population is anemia most likely to be found in?

A

All. It may occur in all age groups and both genders.

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14
Q

What is aplastic anemia characterized by?

A

Peripheral pancytopenia accompanied by a hypocellular marrow.

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15
Q

What is the pathophysiology of aplastic anemia?

A

Reduction or depletion of hematopoietic precursor stem cells with decreased production of all cell lines.

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16
Q

What are the 3 causes of decreased stem cells in aplastic anemia?

A
  1. Damage to the pluripotential stem cell
  2. Defective BM microenvironment
  3. Cellular or humoral immunosuppression of hematopoiesis (autoimmune)
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17
Q

What are the 2 congenital causes of aplastic anemia?

A
  1. Fanconi’s anemia

2. Familial aplastic anemia (subset of Fanconi’s anemia)

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18
Q

Describe Fanconi’s anemia.

A

Progressive BM hypoplasia. Usually becomes symptomatic around 5 years of age. Skin hyperpigmentation and small stature also observed.

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19
Q

Most cases of aplastic anemia are due to what?

A

Idiopathic etiology.

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20
Q

Exposure to ionizing radiation can lead to what disease?

A

Aplastic anemia. Hematopoietic cells especially susceptible to it. Whole body radiation of 300-500 rads can completely wipe out the bone marrow.

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21
Q

What 3 groups of chemical agents can lead to aplastic anemia?

A
  1. Contain a benzene ring
  2. Chemotherapeutic agents
  3. Certain insecticides
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22
Q

What 2 drugs are known to result in aplastic anemia?

A
  1. Chloramphenicol

2. Quinacrine

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23
Q

What 5 infections can lead to aplastic anemia?

A
  1. Infectious mononucleosis
  2. Infectious hepatitis
  3. Parvovirus
  4. CMV
  5. Miliary TB
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24
Q

T or F. In rare cases, pregnancy can result in aplastic anemia.

A

T

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25
Q

PNH can lead to what disease?

A

Aplastic anemia. PNH is an autoimmune, stem cell disease in which membranes of RBCs, WBCs, and platelets have abnormalities making them susceptible to complement-mediated lysis.

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26
Q

What are the 5 labratory findings in aplastic anemia?

A
  1. Severe pancytopenia with relative lymphocytosis
  2. Normochromic, normocytic anemia
  3. Mild to moderate anisocytosis and poikilocytosis
  4. Decreased retic count
  5. Hypocellular marrow (>70% yellow marrow)
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27
Q

Why is a relative lymphocytosis observed in aplastic anemia?

A

Lymphocytes live much longer than neutrophils.

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28
Q

What 4 things should treatment of aplastic anemia include?

A
  1. Withdrawal of potentially offending agents
  2. Supportive care: transfusions, antibiotics, etc.
  3. Immunosuppressive regimens
  4. HSCT
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29
Q

What immunosuppressants can be given to a patient with aplastic anemia?

A

ATG, cyclosporine, steroids.

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30
Q

What is pure red cell aplasia?

A

Decreased erythroid precursors. WBCs and platelets unaffected.

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31
Q

What are the 3 causes of acquired pure red cell aplasia?

A
  1. Viral or bacterial infections
  2. Hemolytic anemias: may suddenly halt erythropoiesis
  3. Thymoma: can produce T cell-mediated response against BM erythroblasts or EPO
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32
Q

What is the treatment for pure red cell aplasia?

A

Supportive care and immunosuppression.

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33
Q

Myelodysplastic Syndromes (MDS) are characterized by what?

A

Primary, neoplastic stem cell disorders that tend to terminate in acute leukemia.

34
Q

The BM in MDS is…

A

Normocellular or hypercellular with abnormalities in one or more cell lines. Results in ineffective erythropoiesis and/or granulopoiesis and/or megakaryopoiesis.

35
Q

In MDS, what does the peripheral smear show?

A

Nucleated RBCs, oval macrocytes, pseudo-Pelger-Huet neutrophils with hyperchromatin clumping, hypogranulated neutrophils, and giant bizarre platelets.

36
Q

In MDS, what does BM aspirate show?

A

Ringed sideroblasts and other dysplastic changes.

37
Q

What are Pelger-Huet neutrophils?

A

Hyposegmented neutrophils.

38
Q

What differentiates MDS from aplastic anemia?

A

The presence of a neoplastic clone. Aplastic anemia is NOT a neoplastic condition, MDS is.

39
Q

How is MDS thought to arise?

A

Mutations in the multipotent BM stem cells. Specific defects not known.

40
Q

What is wrong with blood precursor cells in MDS?

A

Impaired differentiation and significant increase in levels of apoptosis in BM cells.

41
Q

T or F. In MDS, clonal expansion of the abnormal cells results in the production of cells without the ability to differentiate.

A

T

42
Q

How do you know if MDS has progressed to AML?

A

Overall percentage of BM blasts rises above a cutoff (20% for WHO and 30% for FAB).

43
Q

What are the goals of therapy in MDS?

A

Control symptoms, improve quality of life, improve overall survival, and delay progression to AML.

44
Q

What does the main therapy for MDS involve?

A

Supportive care with blood product support (transfusions I assume) and hematopoietic growth factors such as EPO.

45
Q

What 2 agents have been shown to decrease blood transfusion requirements, delay progression to AML, and increase survival in MDS patients?

A
  1. 5-Azacytidine
  2. Decitabine
    Both are hypomethylating agents.
46
Q

What is the 5q- syndrome?

A

A subset of MDS.

47
Q

What agent was recently approved in the treatment of 5q- syndrome?

A

Lenalidomide

48
Q

In MDS, what therapy has the potential to be curative? Which patients is it given to?

A

HSCT. Younger patients (<60) and more severely affected patients.

49
Q

Define HSCT.

A

The transplantation of hematopoietic progenitor cells that have the ability to proliferate and repopulate the marrow spaces.

50
Q

What is the current procedure for harvesting stem cells for HSCTs?

A

Patient is given a drug that mobilizes the stem cells out of the marrow and into peripheral blood. They are then harvested via apheresis.

51
Q

What are the 3 unique characteristics of hematopoietic stem cells that make transplants possible?

A
  1. Ability to regenerate in the marrow
  2. Ability to migrate to the marrow following IV infusion
  3. Ability to be cryopreserved (frozen) with little or no damage
52
Q

What is an autologous HSCT?

A

The stem cells used in the transplant are harvested from the patient. They are then frozen and re-infused after high dose chemotherapy.

53
Q

What is the purpose of the high dose chemotherapy used in autoSCTs?

A

It wipes out the patient’s faulty bone marrow to allow repopulation by the good stem cells.

54
Q

Why is there a minimal risk of an adverse reaction with autoSCTs?

A

The stem cells are from the patient!

55
Q

AutoSCTs are used mainly in the treatment of what diseases?

A

Lymphomas and multiple myelomas.

56
Q

What is an allogeneic HSCT?

A

Stem cells are harvested from a related or unrelated HLA matched donor.

57
Q

What is the risk of an alloSCT?

A

Graft vs. host disease.

58
Q

What is another source of stem cells in the absence of an HLA matched donor?

A

Umbilical cord blood.

59
Q

What are the advantages of an alloSCT compared to an autoSCT?

A

Can be used when a patient’s BM fails (i.e. aplastic anemia and MDS). Or when the recipient has a certain disease such as lymphoma or leukemia, the donor cells can attack the tumor cells (basicaly GVHD but for a good cause).

60
Q

What are the 3 disadvantages of alloSCTs?

A
  1. Higher risk of chemotherapy-related complications with myeloblative transplant (mucositis, hepatic veno-occlusive disease)
  2. Higher risk of infection compared to autoSCT (CMV, EBV, fungal, and parasitic)
  3. GVHD
61
Q

What are the 2 types of alloSCTs?

A
  1. Myeloblative

2. Non-myeloblative

62
Q

What is the purpose of myeloablation prior to alloSCT?

A

It wipes out the patients BM to get rid of the disease. Also has an immunosuppressive effect to minimize rejection.

63
Q

What is the purpose of non-myeloablation prior to allSCT?

A

Lower doses of chemotherapy and radiation are used and the BM is not completely wiped out. The goal is to prevent rejection of donor cells.

64
Q

When do you give myeloablative therapy vs. non-myeloablative therapy prior to alloSCT?

A

Mainly avoid myeloablative therapy in older patients (they can’t tolerate it as well). Non-myeloablative therapy allows high-risk patients to receive alloSCTs and potentially be cured.

65
Q

What agents are used in myeloablation?

A

Combination of cyclophosphamide with busulfan or total body irradiation.

66
Q

What are the 5 steps of an alloSCT?

A
  1. Collection: from donor
  2. Processing: stem cells isolated, concentrated, and prepared for transplant
  3. Cryopreservation
  4. Chemotherapy: in recipient to destroy cancer cells and induce BM failure
  5. Infusion: into recipient
67
Q

What must be given to patients following alloSCT?

A

Immunosuppressants. Most transplants require life-long immunosuppression but HSCT recipients can be slowly weaned off them.

68
Q

What are the 4 complications of alloSCTs?

A
  1. Infection
  2. Veno-occlusive disease
  3. Mucositis
  4. GVHD
69
Q

Why are alloSCT recipients prone to infection?

A
  1. Recipient’s BM was destroyed: no immune cells
  2. Immunosuppression: further complicates the myeloablation
  3. Recipients lose acquired immunity: must be re-vaccinated with childhood vaccines once they stop immunosuppressants
70
Q

What is hepatic veno-occlusive disease?

A

Severe liver injury.

71
Q

What are the 3 clinical findings of hepatic veno-occlusive disease?

A
  1. Elevated bilirubin
  2. Hepatomegaly
  3. Fluid retention
72
Q

What is mucositis? How is it treated?

A

Injury of the mucosal lining of the mouth and throat. Not life-threatening but very painful and prevents eating/drinking. Treated with pain meds and IV infusions to prevent dehydration and malnutrition.

73
Q

What is GVHD?

A

Inflammatory disease unique to allogeneic transplants. Donor’s immune cells attack recipient’s tissues.

74
Q

T or F. GVHD only occurs when there is an HLA haplotype mismatch.

A

F: GVHD can occur even if donor and recipient are HLA identical (minor histocompatability issues).

75
Q

Describe acute GVHD.

A

Occurs in the first 3 months post transplant. Involves skin, intestine, or liver. Often fatal.

76
Q

How do you prevent acute GVHD?

A

Treat recipient with cyclosporine and methotrexate to suppress immune response of donor cells.

77
Q

How do you treat acute GVHD? What is the downside to this treatment?

A

High dose steroids. Long term steroid use can lead to severe infections.

78
Q

Describe chronic GVHD.

A

Occurs 3 months after transplant. Less often results in death than acute GVHD. Inflammation same as acute plus fibrosis can develop in skin, liver, intestine, conjunctiva, mucosa, and lungs.

79
Q

Chronic GVHD presents the same clinically as what disease?

A

Scleroderma/autoimmune diseases.

80
Q

What cells usually mediate GVHD?

A

Donor T cells react to minor histocompatability antigens of recipient.

81
Q

What is graft vs. tumor effect?

A

Basically, it’s the good use of GVHD. Similar mechanism as GVHD but instead of attacking recipient tissues, the donor T cells attacks the cancerous cells in the recipient. Leads to lower risk of cancer relapse.

82
Q

Graft vs. tumor effect is most beneficial in which diseases?

A

Slow progressing diseases such as chronic leukemia, low-grade lymphoma, and some multiple myeloma cases.

Heme (38 decks)

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