Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathology II (Kiva) > Topic 24-26: lymphoid neoplasia part 1 > Flashcards

Topic 24-26: lymphoid neoplasia part 1 Flashcards

1
Q

what is leukemia, lymphoma and plasma cell dyscrasia? generally

A

Leukemia: tumors that primarily involve the bone marrow and the peripheral blood.

Lymphoma: tumors that produce masses in involved lymph nodes or other tissues.

Plasma cell dyscrasia: tumors of the plasma cells which are usually present as discrete masses in the bones and causes systemic symptoms related to the production of a complete or partial monoclonal immunoglobulins.

In some cases, lymphomas or plasma cell tumors spill over to the peripheral blood, creating a leukemia-like picture. Conversely, leukemias can infiltrate lymph node, creating a histological picture of lymphoma. It’s important to know what kind of tumor cell it is, not location!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What must be done to classify the neoplasia?

A

-immunohistochemistry and flow cytometry to see markers and lineage specific antigens

this is important because lymphoid neoplasms are all monoclonal (originate from single mutated cell)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

There are 4 ways to technically characterize leukemia of lymphoid origin (a lot of overlap with lymphomas)

A
  • Hodkins vs Non-hodkins lymphoma
  • Kiel classification: Low grade vs. High grade lymphoma
  • characterization based on stage that differentiation block occurs
  • WHO lymphoid leukemia classification
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is the characterization based on maturation stage?

A

having a block in a certain stage and accumulate in the previous stage

  • Block in the bone marrow=pre germinal center tumor: acute lymphoblastic leukemia (ALL)
  • Block in the germinal center: follicular lymphoma (t(14:18), Burkitt lymphoma t(8:14)
  • Post Germinal center tumors: multiple myeloma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

the majority of lymphoma are blocked at what stage?

A

The majority of the lymphomas are originated in the germinal center stage (or even post GC stage)

This is due to the somatic hypermutation and the immunoglobulin class switching, which generate genetic instability and place the B cells at relatively high risk for mutations that can lead to transformation.
The genetic alterations are not enough in order to develop lymphoma, but other alterations should be present.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is the treatment for B cell lymphoma?

A

MabtherA

merged Fab (mouse) and Fc (human) that is anti-cd20 to specifically target the B cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

According the the WHO lymphoma classification, how is it roughly divided?

A

B or T/Nk cell vs progenitor or mature stage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the progenitor B cell lymphoid neoplasia? specific markers? How common is it?

A

-B cell acute lymphoblastic leukemia (B-ALL) /lymphoblastic lymphoma (LB)

  • CD19… CD10, CD34,
  • TdT positive B cells

-85% of childhood acute leukemias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the progenitor T/NK cell lymphoid neoplasia? specific markers? How common is it?

A

-T cell acute lymphoblastic leukemia (T-ALL)/lymphoblastic lymphoma (LB)

  • CD2, CD3, variable marker expression!
  • TdT positive T-cells

-15% of child leukemia and 45% of child lymphomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the mature B cell lymphoid neoplasias?

A
  • small lymphocytic lymphoma/chronic lymphocytic lymphoma (SLL/CLL)
  • FL (Follicular lymphoma )
  • DLBCL (Diffused large B cell lymphoma)
  • Burkitt lymphoma
  • Plasma cell myeloma
  • Hairy cell leukemia
  • Mantle cell lymphoma
  • MALT lymphoma

Walt has diffusely large hair follicles that chronically ooze plasma so he wears a small man burka

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what are the mature T/NK cell lymphoid neoplasia?

A
  • Anaplastic large cell lymphoma
  • Peripheral T cell lymphoma
  • Mycosis Fungoides

Ana has a large, peripheral fungal infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

When do the Precursor T- and B-cell lymphoblastic leukemia/lymphoma typically occur?

A

the 1st is at the age of 4 (ALL constitutes 80% of childhood leukemias) and the other is at the age of 70 years old.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the mutations associated with B-ALL? which have good vs. bad prognosis?

A

all are transcription factors:
Bad prognosis
- t(9;22)-BCR/ ABL - philadelphia chromosome
- t(v;11q23) - MLL rearrangement (from 11 to 11 (23h) we rewatch major league lacrosse)

Better prognosis

  • t(1;19)-E2A/PBX1 (even 2day anna watches PBX=public broadcasted porn? from 1 am to 7 (19h))
  • t(12;21)-TEL1/AML1 - tell amelie
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the difference histologically between ALL and AML

A

ALL

  • often has cytoplasmic glycogen granules –> PAS positive
  • TdT positve
  • CD 19 in B cells
  • CD 3 in T cells

AML is peroxidase positive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is the most common adult leukemia?

A

chronic lymphocytic leukemia CLL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is the difference between CLL and SLL

A

basically the same, CLL is when lymphocytes are above 4000 per microliter

Small lymphocytic leukemia is less common, only about 4% of cases

17
Q

CLL/SLL have what genetic problem?

A
  • high levels of BCL2 (inhibits apoptosis). Not rearranged like in Follicular lymphoma!!
  • 50% of the patients have trisomy 12 and deletions of chromosomes 11 and 13
18
Q

what histological sign is common in CLL/SLL?

A

-smudge cells because they are fragile cells

  • In lymph nodes The follicular structure of the lymph node is not preserved!
  • there are foci of larger, mitotically active cells. These foci are called proliferation centers.
19
Q

what are some CLL/SLL surface markers?

A

CD 19, CD20 and 23

20
Q

what are the two clinical outlooks of CLL/SLL?

A

Non progressive: associated with post germinal center mutations

Progressive: associated with pregerminal center mutations. CLL/SLL tends to transform to more aggressive tumors.

21
Q

hair cell leukemia is a subtype of which mature B cell lymphoma?

A

extranodal marginal zone lymphoma

22
Q

extranodal marginal zone lymphoma arises where and under what conditions will it perhaps occur?

A

arrises in epithelial tissues of GI, lung and breast under conditions of chronic infection (h. pylori) or autoimmune diseases

23
Q

extranodal marginal zone lymphoma can have what genetic factors?

A

t(11,18) MALT1/IAP2 (from age 11 to 18, walt and I Ate Pizza) –> suggests a therapy resistant gastric tumor

24
Q

hairy cell leukemia has which surface markers and genetic mutations? what is the overall prognosis?

A

CD 19, 20

  • Almost all have mutations in the serine threonine kinase BRAF
  • good prognosis
25
Q

Myelomas are what and what do they produce that causes problems?

A

-plasma cell tumors that create single complete or partial Igs that are not functional. These Igs are called M proteins

26
Q

there are 6 types of myelomas

A
  1. Multiple myeloma
  2. solitary plasmacytoma
  3. lymphoplasmacytic lymphoma (think LPC)
  4. heavy-chain disease
  5. primary amyloidosis
  6. MGUS (monoclonal gammopathy of undetermined significance)

MaGNUS and Amy have multiple heavy soliloquies for the LPC (liberal party of canada)

27
Q

myelomas are associated with which mutations?

A

-translocation of IgH (chrom 14) to fuse with:

  • FGFR3
  • Cyclin D1 and D3 dysregulation

-myc (later in mutation)

28
Q

Multiple myeloma has which symptoms?

A
  • bone pain
  • hypercalcemia
  • anemia
  • recurrent infections
  • renal dysfunction (due to infections and bence-jones proteins, amyloidosis from M proteins, and hypercalcemia)
  • AL-amyloidosis
  • hyperviscosisty
29
Q

lymphoplasmacytic lymphoma is associated with what problem?

A

-hyper-IgM secretion leading to waldenstrom macroglobulinemia (inc blood viscosity)

  • visual and neurological problems
  • bleeding
  • raynaud phenomenon
30
Q

what are the most common M proteins in multiple myeloma?

A
  • In 60% of the cases → IgG
  • In 20-25% of the cases → IgA
  • in 15-20% of the cases → only ƙ or ƛ light chains. In that case, and due to their low molecular weight, the light chains are excreted in the urine where they are called “Bence-Jones” proteins.

in some cases, both light chains and complete immunoglobulin molecules are produced → M component + Bence Jones protein.

31
Q

solitary plasmacytoma are what?

A

plasma cell tumor from skeletal or soft tissue origin.

can progress to multiple myeloma over 5-10 years

32
Q

lymphoplasmacytic lymphoma (LPC) is what?

A

An incurable b cell tumor (mis of lymphocytes, plasma cells) that secretes IgM,

causes waldenstrom macroglobulinemia too, but no bence-jones proteins!

no osteolysis or amyloidosis

33
Q

heavy chain disease is what?

A

-when only heavy chains are produced by a abnormal B cell, typically IgA

34
Q

MGUS is what?

A
  • precursor lesion to multiple myeloma

- an asymptomatic monoclonal gammapathy

Pathology II (Kiva) (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions