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Neville and Damm > Chapter 13- Hematologic disorders (neoplastic) > Flashcards

Chapter 13- Hematologic disorders (neoplastic) Flashcards

1
Q

What disease? Most people with _______ have too many red blood cells. But it can also cause you to have too many white blood cells and platelets.

A

polycythemia vera

*too many cells, but the cell are normal otherwise

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2
Q

Age group affected by polycythemia vera

A

~ 60 year old, no sex predilection

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3
Q

An acquired mutation of _____ is present in ___% of patients with polycythemia vera

A

JAK2 mutation

>95%

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4
Q

40% of patients have generalized pruritis w/o rash in what condition?

A

polycythemia vera

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5
Q

What is the term for a painful burning sensation, erythema, and warmth of hands and feet….Can lead to gangrene…occurs in polycythemia vera

A

Erythromelalgia

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6
Q

Patients with polycythemia vera experience _____ hemorrhage, _____ thrombus formation

A

increased hemorrhage

increased thrombus formation

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7
Q

Treatment for polycythemia vera

A
  • phlebotomy of 500ml every other day until hematocrit is 45%
  • aspirin
  • megakaryocyte inhibitor
  • hydroxyurea: myelosuppressive agent
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8
Q

___ to ___% of patients with polycythemia vera develop acute leukemia

A

2-10%

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9
Q

____% of all cancers in the US are leukemia and ____% of all cancer deaths

A
  1. 9

4. 1

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10
Q

Genetic disorders predisposed to leukemia (8)

A
  1. Down syndrome
  2. Bloom syndrome: causes problems with DNA repair, resulting in a high number of chromosome breaks and rearrangements.
  3. NF1
  4. Schwachman syndrome: malabsorption, pancreatic insufficiency, impaired bone marrow fn, skeletal abnormalities
  5. Ataxia-telangiectasia syndrome
  6. Klinefelter syndrome
  7. Fanconi anemia
  8. Wiskott-Aldrich syndrome: X-linked recessive disease characterized by eczema, thrombocytopenia (low platelet count), immune deficiency, and bloody diarrhea
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11
Q

genetic mutation in CML(chronic myeloid leukemia)

A

philadelphia chromosome
translocation 9;22
BCR-ABL fusion oncogene

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12
Q

precursor disease to acute myeloid leukemia

A

myelodysplasia syndromes

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13
Q

virus associated with t-cell leukemia/lymphoma

A

HTLV-1

human t cell leukemia/lymphoma virus 1

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14
Q

Prevalence of all leukemia

A

13:100,000

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15
Q

T/F Males are affected more than female with leukemia

A

true

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16
Q

AML:
Age group
median survival

A

broad age range including children

  • 40% 5 yr survival <60 yr old
  • 10% >60
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17
Q

CML:
Age group
median survival

A

3rd-4th decades

-80% 5 year survival, TKIs and BM transplant

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18
Q

CML: indolent period followed by blast transformation which results in death in ____

A

3-6 months

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19
Q

ALL:
Age group
median survival

A

Acute LymphoBlastic Leukemia
predominantly children
90% curable in children
worse prognosis in adults

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20
Q

CLL:
Age group
median survival

A

Chronic LymphoCytic Leukemia (MOST COMMON TYPE OF LEUKEMIA)
older adults
variable course 2-10+ year survival

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21
Q

crowding out of normal blood cells in the marrow by malignant or other proliferation

A

Myelophthisic anemia

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22
Q

Term for tumor like growths of leukemic cells

A

myeloid sarcoma

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23
Q

another name for a leukemic infiltrate in soft tissue. Called this because the tissue looks green.

A

chloroma

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24
Q

_______ is high doses of chemo agents to try to induce remission…What % of CML patients will be resistant to imatinib?

A

induction chemotherapy….33%

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25
Q

6 other names for Langerhans cell histiocytosis

A

histiocytosis X; langerhans cell disease; idiopathic histiocytosis; eosinophilic granuloma; langerhan cell granuloma; langerhan cell granulomatosis

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26
Q

Mutation in langerhan cell histiocytosis

A

BRAF in 40-60%

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27
Q

incidence of langerhan cell histiocytosis per year

A

1-4:1 million

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28
Q

Majority of cases of Langerhans cell histiocytosis occur in patients _____

A

younger than 15

>50%

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29
Q

Jaw is affected in ____% of cases of Langerhans cell histiocytosis

A

10-20

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30
Q

_______ is most affected site in oral LCH and lesions have a _______ appearance…WHAT DOES THE CEPH RADIOGRAPH OF A YOUNG LCH PT RESEMBLE?

A 
posterior mandible
scooped out
**no corticated border
"teeth floating in air"
CEPH resembles multiple myeloma with punched out lesions on the radiograph
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31
Q

What are the 3 general/separate/unique clinical presentations of LCH?

A
  1. monostotic or polyostotic eosinophilic granuloma of bone
  2. Chronic disseminated histiocytosis (hand-schuller-christian disease)
  3. Acute disseminated histiocytes (letterer sewe disease)
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32
Q

Monostotic or polyostotic eosinophilic granuloma of bone is characterized by

A

1 or more bone lesions of LCH, no visceral involvement

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33
Q

Chronic disseminated histiocytosis: What is the HSC triad? What is the alternate name?

A

Bone lesions, skin, viscera HSC TRIAD (only present in few patients): 1. bone lesions, 2. exopthalmus, 3. diabetes insipidus…HAND-SCHULLER-CHRISTIAN disease

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34
Q

3 tissues affected in acute disseminated histiocytosis (Letterer-siwe disease- not really used)…what age group?

A

Cutaneous, visceral and bone marrow involvement in INFANTS

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35
Q

LCH is classified into ____ organ involvement or ______

A

single organ involvement

Multi-organ involvement

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36
Q

Multi-organ involvement LCH is separated into _____ dysfunction and no ____ dysfunction.

A

organ dysfunction

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37
Q

LCH with organ dysfunction is separated into low risk and high risk organ involvement
Low risk organs=
High risk organs=

A

low risk- skin, bone, LNs pituitary gland

High risk- lung liver spleen BM

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38
Q

Distinctive feature of a langerhan cell on electron microscopy? Distinctive radiographic appearance of oral cavity lesions?

A

Birbeck granules- rod shaped cytoplasmic structures….”teeth floating in air” on radiograph

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39
Q

2 IHC stains for langerhan cells…2 common histo features? (only one is in neville)

A

CD1a CD207(Langerin)….lots of EOs and “coffee bean” nuclei

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40
Q

Hodgkin lymphoma: Neoplastic cells( _____cells) only account for ____% of the cells in enlarged lymph nodes

A

reed-sternberg cells

0.1-2%

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41
Q

Hodgkin lymphoma is _____ as common as non-hodgkin lymphoma…what virus is found in a significant number of HL cases?

A

1/6th…EBVx`

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42
Q

Most common first site for Hodgkin lymphoma

A

Cervical supraclavicular nodes 70-75%

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43
Q

Hodgkin lymphoma presents in axillary and mediastinal LNs in ____%, and abdominal and inguinal nodes in _____%

A

5-10% each

<5%

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44
Q

Hodgkin lymphoma demographics

A

Male predilection

Bimodal age: 15-35; >50

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45
Q

Hodgkin lymphoma:

Category A vs. Category B

A

Category A= no systemic signs

Category B= systemic symptoms (weight loss, sweating, pruritis)

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46
Q

What is the classification system for Hodgkin Lymphoma called

A

Ann Arbor system

stages 1-4

47
Q

What are the 2 main histological types of Hodgkin lymphoma

A
  1. Nodular lymphocyte predominant

2. Classical

48
Q

What are the subtypes(5) of classical hodgkin lymphoma

A
  1. lymphocyte rich
  2. nodular sclerosis
  3. mixed cellularity
  4. lymphocyte depleted
  5. Unclassifiable
49
Q

What histologic subtype of Hodgkins lymphoma supposedly is the most aggressive (even though the book says in the prognosis section that histo subtype doesn’t matter)

A

Lymphocyte depleted

50
Q

What histologic subtype of classical Hodgkin Lymphoma is the most common

A

Nodular sclerosis 60-80%

51
Q

What are Reed-Sternberg cells called in nodular sclerosis type classical hodgkin lymphoma

A

lacunar cells

52
Q

If Reed-Sternberg cells are binucleated the term _____ is used. If they are multinucleated the term _____ is used.

A

owl-eyes

pennies on a plate

53
Q

Lymphoid cells in nodular-predominant hodgkin lymphoma are called _____

A

popcorn cells

**these are mimicked in infectious processes like mono

54
Q

____% of hodgkin lymphomas are nodular lymphocyte-predominant histologic subtype

A

4-5%

55
Q

Current classification system for Non-hodgkin lymphoma

A

Modified REAL/WHO classification

56
Q

____ Hodgkin cases/year

_____Non-hodgkin cases/year

A

9000

70,000

57
Q

Increased prevalence of Non-hodgkin lymphoma in what immunologic conditions? (6)

A
  • Bloom syndrome
  • Wiskott-Aldrich syndrome
  • Common variable immunodeficiency
  • AIDS
  • Organ transplant
  • Autoimmune disease: SLE, Sjogrens, RA
58
Q

HHV8 is implicated in kaposi sarcoma but also in _________

A

primary body cavity lymphoma and some plasmablastic lymphoma

59
Q

4 Viruses associated with lymphoma

A
  1. HHV8(primary body cavity/plasmablastic)
  2. HTLV-1 (peripheral t cell)
  3. EBV(Burkitt, some Hodgkin, NK-T cell lymphoma)
  4. HIV
60
Q

Non-hodgkin lymphoma: _____% present extranodal in the US, ~___% outside the US

A

20-40%

50%

61
Q

What term is used for leukemic and lymphoma infiltration in the soft tissue of the oral cavity

A

boggy

62
Q

Most common oral cavity sites for non-hodgkin lymphoma:

A

buccal vestibule, posterior hard palate, gingiva

63
Q

Numb chin syndrome:

A

bony involvement of lymphoma in the mandible causing paresthesia

64
Q

most common lymphoma presenting in the oral cavity

A

DLBCL (60%)

65
Q

Another name for cutaneous T cell lymphoma

A

Mycosis fungiodes

66
Q

Most common cutaneous lymphoma

A

mycosis fungoides

67
Q

Prevalence of mycosis fungoides

A

1200/year

68
Q

Mycosis fungoides demonstrates ________, a propensity to invade the epidermis of the skin

A

epidermotropism

69
Q

Demographics of MF

A

M>F 2:1
middle aged
A.A. 1.5x more

70
Q

3 stages of MF

A
  1. eczematous (erythematous) stage
  2. plaque stage
  3. tumor stage
71
Q

Sezary Syndrome

A

aggressive form of mycosis fungoides

->separated from MF by the presence of cancerous lymphocytes in the blood

72
Q

Mycosis cells aka Sezary cells have a ______ nucleus

A

cerebriform

73
Q

Mycosis cells form aggregates called _____

A

Pautrier microabscesses

74
Q

T cells of MF stain with

A

CD4- among other things but MF is a CD4 (t helper cell) derived lymphoma

75
Q

Clinical classifications of Burkitt Lymphoma (3)

A
  • endemic BL
  • Sporadic or American BL
  • Immunodeficiency-associated BL
76
Q

In endemic BL more than ____% of tumor cells are positive for EBV.

A

90%

77
Q

What is a predictor for the development of malignancy in patients with endemic type BL

A

levels of plasmodium falciparum (malarial organism). Higher levels= more likely to develop malignancy

78
Q

% of BL cases that occur in the jaws

A

50-70%

79
Q

Demographics associated with endemic type BL

A

children
central africa
male
maxilla>mandible (posterior)

80
Q

Jaw involvement by endemic BL is age dependent: the older the patient the _____ likely in the jaw

A

less likely

90% of 3 year olds, 25% of >15 year olds

81
Q

Most common clinical presentation for Sporadic/American BL

A

abdominal mass

82
Q

EBV positivity is _______ frequent in Sporadic BL compared to endemic

A

less

83
Q

patchy loss of lamina dura may be an early sign of?

A

Burkitt lymphoma

84
Q

Classic histo appearance of Burkitt lymphoma

A

starry sky= histiocytes on a background of malignant dark blue lymphocytes

85
Q

Most common translocation in Burkitt lymphoma

A

8;14 (q24;q32)

over expression of oncogene c-myc

86
Q

Midline lethal granuloma is a non-specific name for multiple entities including ____

A

extranodal NK/T cell lymphoma, nasal type

-also any condition destroying the midface: infectious, wegners

87
Q

According to the WHO what marker must be positive to call extranodal NK/T cell lymphoma, nasal type

A

EBV positivity

88
Q

This was originally considered an angiocentric T cell lymphoma and is now recognized as an EBV+ B cell process with a marked T cell response

A

Lymphomatoid granulomatosis

89
Q

what disease: Swelling of the palate → deep necrotic ulceration usually at midline.
Creation of an oronasal fistula
Secondary infection may be life threatening

A

Extranodal NK/T cell lymphoma, nasal type

90
Q

T/F Females are more affected by Extranodal NK/Tcell lymphoma

A

false- males

91
Q

Histo findings for EN NK/T cell lymphoma…which 3 stains can help distinguish?

A

angiocentric (mixed inflammatory cells around blood vessels, necrosis, large angular atypical lymphocytes)…CD56 (NKcell marker), CD3 (T cell marker), EBER (EBV-encoded RNA)

92
Q

Best treatment for NK/T cell lymphoma, nasal type that is localized

A

Radiation 40-50Gy

similar to other T cell lymphomas

93
Q

Disseminated disease is present in __% NK/T cell lymphoma, nasal type

A

30%

94
Q

Multiple myeloma represents ___% of all malignancies

A

1%

95
Q

What % of hematologic malignancies are multiple myeloma

A

10-15%

96
Q

Demographics for Multiple myeloma

A

M>F, adults 60-70

2x more common in blacks

97
Q

Most common hematologic malignancy in blacks

A

multiple myeloma

98
Q

Characteristic presenting sign in MM

A
  • bone pain frequently lumbar

- can also be renal failure from deposition of faulty light chains

99
Q

Myelophthisic anemia

A

displacement of hemopoietic bone-marrow tissue by fibrosis, tumors, or granulomas

100
Q

Characteristic finding in urine of 30-50% of MM patients

A

Bence-Jones proteins

101
Q

Amyloid deposition occurs in ___% of MM patients

A

10-15%

intraoral: tongue

102
Q

The jaw is involved in ___% of cases of multiple myeloma and can be seen radiographically as _____

A

30%

punched-out lesions

103
Q

Protein immunoelectrophoresis in multiple myeloma should show _______

A

monoclonal gammopathy

104
Q

What genetic finding is associated with more aggressive behavior in MM

A

hyperdiploid tumors

105
Q

What are the 3 risk categories for multiple myeloma patients and what is the survival estimate for each

A

Standard risk: 6-7yrs
Intermediate
High-risk: 2-3yrs

106
Q

What term is used for a plasmacytoma occurring outside of the bone

A

extramedullary plasmacytoma

107
Q

% of extramedullary plasmacytomas ocuring in the head and neck

A

25%

108
Q

____% of patients with a plasmacytoma show monoclonal gammopathy on evaluation of serum protein immunoelectrophoresis

A

25-50%

109
Q

What two IHC markers are decreased in plasmacytoma in comparison to multiple myeloma lesions

A

cyclin D1

CD56

110
Q

T/F radiation therapy is most common for plasmacytoma

A

true

111
Q

What % of patients develop multiple myeloma after diagnosis of plasmacytoma

A

50% in 2-3 years

112
Q

_____ prognosis for extramedullary plasmacytoma

compared to lesions of bone

A

Better

  • Less than 30% progress to MM
  • 70% disease free for 10 years after treatment
113
Q

Most common type of leukemia?

A

Chronic lymphocytic leukemia

Neville and Damm (72 decks)

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