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Clin Med- Heme/Oncol > Myelomas & Lymphomas > Flashcards

Myelomas & Lymphomas Flashcards

1
Q

What are 6 lymphoid organs?

A 
Bone marrow
Thymus
Lymph nodes
Spleen
Tonsils, adenoids
Digestive tracts
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2
Q

Where is the origin of MOST lymphomas?

A

Most Lymphomas are of B Cell Origin. B Stands for Bowel or Bursa. B Cells are the Immunoglobulin Antibody Producing Cells.

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3
Q

What are the 2 classes of lymphomas?

A

Non-Hodgkin’s Lymphoma (NHL)

Hodgkin’s Disease (H.D.)

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4
Q

which lymphoma is composed of larger cells?

which of smaller cells?

A

The More Aggressive Non-Hodgkin’s Lymphomas are Generally Composed of Larger Cells. BIG-BAD-CELLS. (Curable)
The Less Aggressive Non-Hodgkin’s Lymphomas are Generally Composed of Smaller Cells. (non curable)

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5
Q

All Lymphomas are Characterized by

A

Lymphadenopathy and the Histology of the Lymph Node is the Most Fundamental Way the Different Lymphomas are Characterized

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6
Q

Lymphadenopathy in NHL spreads how?

A

Spreads Non-Contiguously from Peripheral Lymph Node Groups Inward Toward the Central Lymph Node Groups.

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7
Q

Lymphadenopathy in H.D. spreads how?

A

Contiguous from Central Lymph Node Groups Outward

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8
Q

In H.D. what are the majority of the cells?

A

Minority of the Cells in the Diagnostic Lymph Node are Malignant, the Reed-Sternberg Cell. The Majority of the cells in the Lymph Node that are associated with the Reed-Sternberg Cell are Inflammatory & Benign

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9
Q

In the NHL what are the majority of the cells?

A

Most of the Cells in the Diagnostic Lymph Node are Malignant

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10
Q

Chronic Lymphocytic Leukemia; Small Lymphocytic Lymphoma Epidemiology

A

This is the most common adult leukemia, and the 3rd most common lymphoma
This is a disease of older age, median age 72 y.o.
CLL & SLL are identical and differ only in the method by which they are initially diagnosed
CLL & SLL have a Strong Familial Tendency

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11
Q

2 MC symptoms of Chronic Lymphocytic Leukemia; Small Lymphocytic Lymphoma

A

Fatigue and Lymphadenopathy.

If the Patient with CLL has a Fever, Assume Infection.

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12
Q

3 Lab finding for Chronic Lymphocytic Leukemia (CLL); Small Lymphocytic Lymphoma (SLL)

A

Lab findings besides Lymphocytosis include Anemia and Thrombocytopenia. Most Patients have decreased IgG

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13
Q

Staging, The Rai System for CLL

A

0 Lymphocytosis only No Lymphadenopathy; > 12.5 y
1 Lymphocytosis & Lymphadenopathy; 8.5 y
2 Lymphocytosis & Splenomegaly
+/- Lymphadenopathy; 6 y
3 Lymphocytosis and Anemia d/t CLL; 1.5 y
4 Lymphocytosis and Thrombocytopenia

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14
Q

Treatment of CLL/SLL

A

Not Curable with Chemotherapy
Rx Rai Stage 0 or 1: Observation
Rx Rai Stage 2,3 or 4, or Patients with Sx of Weight Loss etc.
purine analogs**
Fludarabine + Rituximab (Anti-CD20 Ab) May  Survival
Bendamustine (Bi-functional Alkylating Agent) is effective in Relapse

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15
Q

Hairy Cell Leukemia (HCL)

A

Patients Present with Sx d/t/Cytopenias including: Fever, Bleeding, Fatigue and SOB
Splenomegaly is Common
Lymphadenopathy is NOT Common
The Classic Cell is a Small Lymphocyte with Hairy Projections
Hairy Cells are + for CD 19 & 20, as well as CD 11c & CD 103.
Purine analogues e.g. Cladribine

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16
Q

Splenic Marginal Zone Lymphoma/Leukemia

A

Has Splenomegaly and Lymphocytosis like HCL without Lymphadenopathy
Has a Clinical Course like CLL & is Not Curable with Purine Analogues

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17
Q

Marginal Zone Lymphomas (MZL) and the Mucosal-Associated Lymphoid Tissue Lymphomas (MALT Lymphomas)

A

These are Low Grade Lymphomas usually of Lymphoid Tissue associated with Exocrine Glandular Tissue
Gastric MALTs are Frequently Caused by Chronic Ag Stimulation d/t H. pylori infections.
Ocular adnexal MALTs are Likewise associated with Infections, in this case the chlamydial infection, Psittacosis

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18
Q

Marginal Zone Lymphomas (MZL) and the Mucosal-Associated Lymphoid Tissue Lymphomas (MALT Lymphomas) Treatments*

A

Treatment of the Infections that Cause Chronic Ag Stimulation can Cure Some of these Lymphomas at an Early Stage
More Advanced Stages can be Rx’d with Chemotherapy as would be Used in CLL or other Low Grade Malignancies

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19
Q

The Follicular Lymphomas (FL)

A

FL is the 2nd most Common of the Adult Lymphomas, ~ 30%.

They are usually Considered Low Grade

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20
Q

Staging is Based on the Follicular Lymphoma International Prognostic Index (FLIPI)

A 
One Point for Each Characteristic:
Age > 60 y.o.
increased LDH
Ann Arbor Stage III or IV
Hgb < 12
Extra-nodal Disease
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21
Q

Follicular Lymphomas: Characterized by?

A

Positive B Cell Markers

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22
Q

Follicular lymphoma treatment?

A

Treatment is Reserved for Patients with Sx d/t their Lymphoma.
If the Disease is Localized (Ann Arbor Stage 1 or 2), Radiation May be Very Effective
If the Disease is More Disseminated= Chemotherapy, usually Prescribed with an Alkylating Agent and an Anti-CD 20 Ab

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23
Q

Diffuse Large B Cell Lymphoma (DLBCL)

A

Most Common of the Adult Lymphomas ~35%

An Aggressive Lymphoma that Frequently Involves Extra-nodal Sites e.g. the GI Track

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24
Q

International Prognostic Index for Aggressive High Grade Lymphomas

A 
1 Point for Each:
Age > 60
increased LDH
PS 2-4
Extra-nodal Sites
Ann Arbor Stage 3 or 4
25
Q

DLBCL Treatment

A

Ann Arbor Stage 1 & 2 Disease with a Low IPI Score can be Treated with Rituximab, Cyclophosphamide, Hydroxy-daunorubicin (Adriamycin), Oncovin and Prednisone
Likelihood of Cure in this Group is > 80%
In More Advanced Disease, CR can be Expected with R-CHOP
Relapsed Disease is Rx’d with Autologous HSCT; few are cured in this group

26
Q

Burkitt’s Lymphoma

A

This is a Highly Aggressive Lymphoma.
It is endemic in Sub-sahara Africa, where it Presents Most Typically with a Jaw Mass.
All these cases are associated with EBV Infection
In the United States, It largely presents as an Intra-abdominal Mass

27
Q

Burkitt’s Lymphoma Treatment

A

Burkitt’s is treated with High Dose Chemotherapy

28
Q

Mantle Cell Lymphoma

A

Often Present with Bowel Obstruction or Intra-abdominal Masses
Their growth rate is more like DLBCL, but these Lymphomas are Not Curable with Current Therapy
They do Respond to a variety of Chemotherapeutic Agents
The Median Survival is 3.5 – 5 years

29
Q

T Cell Lymphomas

A

rare
Tendency to Involve the Skin and Subcutaneous Tissue
Small Cell Varieties are Indolent and Not Curable

30
Q

Peripheral T Cell Lymphoma Not Otherwise Specified (PTCL NOS)

A

Most Common of the T Cell Lymphomas
Tend to Present with Nodal and Skin Involvement; Stage IV.
Cells are of Intermediate Size and Lymph Nodes may have a Mixture of Small and Larger Malignant Cells Admixed with Inflammatory Cells e.g. Polys and Eos

31
Q

Peripheral T Cell Lymphoma Not Otherwise Specified (PTCL NOS) Treatment

A

Treatment is Generally with CHOP but is Unsatisfactory with a 56% CR

32
Q

Anaplastic Large Cell Lymphoma Alk +

A

Usually a Disease of Individuals < 40 y o
Nodal and frequently Advance with “B” Sx
Responds to CHOP with a 75% 5 y DFS

33
Q

Anaplastic Large Cell Lymphoma Alk negative

A

Disease of the Elderly with Skin Only Involvement is Usually an Indolent Disease, but Incurable
Alk Negative Nodal disease is Poorly Responsive to Chemotherapy (CHOP) and has a Poor Prognosis

34
Q

Nasal NK (Natural Killer Cells) T Cell Lymphoma

A

Usually Presents as an Ulcerative Lesion of the Nasal Septum or Sinuses.
More Common in Individuals of Asian Extraction and Associated with EBV
Usually Treated with a Combination of Chemotherapy and Radiation Therapy (RT)

35
Q

Hodgkins Lymphoma Hodgkins Disease (HD)

A

Presents with Central Lymphadenopathy and Frequently with B Symptoms (Fever, Wt. Loss, Sweats +/- Puritis)
Spreads from one Nodal Group to Adjacent Nodal Groups.

36
Q

Classic Hodgkins Lymphoma Hodgkins Disease (HD)

A

The Malignant Cell is the Hodgkin Reed Sternberg Cell (HRS)
These Cells have a Multi-lobed nucleus with an “Owl-eyed” Appearance.
They are CD 30 + and have Immunoglobulin Gene Rearrangement. They are B Cells

37
Q

What are the Four Subtypes of Classical HD

A

Lymphocyte Rich
Nodular-Sclerosis
Mix Cellularity
Lymphocyte Depleted

38
Q

Lymphocyte Rich

A

Mostly a Disease of Mediastinal LNs in Older Males

Relatively Good Prognosis

39
Q

Nodular-Sclerosis

A

Most Common Sub-type
Usually Involves the Mediastinum in Young Adults
Histologically there are Broad Bands of Fibrosis Creating Pseudo-nodules
The Classic HRS Cell in NS HL is surrounded by a clear zone: A Lacunar Cell

40
Q

Mix Cellularity

A

Tend to Present as Stage III or IV Disease with B Sx in Older Men
The LN is Diffusely Involved

41
Q

Lymphocyte Depleted

A

The Most Aggressive Variety of HL
Tends to Present at an Advanced Stage with “B” Sx
More Common in Older Men and HIV+ Individuals
HRS Cells are Numerous in the LN and Inflammatory Cells Scant

42
Q

Nodular Lymphocyte Predominant HD

A

a Disease Distinct from Classical HD
An Indolent Lymphoma Usually of Low Stage and Without “B” Symptoms
The Malignant Cell is the Large “Popcorn Cell”
NLP HL is Characterized by Long Remissions BUT Frequent Relapses; Survival is Excellent

43
Q

Tests done for patients with Hodgkin Lymphoma

A

Routine Lab Tests (Anemia, Leukocytosis, Lymphopenia, and Hypo-Albuminemia are all Bad Signs) including ESR
CT Scans of Chest, Abdomen and Pelvis
Baseline PET-CT
Bilateral Bone Marrow Biopsies if “B” Sx or Stage 3 or 4

44
Q

What are 4 special tests that might be needed in patients with lymphoma?

A

Fertility Testing & Counseling with Sperm Banking or Embryo Freezing
Pulmonary Function Tests if Bleomycin is to Be Used
Cardiac Evaluation with Cardiac Ultrasound if Adriamycin is to be Used to Check Baseline E.F.
HIV, Hepatitis B &C Testing & PPD as indicated

45
Q

Therapy of Classic H.D. Based on Stage

A

Classic Chemotherapy for HL is ABVD: Adriamycin, Bleomycin, Vinblastine and DTIC,
ABVD is given every 2 weeks intravenously

46
Q

Multiple Myeloma the Immunoglobulin is

A

Usually IgG

47
Q

Waldenstrom’s Macroglobulinemia the Immunoglobulin is?

A

is IgM

48
Q

Multiple Myeloma (MM) and Waldenstrom’s Macroglobulinemia are both diseases of?

A

Mature Functioning B Cells, therefore they are characterized by the Production and Over Production of Immunoglobulins

49
Q

Multiple Myeloma (MM)

A

Median Age at Presentation is 65 y o
MM is twice as Frequent in African-Americans as in Caucasians
The Disease is Incurable at this time, but Remission Duration is Improving

50
Q

What is Multiple Myeloma (MM) defined by? (CRAB)

A 
Hyper-Calcemia
Renal Failure
Anemia
Lytic Bone Lesions
Also:
> 10% Monoclonal Plasma Cells in the Bone Marrow
Serum Monoclonal IgG or IgA > 3g/dL
51
Q

Monoclonal Gammopathy of Unclear Significance: MGUS

A

Monoclonal Protein < 3 g/dL
< 10% Plasma Cells in B.M.
No End Organ Damage

52
Q

Presenting symptoms of multiple myeloma

A 
Fatigue
SOB
Bone Pain, Particularly Back Pain
Pathological Fracture
Infections Particularly Pneumonias
Hypercalcemia: Lethargy, Polydipsia & Polyuria
53
Q

diagnosis of multiple myelomas

A

Evidence of End Organ Damage
Anemia
Monoclonal increased IgG or IgA with Compensatory decreased Other Normal Igs
Increase in Urinary Light Chains
Bone Marrow Aspirate or Bx: > 10% Plasma Cells

54
Q

Testing Required if MM is Suspected

A

CBC
CMP (Chem 20) & Uric Acid
Serum Protein Electrophoresis (SPEP) to look for a Monoclonal Protein in the Gamma Region
Serum Free Light Chains
Bone Marrow Aspirate &/or Biopsy including Karyotyping
Plane Films of Skull and any Painful Bones
MRI or Non-contrast CT Scan of Painful Bones Depending on Adequate Renal Function

55
Q

Treatment of Multiple Myeloma (MM)

A

First Make Sure that any End Organ Damage is Minimized
If the BUN & Cr are increased this needs urgent attention.
NO Radiographic Contrast
Rx increased Ca++ &/or increased Uric Acid
Transfuse Packed Red Blood Cells (PRBCs) for Symptomatic Anemia
If there is Evidence of Spinal Cord Compression this Also Needs Urgent Neurosurgical &/or RT Evaluation & Rx.
If Infection is Suspected, Rx till R/O
Call the Hematologist

56
Q

What is a standard part of MM treatment?

A

HSCT

57
Q

Waldenstrom Macroglobulinemia

A

This is an Entity that has Some Characteristics of a Lymphoproliferative Disease and Some Characteristics it Share with MM
Usually a Disease of Individuals > 65 y.o.

58
Q

What are the main symptoms of Waldenstrom Macroglobulinemia

A

Lethargy, Confusion, and Weakness
Anemia is Common and may be Sx’ic
Bone Lesions are Not Seen
Peripheral Neuropathy is Fairly Common in Waldenstrom’s

59
Q

Waldenstrom Macroglobulinemia treatment

A

Plasmapharesis is frequently Necessary to Control  Levels of IgM till Chemotherapy Can Be Effective
supportive care

Clin Med- Heme/Oncol (10 decks)

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