Chapter 13 - WBC Disorders Flashcards

1
Q

EBV in IM primarily infects 3 organs.

GIMME THOSE FUCKING ORGANS

A
  1. Oropharynx
  2. Liver
  3. B cells
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2
Q

EBV in IM spreads to B cells in lymph nodes. The virus attaches to CD.. receptors on B cells.

A

CD21

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3
Q

3 drugs causing neutrophilic leukocytosis (decreased adhesion)

A
  1. Corticosteroids
  2. Catecholamines
  3. Lithium
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4
Q

Classic Triad in Acute Leukemia

A
  1. Fever (infection due to neutropenia)
  2. Bleeding (thrombocytopenia)
  3. Fatigue (anemia)
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5
Q

In which type of leukemia can I see thrombocytosis in 35% of cases, unlike other leukemias?

A

CML.

Other leukemias having thrombosis:

  • PV
  • ET
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6
Q

2 situations that I have ectopic production pf EPO and so inappropriate absolute polycythemia

A
  1. Renal cell CA (MCC)

2. HCC

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7
Q

Which is the risk factor for developing CML?

A

Exposure to ionizing radiation

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8
Q

MCC of massive splenomegaly in people >50y

A

MMM

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9
Q

Which myeloproliferative disorder presents with portal HTN and left-sided pleural effusions?

A

MMM.
PHTN-> due to massive splenomegaly
Left sided pleural effusions-> due to splenic infarcts

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10
Q

T-ALL presents as … (2)

A
  1. Anterior mediastinal mass
  2. ALL
    (in a teenager)
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11
Q

B-ALL commonly metastasizes to .. (2)

A
  1. CNS

2. Tests

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12
Q

Which is the MC leukemia in Western countries?

A

CLL

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13
Q

Which is the MCC of generalized lymphadenopathy in people >65y?

A

CLL

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14
Q

Which leukemia increases the risk for IHA?

A

CLL

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15
Q

Leukemias (both acute and chronic) more common in males or females?

A

Males

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16
Q

Translocation in CML?

A

t(9;22) : BCR-ABL fusion protein-> increased TK activity.
Phil chromosome: chr. 22 with the mutation
BCR: chr. 22
ABL: chr. 9, proto-oncogene

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17
Q

Headache: common in which leukemia?

A

ALL (CNS involvement)

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18
Q

Which leukemias can transform into AML?

A

MDSs, CML

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19
Q

JAK2 gene is on which chromosome and affects which WBC disorders?

A

9p

PV, ET, MMM

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20
Q

MCC of Budd-Chiari syndrome?

A

PV

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21
Q

Which leukemia can have PUD as a complication?

A

PV. Increase in mast cells-> increase in Histamine-> increase in Gastrin release

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22
Q

Exact mutation in PV.

A

JAK2 V617F mutation in exon 14.

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23
Q

All myeloproliferative disorders present with enlarged spleen. Which one of those has enlarged liver too?

A

PV.

CML-> 8% of cases

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24
Q

Translocation in CML?

A

t(9;22) : BCR-ABL fusion protein-> increased TK activity.
Phil chromosome.
BCR: chr. 22
ABL: chr. 9, proto-oncogene

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25
Basophilia. Present in...
MPDs. Characteristic for CML.
26
Which leukemias can transform into AML?
MDSs, CML
27
Which MPDs can present with Portal HTN?
1. PV | 2. MMM
28
Clinical findings in MMM
1. Massive Splenomegaly -> maybe: Portal HTN | 2. Splenic infarcts -> maybe: left-sided pleural effusions
29
How is LAP (leukocyte alkaline phosphatase) in 1. PV 2. CML 3. MMM ?
1. PV: increased 2. CML: decreased 3. MMM: normal to increased
30
How are platelets in Iron Def Anemia?
Increased
31
In ET, which is more common: bleeding or thrombosis?
Bleeding
32
Major rick factors (2) for MDSs?
1. Alkylating agents | 2. Radiotherapy
33
DDx: Sideroblasts in BM.
1. Sideroblastic Anemia | 2. MDSs
34
3 causes of death in MDSs
1. Infection 2. Bleeding 3. Progression to AML
35
Which leukemia has the worst prognosis?
AML. (5y-survival rate=20%) [CLL: 5y-survival rate=75% CML: 5y-survival rate=90% ALL: best prognosis of all, those with t(12;21). 75-85% are cured, 95%: complete remission]
36
Alkylating agents: risk factor for which leukemias?
ALL, AML, CLL
37
In which chromosomal abnormalities can we see AML?
Down s. Klinefelter s. Turner s.
38
Person with Down s. >5y. Risk for which leukemia?
ALL
39
Cytogenetic abnormalities in AML (2).
1. Mutations on chromosome 8 | 2. t(8;21)
40
If a patient with CML progresses to AML, will we see Auer Rods in his WBCs?
NO.
41
Auer-Rods are present in which types of AML?
M2,M3. | Rarely seen in M1, M4.
42
t(15;17) is seen in which leukemia?
APL (M3 AML) This translocation causes abnormal retinoic acid metabolism.
43
Which leukemia has the worst prognosis?
AML. (5y-survival rate=20%) [CLL: 5y-survival rate=75% CML: 5y-survival rate=90% ALL: best prognosis of all. 75-85% are cured, 95%: complete remission]
44
Which is the MC type of AML (30-40% of cases)?
M2: AML with maturation
45
Person with Down s.
AML (M7: Acute megakaryocytic)
46
Person with Down s. >5y. Risk for which leukemia?
ALL
47
TdT+ is a marker for ...
All types of ALL.
48
t(9;22) is seen in...
Adult ALL more common. Related to poor prognosis.
49
Risk factors for ALL. (6)
1. Antineoplastic agents 2. Hodgkin lymphoma 3. Ionizing radiation 4. Benzene exposure 5. MM 6. Down s.
50
t(12;21) is seen in ...
Children's ALL more often. Favorable prognosis
51
CD10+ (CALLA), TdT+ : diagnosis
Pre-B-cell ALL
52
CD10-, TdT+, CD7+ : diagnosis
T-cell ALL
53
Sites of metastasis of B-cell ALLs
1. CNS | 2. Testicles
54
Presentation of T-cell ALL
Usually: Mediastinal (thymic) mass -> called Acute Lymphoblstic Lympoma in a Teenager.
55
WBC disorder associated with HTLV-1
ATLL
56
In CLL, naive B cells co-express CD.. and CD..
CD5 and CD20
57
In ATLL and Mycosis Fungoides, there is neoplastic proliferation of mature CD.. T cells
CD4 (helper cells)
58
TAX gene is activated in ...
ATLL. It inhibits p53 suppressor gene.
59
A person has HSM, hypercalcemia, lytic bone lesions, and a rash. He is (-) for TdT. What WBC disorder has he?
ATLL. (DDx from MM: he has a rash!)
60
Where do we see lymphocytes with cerebri-form nuclei in a blood smear?
These cells are called Sezary cells. | We see them in Sezary s.
61
In Mycosis Fungoides, there is neoplastic proliferation of CD4+ T cells. The aggregates of these neoplastic cells are called Pautrier microabscesses. Which is the site of these microabscesses?
The epidermis. They can form rash, plaques or nodules.
62
Which is the MC leukemia overall?
CLL
63
Where do I see smudge cells?
CLL
64
Where do I see CD5+,CD20+ B cells?
CLL
65
What is Richter transformation?
CLL cells can pick up additional mutations and CLL transforms into DLBCL. This is noted clinically by an enlarging lymph node or spleen.
66
Which WBC disorder can result in Autoimmune vasculitis and arthritis?
HCL
67
In which WBC disorder lymphadenopathy in uncommon?
HCL
68
SLL may arise from ...
CLL