Lecture 48/49 - Lymphomas and Myelomas Flashcards by Benjamin Bloom

what is a luekemia vs lymphoma vs myeloma? where do they abnormalities present

L eukemia vs Lymphoma vs Myleoma
○ Leukemia – abnormal proliferation of immature lymphoid/myeloid cells (blasts; ALL)
§ Bone marrow

	○ Lymphoma -- Mature B cells growing out of control 
		§ Lymph Nodes 

	○ Myeloma -- plasma cell growing out of control 
		§ Bone marrow

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what are the layers of a normal reactive LN from inner to outer?

Follicle
Mantle Zone
Marginal Zone

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Describe the Ann Arbor Tumor staging for Lymphomas

Stage 1 – Single Location
Stage 2 – Multiple sites; same side of the diaphragm
Stage 1,2 – Localized disease; consider radiation therapy

Stage 3 – both sides of the diaphragm
Stage 4 – diffuse involvement of non lymphoid organs
Wide spread
Consider chemotherapy

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Hodgkin Lymphoma –

what is it?

Localized single group of Nodes; contiguous spread

Extra nodal involvement is rare

Better Prognosis than Non-Hodgkin Lymphoma

Strongly associated with EBV

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What is the classical morphology associated with Hodgkin Lymphoma

Reed Sternberg Cells

+Reactive benign Infiltrate

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What are the 4 classical subtypes of Hodgkin Lymphoma?

What is the immunophenotype in general?

which are associated with EBV

CD15, 30+) and are EBV associated but to varying degrees

Nodular sclerosing (most common)
Rare EBV association

Lymphocyte-rich
(do not confuse with lymphocyte-predominant)
40% EBV+

Mixed cellularity
70% EBV+

Lymphocyte depleted
90% EBV+

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Immunophenotype of nonclassical HL subtype

CD15, CD30 and EBV Negative

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Common Presentation + symptoms of HL

Enlarged LN in the neck, mediastinum, axilla

○ Often positive for EBV
○ 40% have B symptoms: night sweats, fevers, weight loss;
○ Itch
Sensitivity to alcohol

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Treatment of HL:

(ABVD) +/- radiation therapy

□ Adriamycin (Doxorubicin), Bleomycin, Vinblastine, Dacarbazine

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Outcomes/prognosis of HL

○ Limited stage – 85% disease free 10 years later

○ Wide spread - 70% disease free 10 years later

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What are the NHL - B Cell Lymphomas?

Which are aggressive(more treatable)?

Which are indolent (less treatable)?

CLL/SLL

Diffuse Large B Cell Lymphoma — aggressive

Burkitts Lymphoma —- (some are EBV ASSOCIATED) — aggressive

Follicular Lymphoma – indolent

Marginal Zone – indolent
(H. Pylori associated)

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NHL B Cell: DLBCL: 
- what is it/presentation 
- what are the causes? -
- 
-

Fast growing, large Bcell tumor mass; Disease may extend beyond LNs

Causes not well understood. Very Heterogeneous Origins

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what are the virally associated subtypes of DLBCL?

- what is are some possible genetic makers (translocation?

Immunodeficiency associated LBCL (eg such as with acquired immunodef or HIV)

Primary Effusion Lymphoma
HHV8 (Kaposis’ Sarcoma) Associated

Potential translocations: BCL6
t(14:18)

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Treatment of DLBCL

+ what drug is used for CNS prophylaxis

treatment: RCHOP
Rituximab (anti CD20); Cyclophosphamide, Doxorubicin, Vincristine, Prednisone +/- Radiation

CNS prophylaxis: Methotrexate

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NHL B Cell: Burkitt’s Lymphoma

what is it/presentation?

an aggressive B cell tumor: extremely fast growing. Medical emergency

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NHL B Cell: Burkitt’s Lymphoma

what causes it?
- is there a viral association?

cMYC Translocations with an Ig – proto-oncogene that gets activated when attached to an Ig Loci and becomes constitutively activated

t(8:14) translocation

Viral association for some of
subtypes:
- African – all EBV associated
Sporadic and HIV – few are EBV associated

What is the morphology of Burkitts?

“starry sky” – Sheets of lymphocytes with interspersed macrophages

High apoptotic rate: Macrophages in the background full of dead lymphoma cells

NHL - B Cell – Follicular Lymphoma

what is it?

an indolent, slow growing B cell Lymphoma

Presentation: stage 3,4 disease; usually does not grow out of the Lymph Nodes

Can gradually transform to DLBCL

NHL - B Cell – Follicular Lymphoma

what is the genetic origin?
how can this help with diagnosis (immunophenotype)

T(14:18): Translocations of BCL2 with IgH

Immunophenotype:
Test: Over expression of BCL2
A normal follicular center is BCL2 negative

NHL - B Cell – Follicular Lymphoma

what is the standard of care?
when do you treat?

§ Current standard of Care:
□ Advanced Disease – Close surveillnace; treatment is not worth it

Treatment when the patient is symptomatic due to the lymphoma (eg the mass is compressing an important nerve or organ)

Rituximab + Chemo
Rituximab + Targetted therapy

NHL - B Cell – Marginal Zone Lymphoma (MALToma)

what is it?
What bacteria is it associated with?
treatment

Indolent B cell Lymphoma that involves the mucosa;
— no enlarged LNs

– (H. Pylori)

– Treatment: if in the setting of H. pylor
® Triple therapy – 2 abx + PPI; followed by Surveillance Endoscopy

Name of the Mature T Or NK-Cell Lymphomas:

Adult T Cell Lymphoma:

Anaplastic Large Cell Lymphoma (ALCL)

Extranodal NK/T Cell Lymphoma

Mycosis Fungoides/Sezary Syndrome:

Peripheral T Cell Lymphoma – Not Otherwise Specified

Adult T Cell Lymphoma: What is the viral association?

Human T Lymphotrophic Virus 1: HTLV 1

○ Anaplastic Large Cell Lymphoma (ALCL)

what is the Constitutively Activated protein

ALK

Extranodal NK/T Cell Lymphoma

where does it commonly occur? What structures are invaded?
what is the viral association?

§ Very destructive tumor often involving the nasopharynx

§ Invades into blood vessels —> ischemic necrosis

§ Strongly associated with EBV

Mycosis Fungoides vs Sezary Syndrome:

what is it? what is the difference between the two?
Treatment for the two:

Mycosis Fungoides: Malignant T Helper cells infiltrate the skin, eventually form discrete plaques and tumors; can progress to Sezary — treat topically

Sezary Syndrome: Typically associated with a leukemia
treat with chemotherapy

Describe the progression of Plasma Cell Neoplasms

what is the common finding of all of these ?

1) MGUS: Monoclonal Gammopathy of Uncertain Significance
2) Smoldering Myeloma
3) Plasma Cell Myeloma – Plasmacytoma
4) Multiple Myeloma: Multiple Plasmacytomas

Common finding: Elevated M (monoclonal para) protein

1) MGUS: Monoclonal Gammopathy of Uncertain Significance –

Presentation
what % become multiple myeloma
Clinical Findings:
what is notably absent

No masses; Asymptomatic

§ 1% will develop multiple myeloma

Clinical Findings: M protein
no CRAB findings

Smoldering Myeloma:

presentation
clinical findings

§ No Masses
§ Asymptomatic

M protein elevated
Elevated Marrow Plasma Cells (>10%)

Plasma Cell Myeloma – Plasmacytoma

§ a single Marrow based Tumors

§ Increased marrow plasma cells

• Elevated monoclonal Component

§ Solitary marrow mass; (if multiple, then its called Multiple Myeloma)

Mutlitple Myeloma:
- what is it?

how do patients commonly present?

proliferation of plasma cells (in the marrow at multiple sites) – fully developed B cell that makes Ig

§ Excess Monoclonal Ig — M Protein;

§ Patient often presents with bony pain; new renal failure; new anemia

What are the criteria for diagnosis of a Multiple Myeloma?

§ Dx – Bone marrow aspirate and biopsy

Criteria for Multiple Myeloma Diagnosis:

> 10% plasma cells in the bone marrow

An M protein (monoclonal protein, Ig) – -quantitative Ig Levels; serum, urine and electrophoresis

End organ Damage: CRAB findings

What are the CRAB findings?

What symptoms can they cause?
what causes the renal damage?

◊ Calcium: hypercalcemia — can lead to dehydration and mental status changes (confusion)
◊ Renal failure — some of the M proteins can damage the kidneys

◊ Anemia

◊ Bone Lesions – skeletal survey — get every part of the body X-ray
Findings: Punched out lytic bone lesions;

How is serum protein electrophoresis used in Multiple Myeloma?

Used to classify the type of Ig that being abnormally produced (IgG, IgA, or IgM ) and whether it is kappa or lambda light chain

treatment of multiple myeloma?

treatment:
§ Multi-agent

Bisphosphonate therapy to stabilize bones

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Lecture 48/49 - Lymphomas and Myelomas Flashcards

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