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98 BM Failure Flashcards

Chapter 98 Bone Marrow Failure Syndromes

1
Q

What are the bone marrow failure states?

A

Aplastic anemia
Myelodysplastic syndrome
Pure red cell aplasia
Myelophthisis

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2
Q

More frequent hematologic finding in bone marrow failure states

A

Pancytopenia

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3
Q

True or false. Hematopoietic failure syndromes are classed based on peripheral blood smear features.

A

False. Classified by dominant morphologic characteristic features of the bone marrow

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4
Q

Hypocellular bone marrow +/- pancytopenia

A 
Q fever
Legionnaires disease
Anorexia nervosa
starvation
Mycobacterium
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5
Q

Pancytopenia with cellular bone marrow

A 
Primary bone marrow diseases
MDS
PNH
Myelofibfozjs
BM lymphoms
Hairy cell leukemia
================
Secondary systemic disease
SLE
Hypersplenism
B12, Folate deficiency
Alcohol
HIV infection
Sepsis
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6
Q

Pancytopenia with hypocellular bone marrow

A

Acquired Aplastic anemia

Rare a leukemic leukemia

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7
Q

Pancytopenia with marrow hypocellularity

A

Aplastic anemia

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8
Q

True or false. Aplastic anemia is related to PNH and MDS

A

True

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9
Q

Major cause of aplastic anemia

A

Idiopathic

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10
Q

Major acute sequela of radiation; radiation damages DNA

A

Marrow plasia

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11
Q

True or false. Aplastic anemia can be secondary to radiation, drugs and chemicals, viruses, and immune diseases

A

True

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12
Q

Chemical notorious in causing bone marrow failure

A

Benzene

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13
Q

Example of agents that produce marrow depression as major toxicity

A

Cytotoxic drugs

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14
Q

Agents associated with Aplastic anemia but with relatively low probability

A 
Chloramphenicol
Insecticides
Antiprotozoals
NSAIDs
Anticonvulsant
Heavy metals
Sulfonamides
Antihistamines
Estrogen
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15
Q

Acquired mutation in what gene in hematopoietic stem cell is required for the development of PNH?

A

PIG-A

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16
Q

What is the clone progeny deficient in PIG A mutation?

A

Glycosylphospatodylinositol-linked cell surface membrane proteins

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17
Q

Autosomal recessive disorder that manifest as congenital developmental anomalies, progressive pancytopenia and increased risks of malignancy; short stature, cafe au lait spots, and anomalies involving the things, radius and GUT

A

Fanconi anemia

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18
Q

Most common generic defect in Fanconi Anemia

A

Mutation in FANCA

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19
Q

Most fanconi anemia gene products form protein complex that activates with what that play a role in cellular response to DNA damage esp interstrand cross linking

A

FANCD2

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20
Q

Characterized by triad of mucous membrane leukoplasia, dystrophic nails, reticular hyperpigmentation, and with development of aplastic anemia in childhood

A

Dyskeratosis congenita

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21
Q

Dyskeratosis congenital is due to mutation in what genes which acts to maintain telomere length in replicating

A

Mutations in DKC1 (dyskerin); genes of the telomere repair complex

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22
Q

Other autosomal mutation related to Dyskeratosis congenita due to mutation RNA template

A

TERC

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23
Q

Genetic mutations associated with Dyskeratosis congenita in the catalytic reverse transcriptase telomerase

A

TERT

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24
Q

Genetic mutation associated with Dyskeratosis congenita in genes that Encode shelterin proteins which bind telomere DNA

A

TNF2

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25
Q

Syndrome which presents early in life with neutropenia wth pancreatic insuffiency and Malabsorption with mutation in SBDS that affect ribosomal biogenesis

A

Shwachman-Diamond syndrome

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26
Q

Physical anomaly that gives clues to TERT and TERC mutations

A

Early hair graying

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27
Q

What is apparent in the morphology of the bone marrow biopsy specimen in Aplastic anemia?

A

Replacement of bone marrow with fat

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28
Q

Marker of early hematopoietic cells; it is increased or decreased in Aplastic anemia?

A

CD34 antigen is a marker of early hematopoietic cells and is diminished in Aplastic anemia

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29
Q

Responsible for Benzene induced tissue injury

A

Hydroquinone and quinolones

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30
Q

Most common early symptom of aplastic anemia

A

Bleeding

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31
Q

Unusual first symptom of aplastic anemia

A

Infection

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32
Q

What is the character of the bone marrow aspirate in Aplastic anemia

A

Readily aspirated but dilue on smear;may be grossly pale on withdrawal due to fats

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33
Q

What test is done on peripheral blood in children and young adults to exclude Fanconi anemia

A

Diepoxybutane
Mitomycin C
Shortened telomere suggest telomerase and shelterin mutation

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34
Q

How is Aplastic anemia diagnosed?

A

Straight forward: pancytopenia with fatty bone marrow

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35
Q

True or false. Aplastic anemia is disease of the elderly population

A

False. It is the leading diagnosis of pancytopenia in adolescent and young adult

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36
Q

What is the natural history of aplastic anemia?

A

Rapid deterioration and death

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37
Q

The major prognostic determination

A

Blood count

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38
Q

What is the criteria severe Aplastic anemia?

A 
2 out of 3
1. Neutrophils count less than 500/uL
2. Platelet count less than 20K/uL
3. Reticulocyte count less than 1% OR
Absolute Reticulocyte count less than 60K/uL
39
Q

Better predictors of treatment response and long term outcome

A

Lymphocytes more than 1K/uL

Reticulocyte count more than 25K/uL

40
Q

How can Aplastic anemia be cured?

A

Aplastic anemia can be cured by stem cell transplant

41
Q

True or false. Glucocorticoids are the primary therapy in Aplastic anemia

42
Q

In preparation for stem cell transplantation, patient with Aplastic anemia should be given blood transfusion

A

Blood transfusion fro. Family members should be avoided to prevent sensitization to histocompatibility antigen

43
Q

Induces hematolofic recovery in 60-70% of patient with Aplastic anemia

A

Antithymocyte Globulin + cyclosporine

44
Q

What antithymocyte Globulin are available?

A

Horse ATG

Rabbit ATG: less effective

45
Q

Characterized by cutaneous eruptions and arthalgia developing 10 days after initiating treatment

A

Serum sickness

46
Q

Administered with ATG to ameliorate the immune consequences of heterologous protein infusion

A

Methylprenisolone

47
Q

Important side effect of cyclosporine

A

Nephrotoxicity, hypertension and seizures

48
Q

What is curative treament of aplastic anemia? What is the treatment of choice for Aplastic anemia?

A

Curative: stem cell transplant

Treatment of choice: immunosuppression

49
Q

True or false. Survival is higher in patient receiving stem transplantation versus immunosuppresion

A

False. Survival between both is equivalent

50
Q

True or false. Hematopoietic growth factors such as EPO and G CSF are effective in managing pancytopenia in Aplastic anemia

A

False. Endogenous blood levels of these factors are already high

51
Q

Added to first line immunosuppresion therapy in Aplastic anemia

A

Eltrombopag

52
Q

Up regulate telomerase gene activity and improve marrow function

53
Q

Should be added at the 50th transfusion to avoid secondary hemochromatosis

A

Deferoxamine and deferasirox

54
Q

Single best method of preventing spread of infection in Aplastic anemia

A

Handwashing

55
Q

Characterized by anemia, Reticulocytopenia and absent or rare erythroid precursors cells in the bone marrow

A

Pure red call aplasia

56
Q

True or false. Pure red cell aplasia in adults is genetic

A

False. In adults, PRCA is acquired

57
Q

What is the etiology of PRCA

A

Mutations in ribosome protein

58
Q

True or false. PRCA has important associations with immune system diseases

59
Q

What immune system disease is PRCA frequently a major manifestation

A

Large granular lymphocytosis

Complicate chronic lymphocytic leukemia

60
Q

True or false. EPO has provoked PRCA mediated by neutralizing antibodies to hormone

61
Q

More common immune mechanism in PRCA

A

T cell inhibition

62
Q

Important curable cause of PRCA

A

Chronic parvo virus infection

63
Q

Cytopathic sign of B19 parvo virus infection

A

Giant pronormoblasts

64
Q

True or false. PRCA has poor long term survival

A

False. Long survival with supportive care of blood transfusion and iron chelation

65
Q

What other drugs given to PRCA

A 
Glucocorticoids
Cyclosporine
ATG
Azathioprine
Cyclosphosphamide
66
Q

Two main characteristic of myelodysplasia

A
  1. Cytopenias due to bone marrow failure

2. High risk development of AML

67
Q

5 subtypes of Myelodysplastic syndrome

A
  1. Refractory anemia
  2. Refractory anemia with sideroblasts
  3. Refractory anemia with excess blast
  4. Refractory anemia with excess blast in transformation
  5. Chronic myelomonocytic leukemia
68
Q

Single somatic mutation a feature of Sideroblastic anemia

69
Q

True or false. Myelodysplastic syndrome is a disease of the elderly. What is the mean age of onset.

A

True. Mean age of onset is 70 years old

70
Q

True or false. MDS is associated with environmental exposures such as radiation and benzene

71
Q

True or false. Therapy related MDS occur as early toxicity of cancer treatment

A

False. Late toxicity

72
Q

Common chemothepeutic agents associated with MDS

A

Ankylating agents: busulfan, nitrosourea, procarbazine

DNA topoisomerase inhibitors

73
Q

Pathophysiology of Myelodysplastic syndrome

A

Disordered cell proliferation and impaired differentiation

74
Q

What cytopenia dominate the early course of Myelodysplastic syndrome?

75
Q

True or false. Patients with Myelodysplastic syndrome have splenomegaly.

A

True. About 20%

76
Q

What is the bone marrow picture of MDS?

A

Bone marrow is usually normal or hypercellular in 20% of cases but not single characteristic feature of marrow morphology distinguishes MDS

77
Q

Differential diagnoses of MDS

A

B12 or Folate deficiency should be excluded

78
Q

Criterion set by WHO to differentiate between AML and MDS

A

AML has 20% blast in bone marrow

79
Q

True or false. Most patients die as result of complications from pancytopenia rather than from leukemic transformation

80
Q

What is the only cure of MDS

A

Hematopoietic stem cell transplantation offers cure of MDS

81
Q

True or false. MDS is refractory to cytotoxic chemotherapy regimens

82
Q

Hypomethylating agents for MDS

A

Azacitudine

Decitabine

83
Q

Major toxicity of decitabine and azacitudine?

A

Myelosuppresion

84
Q

Effective in reversing anemia in MDS, a derivate of thalidomide

A

Lenalidomide

85
Q

What are the toxicities of lenalidomide?

A

Myelosuppresion

DVT and PE

86
Q

Monoclonal antibody effective in young patients with MDS? Anti CD52

A

Alemtuzumab

87
Q

Another name of secondary myelofibrosis

A

Myelophthisis

88
Q

What are the three distinct features of myelophthisis

A
  1. Proliferation of fibroblasts in the marrow space (myelofibrosis)
  2. Extension of hematopoiesis into the long bones and into extramedullary sites usually spleen, liver and Lymph nodes (myeloid metaplasia)
  3. Ineffective erythropoiesis
89
Q

Infection that precedes Aplastic anemia

A

Seronegative hepatitis

90
Q

Causes transient Aplastic anemia

A

Parvo virus B19

91
Q

Agents that regularly produce marrow depression as major toxicity in commonly used doses or normal exposure

A

Cytotoxic drugs: ankylating agents, antimetabolite, antimitotics, some antibiotics
Table 98-3

92
Q

Agents that frequently but not inevitably produce marrow aplasia

93
Q

Agents associated wiht Aplastic anemia but with a relatively low probability

A 
Chloramphenicol
Antiprotozoals
Anticonvulsant
Heavy metals
Sulfonamides
Antihistamines
A penicillamine
Estrogen
94
Q

Agents associated with Aplastic anemia is more tenuous

A 
Streptomycin, tetracycline, methicillin, mebendazole
Sedatives tranquilizers
Allopurinol
Methydopa
Quinidine
Lithium
Guanidine
Potassium perchlorate
Thiocyanate
Carbimazole

Hematology (11 decks)

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