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Blood, Lymph, Immune, Bone > Lymphoid Neoplasms I > Flashcards

Lymphoid Neoplasms I Flashcards

1
Q

What is the most common childhood malignancy?

A

ALL

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2
Q

All acute lympoblastic leukemias are (+) for what?

A

All acute lymphoblastic leukemias are (+) for TdT

*myloblastic lymphomas are (-) for TdT

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3
Q

What are signs and symptoms of ALL?

A
  1. abrupt onset
  2. Symptoms related to overcrowding of the BM
    1. fatigue/anemia
    2. fever (neutropenia)
    3. bleeding (thrombocytopenia)
  3. Mass effects
    1. bone pain
    2. generalized lymphadenopathy
    3. hepatosplenomegaly
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4
Q

What characteristics portend a good prognosis for ALL?

a bad prognosis?

A
  1. Good prognosis:
    1. hyperdiploidy
    2. t(12;21)
  2. Bad Prognosis:
    1. hypodiploidy
      1. t(9;22)- philadelphia chromosome. This chromosome is usually found in the adult forms of the disease
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5
Q

What is the most common adult leukemia of the western world?

what is the absolute lymphocyte count to classify it as leukemia?

A

Chronic lymphocytic leukemia/small lymphocytic lymphoma

>5000/microL

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6
Q

CLL/SLL

genetic abnormalities

A

CLL/SLL

deletions: 13q14:3, 11q, 17p and trisomy 12

NOTCH1

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7
Q

CLL/SLL

immunophenotype

A

CLL/SLL

CD5, CD19, CD20, CD23

*CD5 is normally a marker for T-cells, but it appears in leukemic B-cells along with the pan B-cell markers (CD19 and CD20)

CD23 is (+) in CLL/SLL. It is negative in mantle cell lymphoma

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8
Q

CLL/SLL

histology

A

CLL/SLL

condensed chromatin and scant cytoplasm

smudge cells

co-existent autoimmune hemolytic anemia can cause spherocytes

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9
Q

What are we looking at?

A

Lymphnode effacement

and

proliferation centers are pathognomonic for

CLL/SLL

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10
Q

CLL/SLL

good prognosis:

bad prognosis:

A

CLL/SLL

  1. Good prognosis:
    1. 13q14 deletion
    2. trisomy 12q
    3. Somatic hypertation
  2. Bad prognosis
    1. NOTCH1 mutation
    2. Richter syndrome
    3. 11q and 17p deletions
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11
Q

What is Richter Syndrome?

A

Richter syndrome is the transformation of CLL into a diffuse large B-Cell lymphoma

Richter syndrome presents with a rapidly enlarging mass within a lymph node or spleen and is associated with a v bad prognosis (survival <1 year)

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12
Q

what type of lymphoma is associated with

t(14;18)?

A

t(14;18) is associaed with Follicular lymphoma. This translocation involves BCL2 and IGH which leads to an overexpression of BCL2 which decreases apoptosis

Follicular lymphoma is the most common indolent NHL in the US

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13
Q

Follicular lymphoma

immunophenotype

clinical features

A

Follicular lymphoma

  1. immunophenotype
    1. (+) CD19, CD20, CD10, BCL2
    2. (-) CD5 (this distinguishes it from CLL)
  2. Clinical features:
    1. painless, generalized lymphadenopathy
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14
Q

What type of cell do we see at high power in CLL/SLL?

A

Prolymphocytes are a larger cell with a centrally located nucleolus

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15
Q

This type of tumor may involve translocations in BCL6, but is more classically associated with immunodeficiency (adanced HIV or bone marrow transplant) or as a result of EBV infections

A

Diffuse Large B-Cell lymphoma

anaplastic cells will ususally be negative for surface B or T cell markers, but have high IgH rearrangements

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16
Q

DLBCL

clinical features

prognosis

A

DLBCL

  1. Clinical features:
    1. rapidly enlarging mass at nodal or extra nodal sites in the body
      1. Waldeyer ring, liver, spleen
      2. GI, skin, bone, brain
  2. Prognosis:
    1. rapidly fatal without treatment.
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17
Q

Burkitt Lymphoma

Pathogenesis

A

Burkitt Lymphoma

MYC translocations on chromosome 8

t(8;14)

MYC is a transcription regulator that increases gene expression for aerobic glycolysis and allows cells to syntehseize elements for growth and cell division

18
Q

What type of lymphoma is this?

A

The numerous pale tingible body macrophages produce the “starry sky” appearance of Burkitt Lymphoma at low power.

19
Q

Burkitt Lymphoma

what type of viral infection is associated with Burkitt Lymphoma?

A

EBV infections

20
Q

t(11;14) is associated with what type of lymphoma?

A

t(11;14) is associated with

Mantle Cell Lymphoma

the translocation of IgH (ch 14) with cyclin D1 (ch 11) leads to overexpression of cyclin D1 which stimulates the progression of G1 to S phase in the cell cycle

21
Q

Mantle Cell Lymphoma

Immunophenotype

clinical features

A

Mantle Cell Lymphoma

  1. immunophenotype
    1. (+) cyclin D1, CD19, CD20, CD5
    2. (-) CD23
  2. Clinical Features
    1. painless lymphadenopathy
    2. organ dysfunction that leads to death
    3. can’t use conventional chemotherapy
      4.
22
Q

This lymphoma begins as a polyclonal immune response that evolves into a clonal disorder due to mutations that make tumor growth and survival antigen-independent

A

Marginal Zone Lymphomas

t(11;18)

this upregulates MLC10 or MALT1 which activates NF-kB that promotes growth and survival of B cells.

May transform into DLBCL

23
Q

Marginal Zone Lymphomas are often located where?

A

Marginal Zone Lymphomas are often located at the site of chronic inflammatory disorders, both infectious and autoimmune.

ex: stomach dt H. pylori gastritis, salivary gland dt sjogren disease

24
Q

This type of leukemia is associated with point mutations in BRAF

A

Hairy Cell Leukemia

25
Hairy Cell Leukemia Immunophenotype
Hairy Cell Leukemia 1. immunophenotype 1. (+) CD19, CD20, Surface Ig; **CD11c, CD25, CD103, ANXA1**
26
Hairy Cell Leukemia Clinical Features \*will we have lymphadenopathy?
Hairy Cell Leukemia pancytopenia, splenomegaly weakness, fatigue, LUQ pain, fever, bleeding we rarely have lymphadenopathy because the cells are trapped in the bone
27
What type of leukemia causes a "dry tap" and why?
Hairy Cell Leukemia causes a dry tap because the cells are trapped in an extracellular matrix composed of reticulin fibrils and will not be aspirated.
28
Adult T-Cell Leukemia/Lymphoma affects adults that are infected with what virus? the viral gene encodes what protein?
Adult T-Cell Leukemia/Lymphoma **Human T-cell leukemia retrovirus type 1 (HTLV-1)** HTLV1 encodes TAX protein that activates NF-kB
29
These types of neoplasms are most common in the Far East
Peripheral T-Cell and NK-cell neoplasms
30
Adult T-Cell Leukemia/Lymphoma Clinical Presentation
Adult T-Cell Leukemia/Lymphoma 1. skin lesions (T-cell lymphomas like to migrate to the skin) 2. lymphadenopathy 3. hypercalcemia with lytic bone lesions 1. Sometimes the virus will cause a demyelinating disease that affects the CNS and spinal cord 2.
31
These skin lesions are common in what lymphoma?
Mycosis Fungoides this is the most common cutaneous T-cell lymphoma
32
Buzzwords: "cerebriform" cells Pautrier microabscesses
Mycosis Fungoides
33
What is Sezary Syndrome
Sezary Syndrome is a generalized exfoliative erythroderma with a poor prognosis and a median survival of 32 months
34
What is this?
Sezary syndrome with diffuse erythroderma
35
Buzzword: "Brisk neoangiogenesis"
Peripheral T-Cell Lymphoma
36
Anaplastic Large-Cell Lymphoma immunophenotype "buzzwords"
Anaplastic Large-Cell Lymphoma 1. (+) ALK, CD30 1. The ALK gene (2q23) create fusion proteins that constitutively activate tyrosine kinases that trigger the **RAS and JAK/STAT pathways** 2. Buzzwords 1. hallmark cells with **horseshoe-like** or embryoid nuclei
37
Large Granular Lymphocytic Leukemia Immunophenotype (T-cell type, NK-type) mutations
Large Granular Lymphocytic Leukemia 1. immunophenotype 1. T-cell: (+) CD3 2. NK-cell: (+)CD56, (-) CD3 2. mutations in STAT3 leading to activation
38
Felty Syndrome: presentation: associated with what type of leukemia?
RA, neutropenia, splenomegaly Large Granular Lymphocytic Leukemia
39
Buzzword: "nasopharyngeal mass"
Extranodal NK/T-cell lymphoma
40
Extranodal NK/T-cell Lymphoma immunophenotype Clinical
Extranodal NK/T-cell Lymphoma 1. immunophenotype: 1. (-) CD3, CD21, and T-cell receptor rearrangements 2. (+) NK markers 2. Clinically 1. agressive 2. resistant to chemotherapy but responds to radiation therapy

Blood, Lymph, Immune, Bone (13 decks)

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