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Goljan > Chapter 13 - WBC Disorders > Flashcards

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
Q

Basophilia. Present in…

A

MPDs. Characteristic for CML.

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26
Q

Which leukemias can transform into AML?

A

MDSs, CML

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27
Q

Which MPDs can present with Portal HTN?

A
  1. PV

2. MMM

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28
Q

Clinical findings in MMM

A
  1. Massive Splenomegaly -> maybe: Portal HTN

2. Splenic infarcts -> maybe: left-sided pleural effusions

29
Q

How is LAP (leukocyte alkaline phosphatase) in

  1. PV
  2. CML
  3. MMM ?
A
  1. PV: increased
  2. CML: decreased
  3. MMM: normal to increased
30
Q

How are platelets in Iron Def Anemia?

A

Increased

31
Q

In ET, which is more common: bleeding or thrombosis?

A

Bleeding

32
Q

Major rick factors (2) for MDSs?

A
  1. Alkylating agents

2. Radiotherapy

33
Q

DDx: Sideroblasts in BM.

A
  1. Sideroblastic Anemia

2. MDSs

34
Q

3 causes of death in MDSs

A
  1. Infection
  2. Bleeding
  3. Progression to AML
35
Q

Which leukemia has the worst prognosis?

A

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
Q

Alkylating agents: risk factor for which leukemias?

A

ALL, AML, CLL

37
Q

In which chromosomal abnormalities can we see AML?

A

Down s.
Klinefelter s.
Turner s.

38
Q

Person with Down s. >5y. Risk for which leukemia?

A

ALL

39
Q

Cytogenetic abnormalities in AML (2).

A
  1. Mutations on chromosome 8

2. t(8;21)

40
Q

If a patient with CML progresses to AML, will we see Auer Rods in his WBCs?

A

NO.

41
Q

Auer-Rods are present in which types of AML?

A

M2,M3.

Rarely seen in M1, M4.

42
Q

t(15;17) is seen in which leukemia?

A

APL (M3 AML)

This translocation causes abnormal retinoic acid metabolism.

43
Q

Which leukemia has the worst prognosis?

A

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
Q

Which is the MC type of AML (30-40% of cases)?

A

M2: AML with maturation

45
Q

Person with Down s.

A

AML (M7: Acute megakaryocytic)

46
Q

Person with Down s. >5y. Risk for which leukemia?

A

ALL

47
Q

TdT+ is a marker for …

A

All types of ALL.

48
Q

t(9;22) is seen in…

A

Adult ALL more common. Related to poor prognosis.

49
Q

Risk factors for ALL. (6)

A
  1. Antineoplastic agents
  2. Hodgkin lymphoma
  3. Ionizing radiation
  4. Benzene exposure
  5. MM
  6. Down s.
50
Q

t(12;21) is seen in …

A

Children’s ALL more often. Favorable prognosis

51
Q

CD10+ (CALLA), TdT+ : diagnosis

A

Pre-B-cell ALL

52
Q

CD10-, TdT+, CD7+ : diagnosis

A

T-cell ALL

53
Q

Sites of metastasis of B-cell ALLs

A
  1. CNS

2. Testicles

54
Q

Presentation of T-cell ALL

A

Usually: Mediastinal (thymic) mass -> called Acute Lymphoblstic Lympoma in a Teenager.

55
Q

WBC disorder associated with HTLV-1

A

ATLL

56
Q

In CLL, naive B cells co-express CD.. and CD..

A

CD5 and CD20

57
Q

In ATLL and Mycosis Fungoides, there is neoplastic proliferation of mature CD.. T cells

A

CD4 (helper cells)

58
Q

TAX gene is activated in …

A

ATLL. It inhibits p53 suppressor gene.

59
Q

A person has HSM, hypercalcemia, lytic bone lesions, and a rash. He is (-) for TdT. What WBC disorder has he?

A

ATLL. (DDx from MM: he has a rash!)

60
Q

Where do we see lymphocytes with cerebri-form nuclei in a blood smear?

A

These cells are called Sezary cells.

We see them in Sezary s.

61
Q

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?

A

The epidermis.

They can form rash, plaques or nodules.

62
Q

Which is the MC leukemia overall?

A

CLL

63
Q

Where do I see smudge cells?

A

CLL

64
Q

Where do I see CD5+,CD20+ B cells?

A

CLL

65
Q

What is Richter transformation?

A

CLL cells can pick up additional mutations and CLL transforms into DLBCL. This is noted clinically by an enlarging lymph node or spleen.

66
Q

Which WBC disorder can result in Autoimmune vasculitis and arthritis?

A

HCL

67
Q

In which WBC disorder lymphadenopathy in uncommon?

A

HCL

68
Q

SLL may arise from …

A

CLL

Goljan (10 decks)

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