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GSSE Pathology > Pathology - WBCs > Flashcards

Pathology - WBCs Flashcards

0
Q

Characteristic features of Haematopoetic Stem Cells include:

A

Pluripotency

Capacity for self-renewal

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1
Q

Besides congenital immunodeficiency diseases, lymphopenia is associated with which diseases/states?

A 
HIV
Viral infections
Glucocorticoid or cytotoxic therapy
Autoimmune disorders
Malnutrition
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2
Q

What is the most common cause of agranulocytosis?

A

Drug toxicity.

Alkylating agents and anti metabolites are often at fault.

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3
Q

Monocytosis is associated with which conditions?

A

Chronic infections; TB, SBE, Malaria

Collagen vascular diseases; SLE

IBD

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4
Q

Eosinophilia is associated with which conditions?

A

Allergies, parasitic infections, drug reactions, lymphomas, vasculitides.

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5
Q

Follicular hyperplasia is characterised by what histological appearance?

A

Prominent large germinal centres (secondary follicles) surrounded by a rim of resting naive B cells (the mantle zone)

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6
Q

Where do B cells reside in lymph nodes?

A

B-cells aggregate in follicles in the outer cortex.

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7
Q

What are primary and secondary follicles?

What is a germinal centre?

A

In unstimulated lymph nodes, B-cells form primary follicles, composed of follicular dendritic cells.

After antigenic challenge they form secondary follicles and express IgG and IgM.

A germinal centre is the pale staining centre of a secondary follicles which contains proliferating B cells and follicular dentritic cells.

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8
Q

What is the importance of the germinal centre in a lymph node?

A

Germinal centres are greatly enlarged in secondary antibody responses and are regarded as important sites of B-cell maturation and generation of B-cell memory.

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9
Q

What causes follicular hyperplasia?

A

Inflammatory processes that activate B-cells:

RA
Toxoplasmosis
Early HIV infection

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10
Q

What is the mantle zone?

A

Simply a rim of resting, naive, B-cells

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11
Q

Acute viral infections cause what type of lymph node reaction?

A

Paracortical hyperplasia:

Large T cell partially efface B-cell follicles.

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12
Q

What are the 3 broad categories of white cell neoplasm?

A

Lymphoid: B, T, NK cells neoplasms

Myeloid: AMLs, Myelodysplasias, chronic myeloproliferative disorders.

Histiocytoses: Macrophage or dendritic neoplasms.

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13
Q

Which 3 viruses are associated with white cell malignancies?

A

Human T Cell Leukaemia Virus 1:
Associated with adult T cell leukaemia

Ebstein Barr Virus:
Subset of Burkitt’s. 30-40% of HL. B-cell lymphomas and NK lymphomas.

Human Herpes Virus-8:
Large B-cell lymphomas.

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14
Q

H. pylori is associated with what malignancy?

Coeliac’s is associated with what malignancy?

A

Gastric B-cell lymphoma.

Intestinal T-cell lymphoma.

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15
Q

How can we distinguish lymphoid reactivity versus malignancy?

A

Reactive lymphocytosis is polyclonal, lymphoid neoplasms are monoclonal.

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16
Q

ALL are neoplasms of what cell type?

A

Immature precursor B (85%) or precursor T lymphoblasts.

17
Q

Pre-T ALL tends to occur in which population?

A

Adolescent boys as thymic lymphoma (50-70% of cases)

18
Q

Are single gene mutations sufficient to cause ALL?

Explain.

A

No. Additional, complementary mutations, which typically increase survival or proliferation are necessary to convert a pre-leukaemic clone to full blown malignancy.

19
Q

Peak incidence for B cell ALL?

Peak incidence for T cell ALL?

A

3 years

Adolescence

Both occur less frequently in adults.

20
Q

Pathognomonic features of CLL/SLL?

A

Proliferation centres (clusters of mitotically active cells).

Smudge cells are often present but not pathognomonic.

21
Q

Prognosis of ALL?

A

With aggressive chemo: 95% of children achieve remission, 75-85% are cured.

Age less than 2 or greater than 10 or t9:22 chromosome are poor prognostic features.

22
Q

Prognosis of CLL?

A

Variable: median survival is 4-6 years. May be up to 10.

With transformation: 1 year.

23
Q

What is Richter syndrome?

A

Transformation of CLL to diffuse Large B-cell Lymphoma. Occurs in 5-10% of CLL cf prolymphocytic transformation in 15-30%. Bad news.

24
Q

Centrocytes and centroblasts are features of which type of lymphoma?

A

Follicular lymphoma.

25
Q

Which 2 chromosomal rearrangements are common in DLBCL?

A
  1. 30% have translocations involving the BCL6 locus, a growth regulator and silencer of p53.
  2. 1-20% have t(14:18) which leads to over expression of anti-apoptotic BCL2.
26
Q

Despite being histologically identical, what are the 3 types of Burkitt’s lymphoma?

A

African (endemic)
Sporadic
HIV associated

27
Q

With which WBC neoplasm is “starry sky” appearance associated with?

A

Burkitt’s lymphoma.

Apoptotic cells ingested by scattered macrophages produce this appearance.

28
Q

Burkitt’s lymphoma is associated with which translocation?

A

c-MYC (chromosome 8) translocation.

Increases c-MYC expression, may suppress p53

29
Q

What are Bence Jones Proteins?

A

Free L chains (small enough) arising from neoplastic cells that end up in the urine.

30
Q

As opposed to NHL ( which occurs in extra nodal sites and spreads unpredictably), Hodgkin’s lymphoma…

A

Arises in a single node or chain and spreads in a predictable way to anatomically contiguous lymphoid tissue.

31
Q

HL is characterised by what histological feature?

A

Reed-Sternberg cells in a background of reactive, non-neoplastic inflammation.

(RS cells can occur with EBV, NHL)

32
Q

RS cells of HL fail to express what?

A

Most B-cell specific genes, including Ig

33
Q

What are Auer rods?

A

Red-staining, per oxidase positive, needle-like structures present on myeloblasts.

34
Q

What is the most common pathogenetic feature of myeloproliferative disorders?

A

Mutated, constitutively active tyrosine kinases.

35
Q

CML is distinguished from other MPDs by what?

A

The presence of a chimeric, constitutively active BCR-ABL tyrosine kinase.

36
Q

CML is associated with which chromosomal abnormality?

A

In more than 90% of CML, the BCR-ABL fusion gene is generated t(9;22).

Termed the Philadelphia chromosome.

37
Q

CML is curable with?

CML is treatable with?

A

Allogenic BMT during the stable period.

Imatinib sustains haematolic remissions in 90% of patients.

38
Q

What mutation underlies PCV and ET?

A

Activating mutations in the JAK2 tyrosine kinase pathway.

39
Q

What is the most common cause of death in multiple myeloma?

A

Infection by pyogenic organisms.

GSSE Pathology (17 decks)

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