Lymphoma Flashcards

1
Q

What does normal B cell differentiation look like?

A
  • Lymphoblasts –> naive B cells –> transform in germinal centers when exposed to mitogen (antigen selection and somatic hypermutation) –> memory type B cells, plasma cells, marginal zone cells
  • Some memory type B cells and marginal zone B cells skip germinal center so non-mutated
  • Resting B cells can transform when exposed to mitogen or antigen –> become larger w/ nucleoli and more abundant cytoplasm
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Normal Lymph Node Architecture

A
  • Follicles have central, pale pink germinal center w/ mantle zones along outside (darker) and may have marginal zone outside of that
  • Should see capsule (not too thick) and sinusoids
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Categorization of Lymphomas

A
  • Hodgkin - Reed-Sternberg cells
    • Nodular Lymphocyte Predominant (NLPHL)
    • Classical
      • Nodular sclerosis
      • Lymphocyte rich (many small lymphocytes)
      • Mixed cellularity (mixed inflammatory cells)
      • Lymphocyte depleted (many RS cells or fibrosis instead)
  • Non-Hodgkin -
    • Divided by cell type and maturity level
    • Can also be divided based on behavior
      • Indolent NHL (slow growing but poor response)
        • Follicular
        • Small lymphocytic
        • Marginal zone
        • Mycosis fungoides
      • Aggressive NHL (grow faster, respond to chemo)
        • Diffuse large B cell
        • Mantle cell
        • Anaplastic large cell
      • Highly aggressive NHL (very fast growing, may spread to CNS, respond very well to chemo)
        • Burkitts
        • Precursor B or T cell
        • Adult T cell
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Risk Factors for Lymphoma (5)

A
  • Immune def (AIDS, CVID, SCID, autoimmune states)
  • Infectious agents (EBV, HH8, HTLV-1)
  • Herbicide exposure
  • Chemicals (agent orange)
  • Prior radiation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Lymphoma Presentation

A
  • Poor appetite / wt loss
  • Lymphadenopathy / splenomegaly
  • “B symptoms” (constitutional) - fever, chills, sweats
  • Fatigue
  • Night sweats
  • Pruritus esp after alcohol use in HL
  • Skin rash
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Lymphoma Labs

A

elevated blood counts, hepatic/ renal dysfunction, inc LDH and uric acid (esp if aggressive - cell turnover), abnormal cells on peripheral blood flow cytometry

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

Tumor Lysis Syndrome

A
  • sudden breakdown cells –> inc uric acid, hyperkalemia, hyperphos, uric acid crystals in urine, renal failure
  • Give allopurinol, IVF, alkalinization
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Lymphoma Staging + Modifiers

A
  • I - one group nodes
  • II- multiple groups nodes but same side of diaphragm
  • III- both sides of diaphragm
  • IV - spread diffusely w/ extra-lymph sites (BM, lungs, liver)
  • **Modifier - letters
    • A = absent constitutional symptoms
    • B = fever, chills, sweats
    • E = extranodal (from node to adjacent tissues)
    • X = bulky disease (large deposit > 10 cm or 1/3 mediastinum)
    • S = spread to spleen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

IPI Score

A
  • 1 pt for ea
    • Age > 60
    • Stage III or IV
    • Elevated LDH
    • Poor performance status
    • > 1 extranodal sites
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Hodgkin’s Lymphoma

A
  • Only 1% all lymphomas
  • Bimodal - peaks in 20s/30s and in >50
  • Associated w/ EBV
  • Hallmark = Reed Sternberg cells (large, bi-nucleate w/ prominent nucleoli that looks like owl’s eyes) surrounded by reactive, non-neoplastic inflammatory cells (lymphocytes, plasma cells, eosinophils, histiocytes, neutrophils)
  • Types
    • Lymphocyte predominant - indolent, CD20+
    • Classical
      • Nodular sclerosing
      • Mixed cellularity
      • Lymphocyte depleted
      • Lymphocyte rich
  • 95% cured
  • Tx
    • Radiation
    • Chemo - ABVD, MOPP, BEACOPP
    • Combo
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Follicular Lymphoma

A
  • 40% of lymphomas
  • Indolent; usually in elderly
  • From B cells in follicles of nodes
  • Genetics - translocation of Bcl2 gene (anti-apoptosis) now next to heavy chain promoter t(14;18) so now very high levels Bcl2
  • 80% 5 yr survival
  • Can transform to high grade lymphoma
  • Tx - rituximab w/ chemo (COMBO)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Small Cell Lymphoma/ Chronic Lymphocytic Lymphoma (SCL/CLL)

A
  • Extremely common in elderly
  • Accumulation of monoclonal lymphocytes in blood
  • Usually asymptomatic and notice lymphocytosis on CBC
  • Rai Staging
    - Low - lymphocytosis
    - Intermediate - Lymphadenopathy, hepatomegaly, splenomegaly
    - High - anemia, thrombocytopenia
  • Complications
    - Early hypogammaglobinemia - infections
    - Autoimmune hemolytic anemia
    - Richter’s Transformation - indolent –> high grade w/ sudden lymphadenopathy, new B symptoms and BM involvement
  • Tx - rituximab, chemo +/- radiation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Marginal Zone Lymphoma

A
  • Indolent
  • Classified by area of involvement
    • Nodal marginal zone, extranodal marginal zone, splenic marginal zone (associated w/ Hep C)
  • Associated w/ chronic infection or inflammation
    • Gastric MALT - H pylori
    • Ocular-adnexal MALT - Chlamydia psittaci
    • SI MALT - C jejuni
    • Cutaneous MALT - Borrelia burgdorferi
  • Tx - abx/ treat underlying infection, occasionally rituximab w/ chemo
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Mycosis fungoides v. Sezary Syndrome

A
  • MF - form of cutaneous T cell lymphomas w/ predominantly skin involvement
    - Hallmark = Pautrier’s microabscesses
  • Sezary - lymphadenopathy, splenomegaly and peripheral blood involvement (beyond skin)
    - Skin biopsy show sezary cells (med to large lymphocytes w/ cerebriform nuclei and scant cytoplasm)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Diffuse Large B Cell Lymphoma

A
  • Most common NHL
  • Aggressive w/ risk CNS spread
  • Present w/ rapidly enlarging tumor at nodal or extra-
    nodal site
  • Genetics - 30% have abnormal 3q27 involving Bcl-6 gene
  • Excellent outcome and response to chemo
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Mantle Cell Lymphoma

A
  • Age 50-70 (males > females)
  • From cells in mantle zone surrounding germinal centers in lymph node
  • Genetics - t(11;14) translocates oncogene cyclin D1 (promotes progress thru G1-S rapidly - division)
  • 5 yr survival is 50%
  • Tx - rituximab and chemo; may require high dose chemo w/ stem cell rescue
17
Q

Burkitt’s Lymphoma (including 3 types)

A
  • Highly aggressive, rapid spread, can involve CNS
  • 3 Types
    • 1- Endemic Type - associated w/ EBV, commonly affects jaw and spread rapidly (African population; kids < 10)
    • 2- Sporadic Type - young healthy adults in US; lymphadenopathy and splenomegaly; less association w/ EBV
    • 3- Immunodef Associated - HIV pts or those receiving immunosuppressants or EBV; poor prognosis
  • Biopsy - numerous macrophages w/ ingested apoptotic tumor cells (starry sky pattern)
  • Genetics - associated w/ C-Myc on chromosome 8
  • Tx - intensive chemo and CHS prophylaxis
    • Can cause tumor lysis syndrome