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Hematology > Hematology Unit 9 (Bone Marrow) > Flashcards

Hematology Unit 9 (Bone Marrow) Flashcards

1
Q

What is the purpose of bone marrow?

A

Hematopoiesis - makes 6 billion cells/kg/day in adults

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2
Q

What happens to bone marrow as we age?

A

At birth, nearly all bones contain red marrow.

As we age, fat cells begin to replace red marrow in the appendicular skeleton.

Red marrow is then limited to only the axial skeleton in late adolescence.

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3
Q

Adipocytes occupy ~____% of red marrow space in 30-70 y/o.

A

50%

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4
Q

BM puncture is PROHIBITED in patients with ___________

A

coagulopathies

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5
Q

Why would a BM exam be ordered on a patient?

A
  • Neoplasia diagnosis/staging
  • BM failure causing cytopenias
  • Metabolic disorders
  • Infections
  • Monitoring of treatment
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6
Q

What are the 2 parts of a BM specimen and how are they different?

A

Aspirate: liquid portion; obtained by BM aspiration and allows for identification of types/proportions of cells and look for morphologic variance

Core biopsy: solid portion; obtained by trephine biopsy and demonstrates bone marrow architecture and estimates cellularity

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7
Q

What is the most commonly used site for a BM collection? What other sites may be collected from, for adults and why?

A

`Posterior superior iliac crest of the pelvis - most common

Other sites:
- Anterior superior iliac crest of the pelvis (for patients who can only lie supine)
- Sternum (aspirate only)
- Spinous process of vertebrae/ribs (rare)

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8
Q

What areas on the body can a BM specimen be collected in children?

A

Posterior superior iliac crest most common

Anterior medial surface of the tibia, can only get aspirate

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9
Q

BRIEFLY list the steps of a BM biopsy/aspiration procedure

A
  • Palpate body site
  • Local anesthetic injected
  • Needle inserted and rotated to core through bone
  • Core biopsy removed
  • Touch preps of biopsy made
  • Aspiration of liquid
  • BM smears made
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10
Q

Advantages of a BM aspirate smear

A

Fast
No need for decalcification
Quantitation of cell type
Material for ancillary studies

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11
Q

Advantages of a BM core biopsy

A

Can analyze cells AND stroma
Represents all cells
Explains dry taps

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12
Q

Disadvantages of a BM aspirate smear

A

May not represent all cells
Dry tap may occur
Doesn’t represent architecture
Inability to analyze stroma

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13
Q

Disadvantages of a BM core biopsy

A

Slow processing
Decalcification required and preludes ancillary studies
Inability to perform quantitative diff count

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14
Q

What is a direct aspirate smear?

A

Made similarly to periph. blood smears (wedge technique)
Bony spicules = good and allows for more cells (do not crush them)

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15
Q

What is a crush smear?

A

Bony spicules placed on slide and crushed by another slide; superior to wedge prep method for morphological exam

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16
Q

What is a buffy coat smear? Why would you use this technique?

A

EDTA aspirate transferred to narrow-bore tube and centrifuged, ME and plasma layers are aspirated and smears are prepared using crush smear technique
- Useful when there is hypocellular marrow

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17
Q

What is a touch prep smear?

A

Made from core biopsy specimen; biopsy is repeatedly touched to a slide and is valuable when the specimen is clotted or there is a dry tap

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18
Q

What is the normal stain to use on a bone marrow specimen?

A

Wright or Wright-Giemsa stains

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19
Q

What is the purpose of the Prussian blue stain for bone marrow specimens?

A

Used to estimate marrow storage iron or iron metabolism abnormalities
Highlights presence of ring sideroblasts

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20
Q

What does MPO stain allow for detection of in BM specimens?

A

Detects myeloid cells

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21
Q

What does SBB stain allow for detection of in BM specimens?

A

Detects myeloid cells

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22
Q

What does PAS stain allow for detection of in BM specimens?

A

Detects lymphocytic cells and certain abnormal erythroid cells

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23
Q

What do esterase stains allow for detection of in BM specimens?

A

Distinguish myeloid from monocytic maturation stages

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24
Q

What does TRAP stain allow for detection of in BM specimens?

A

Hair cell leukemia

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25
Q

What can we perform under low power examination of BM?

A
  • Assess peripheral blood dilution
  • Locate bony spicules, aggregations of bone, and hematopoietic cells
  • Estimate cellularity
  • Search for tumor cells
  • Examine and estimate megakaryocytes
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26
Q

What can we perform under high power examination of BM?

A
  • Observe maturation of nucleated cells
  • Perform a differential
  • Calculate M:E ratio
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27
Q

What is the estimated cellularity of children? 30-70 y/o? How do we estimate cellularity for >70 y/o?

A

Children = 80%
30-70 y/o = 50%
> 70 y/o = subtract patient age from 100% and add +/- 10%
(Ex. 75 y/o = 15-35%

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28
Q

Osteoblast vs osteoclast

A

Osteoblast = bone formation
Osteoclast = bone resorption

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29
Q

What is the purpose of the H&E stain?

A

Used for the examination of core biopsy specimen when aspiration procedure yields a dry tap; assess cellularity but cannot perform a differential

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30
Q

What is the purpose of cytochemical studies on BM?

A

Diagnosis of leukemias/lymphomas

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31
Q

What is the purpose of cytogenetic studies on BM?

A

Diagnosis of acute leukemias via chromosomal abnormalities

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32
Q

What is the purpose of molecular studies on BM?

A

PCR for diagnostic point mutations

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33
Q

What is the purpose of FISH testing on BM?

A

Staining for diagnostic mutations

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34
Q

What is the purpose of flow cytometry testing on BM?

A

Immunophenotyping of malignant hematopoietic cells

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35
Q

What information may be included on a bone marrow examination report?

A
  • Patient medical history and CBC results
  • Diagnostic narrative:
    Summary of the recorded BM findings and additional lab chemical, microbiologic, and immunoassay tests
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36
Q

Each tech counts _____ cells during a BM exam at RH.

A

250

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37
Q

What is meant by the term “bone marrow failure”?

A

Reduction/cessation of blood cell production affecting one or more cell lines (PLTs/WBCs/RBCs)

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38
Q

What is the result of bone marrow failure?

A

Pancytopenia

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39
Q

Name 6 potential causes of bone marrow failure

A
  1. Destruction of HSCs through drugs/chemicals/radiation etc.
  2. Premature senescence and apoptosis of HSCs due to genetic mutations
  3. Ineffective hematopoiesis
  4. Disruption of BM microenvironment
  5. Decreased production of hematopoietic growth factors
  6. Loss of normal
    hematopoietic tissue due to infiltration of marrow space with abnormal cells
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40
Q

What is the major form of BM failure?

A

Aplastic anemia

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41
Q

What are the characteristic features of aplastic anemia?

A

Pancytopenia
Reticulocytopenia
BM hypocellularity
Depletion of HSCs

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42
Q

Name a few associations/causes of aplastic anemia

A

Idiopathic –> unknown
Secondary –> drugs, chemicals, radiation (benzene/cytotoxic drugs)
Viral infections –> EBV, HIV, parvovirus B19

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43
Q

Most cases of aplastic anemia are _________ (85%) and few are ________ (15%).

A

acquired; inherited/congenital

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44
Q

What is the PRIMARY cause of aplastic anemia?

A

Quantitative and qualitative deficiency of HSCs

45
Q

What are some laboratory findings associated with aplastic anemia?

A

Decreased ANC
HGB <10
Decreased retics
Decreased neuts/monos/PLTs
Macrocytic/normocytic RBCs
Toxic granulation
Increased iron and %TSAT
Decreased CD55 and CD59
Decreased CD24 and CD14
Severe hypocellularity in BM

46
Q

What is the most common of the inherited bone marrow failure syndromes?

A

Fanconi anemia

47
Q

What is Fanconi anemia?

A

Inherited chromosome instability disorder characterized by aplastic anemia, physical abnormalities, and cancer susceptibility

48
Q

What population is most affected by Fanconi anemia?

A

Ashkenazi Jewish and South African Afrikaner populations

49
Q

What are the clinical findings of those with Fanconi anemia?

A

Thumb malformations
Cafe-au-lait lesions on skin
Short stature
Increased cancer risk

50
Q

What is the diagnostic test for Fanconi anemia?

A

Chromosomal breakage analysis; FA cells have increased fragility

51
Q

Lab results of those with Fanconi anemia

A

Pancytopenia
Reticulocytopenia
Hypocellular bone marrow

52
Q

What is the prognosis of someone with Fanconi anemia?

A

1/3 develop MDS and/or AML by 14 y/o

1/4 develop solid tumors by 26 y/o

> 90% develop BM failure by 40 y/o

If it goes untreated, death by 20 y/o due to BM failure.

53
Q

Treatment for Fanconi anemia

A

HSCT

54
Q

What are the clinical findings of someone with Dyskeratosis Congenita?

A
  • Mucocutaneous abnormalities (abnormal skin pigmentation, dystrophic nails, oral leukoplakia)
  • Increased cancer risk
  • Multisystem abnormalities
  • BM failure/pancytopenia
55
Q

Genetics and pathophysiology of Dyskeratosis Congenita

A

DC chromosomes have very short telomeres leading to premature cell death and predisposition to cancer

Mutation in one of at least 11 genes, most commonly a mutation of the DKCI gene on the X chromosome

56
Q

How to diagnose/detect Dyskeratosis Congenita?

A

Flow cytometry FISH test for detection of short telomeres

Pancytopenia and macrocytic RBCs

57
Q

Treatment/prognosis of someone with Dyskeratosis Congenita?

A

Median survival = 42 yrs
Androgen therapy treats, but does not halt progression of BM failure

58
Q

What is Shwachman-Bodian-Diamond syndrome (SBDS)?

A

Multisystem disorder characterized by pancreatic insufficiency, cytopenias, skeletal abnormalities, and a predisposition to hematologic malignancies

59
Q

Clinical findings of SBDS?

A
  • Peripheral blood cytopenia
  • Decreased pancreatic enzyme secretion
  • Immune dysfunction
  • Short stature
60
Q

Genetics/pathophysiology of SBDS

A

Biallelic mutations in the SBDS gene

Deficiencies in CD34+ cells

Increased apoptosis in HSCs

Short telomeres in granulocytes

61
Q

Laboratory findings in SBDS

A

Neutropenia
Anemia/thrombocytopenia
Hypocellular bone marrow
Increased fecal fat

62
Q

Treatment for SBDS

A
  • G-CSF for neutropenia
  • Transfusion support for anemia/thrombocytopenia
  • Enzyme replacement for pancreatic insufficiency
  • Stem cell transplant if AML/MDS develops
63
Q

What is PRCA and how does it differ from the other types of BM failure disorders discussed?

A

Rare disorder of erythropoiesis characterized by severe decrease in erythroid precursors ONLY in an otherwise normal bone marrow

**Difference is that this disorder has normal WBC/PLT counts

64
Q

What are the two types of PCRA and describe them

A

Acquired PRCA: aka transient erythroblastopenia of childhood, many patients have a history of viral infection

Congenital PCRA: Diamond-Blackfan Anemia - mutations in the RPS19 gene with craniofacial dysmorphisms, short stature, neck and thumb malformations

65
Q

What are CDAs?

A

Congenital dyserythropoietic anemias characterized by refractory anemia, reticulocytopenia, hypocellular bone marrow with markedly ineffective erythropoiesis and distinctive dysplastic changes in BM erythroblasts

66
Q

Briefly describe CDA I

A

Macrocytic RBCs
Megaloblastoid erythroblasts with internuclear chromatin bridges or nuclear stranding; spongy heterochromatin with a swiss cheese appearance

67
Q

Briefly describe CDA II

A

The most common subtype
Normocytic RBCs
Normoblastic erythroblasts with some binucleated forms

68
Q

Briefly describe CDA III

A

Least common subtype
Macrocytic RBCs
Megaloblastic erythroblsats with up to 12 nuclei

69
Q

Describe myelophthisic anemia

A

Due to infiltration of abnormal cells into the BM and subsequent destruction and replacement of normal hematopoietic cells
- Leukoerythroblastic

70
Q

What is the primary cause of anemia in chronic kidney disease?

A

Inadequate renal production of EPO

71
Q

Define cytogenetics

A

The study of chromosomes, their structure, and their inheritance

72
Q

What is the purpose of cytogenetic testing?

A

To analyze chromosome disorders

73
Q

Why might cytogenetic testing be ordered on a patient?

A

Intellectual disability
Infertility
Ambiguous genitalia
Short stature
Fetal loss
Risk of genetic or chromosomal disease
Cancer

74
Q

What is metaphase and why are metaphase chromosomes important in cytogenetic testing?

A

Metaphase is the stage in mitosis in which chromosomes align on the equatorial plate. This is important because they are highly condensed and have a high mitotic rate

75
Q

Define haploid. What is the haploid number in humans?

A

Haploid represents gametes and have half the diploid number (23 chromosomes in these cells) it is represented as “n”.

76
Q

Define diploid. What is the diploid number in humans?

A

Diploid is the number in dividing somatic cells and is 46 in humans. It is represented as “2n”.

77
Q

Nucleosome

A

DNA looped around a core of histones

78
Q

Solenoid

A

Nucleosomes are coiled into twisted forms

79
Q

Metacentric centromeres

A

Centromere located in the middle of the chromosome

80
Q

Submetacentric centromeres

A

Off centered centromeres

81
Q

Acrocentric centromeres

A

Located at one end of the chromosome

82
Q

Mitogens

A

Stimulate division of specific cell types

83
Q

p arm vs q arm

A

p arm = short arm of chromosome
q arm = long arm of chromosome

84
Q

What is Q banding?

A

Differentiates chromosomes into bands of differing widths and relative brightnesses

Utilizes a dye called quinacrine mustard which binds to A-T rich areas

85
Q

What is G banding?

A

The most common method used for staining chromosomes
Utilizes Giemsa stain, stains A-T rich areas

86
Q

What is C banding?

A

Stains the centromere and the surrounding condensed heterochromatin; Giemsa stain used

87
Q

Karyotype

A

Summary of the chromosome analysis, resulted into a karyogram (picture of all the chromosomes aligned from 1-22)

88
Q

Describe FISH. What information can FISH results provide?

A

A molecular technique used to detect and locate a specific DNA sequence on a chromosome
- Can provide information on structural/numeric abnormalities of chromosomes, Ability to diagnose

89
Q

How would the karyotype nomenclature look like for a patient with a Trisomy of chromosome 8 in a male?

A

47,XY,+8

90
Q

What would the karyotype nomenclature for a normal male and female look like?

A

Male: 46,XY
Female: 46,XX

91
Q

Translocation

A

Designated “t”, gene has moved from one area to another

92
Q

Deletion

A

Designated “del”, gene has been deleted from a chromosome, loss of the segment

93
Q

Aneuploidy

A

ANY abnormal number of chromosomes that is NOT a multiple of the haploid number (23)

94
Q

Trisomy

A

Presence of an extra chromosome

95
Q

Monosomy

A

Absence of a single chromosome

96
Q

Polyploidy

A

Chromosome number higher than 46, but is ALWAYS an exact multiple of the haploid chromosome number (23)
Ex. Triploidy and Tetraploidy

97
Q

Triploidy

A

Karyotype with 69 chromosomes (3n)

98
Q

Tetraploidy

A

Karyotype with 92 chromosomes (4n)

99
Q

Hypodiploid

A

Cell has fewer than 45 chromosomes

100
Q

Hyperdiploid

A

Cell has more than 46 chromosomes

101
Q

High hyperdiploidy

A

Cell has more than 50 chromosomes

102
Q

Inversions

A

One or two breaks in a single chromosome, no loss or gain of material

103
Q

Insertion

A

Movement of a segment of a chromosome from one location to another

104
Q

Duplication

A

Partial trisomy for part of a chromosome

105
Q

What would Down syndrome nomenclature look like for a male?

A

46,XY,21+

106
Q

What is the tissue most frequently used to study the cytogenetics of a hematologic malignancy?

A

Bone marrow

107
Q

What abnormal chromosome is associated with CML and how is it treated? How is the treatment monitored?

A

Philadelphia chromosome
Treated with imatinib mesylate
Pt response is monitored by cytogenetic analysis and FISH

108
Q

What is the amplification of HER2 gene on chromosome 17 associated with?

A

Aggressive form of invasive breast cancer

109
Q

What is a good prognosis for childhood ALL?

A

> 50 chromosomes (high hyperdiploidy)

Hematology (10 decks)

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