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
DLBCL Treatment
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
Burkitt’s Lymphoma
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
Burkitt’s Lymphoma Treatment
Burkitt’s is treated with High Dose Chemotherapy
28
Mantle Cell Lymphoma
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
T Cell Lymphomas
rare Tendency to Involve the Skin and Subcutaneous Tissue Small Cell Varieties are Indolent and Not Curable
30
Peripheral T Cell Lymphoma Not Otherwise Specified (PTCL NOS)
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
Peripheral T Cell Lymphoma Not Otherwise Specified (PTCL NOS) Treatment
Treatment is Generally with CHOP but is Unsatisfactory with a 56% CR
32
Anaplastic Large Cell Lymphoma Alk +
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
Anaplastic Large Cell Lymphoma Alk negative
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
Nasal NK (Natural Killer Cells) T Cell Lymphoma
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
Hodgkins Lymphoma Hodgkins Disease (HD)
Presents with Central Lymphadenopathy and Frequently with B Symptoms (Fever, Wt. Loss, Sweats +/- Puritis) Spreads from one Nodal Group to Adjacent Nodal Groups.
36
Classic Hodgkins Lymphoma Hodgkins Disease (HD)
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
What are the Four Subtypes of Classical HD
Lymphocyte Rich Nodular-Sclerosis Mix Cellularity Lymphocyte Depleted
38
Lymphocyte Rich
Mostly a Disease of Mediastinal LNs in Older Males | Relatively Good Prognosis
39
Nodular-Sclerosis
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
Mix Cellularity
Tend to Present as Stage III or IV Disease with B Sx in Older Men The LN is Diffusely Involved
41
Lymphocyte Depleted
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
Nodular Lymphocyte Predominant HD
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
Tests done for patients with Hodgkin Lymphoma
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
What are 4 special tests that might be needed in patients with lymphoma?
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
Therapy of Classic H.D. Based on Stage
Classic Chemotherapy for HL is ABVD: Adriamycin, Bleomycin, Vinblastine and DTIC, ABVD is given every 2 weeks intravenously
46
Multiple Myeloma the Immunoglobulin is
Usually IgG
47
Waldenstrom’s Macroglobulinemia the Immunoglobulin is?
is IgM
48
Multiple Myeloma (MM) and Waldenstrom’s Macroglobulinemia are both diseases of?
Mature Functioning B Cells, therefore they are characterized by the Production and Over Production of Immunoglobulins
49
Multiple Myeloma (MM)
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
What is Multiple Myeloma (MM) defined by? (CRAB)
``` 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
Monoclonal Gammopathy of Unclear Significance: MGUS
Monoclonal Protein < 3 g/dL < 10% Plasma Cells in B.M. No End Organ Damage
52
Presenting symptoms of multiple myeloma
``` Fatigue SOB Bone Pain, Particularly Back Pain Pathological Fracture Infections Particularly Pneumonias Hypercalcemia: Lethargy, Polydipsia & Polyuria ```
53
diagnosis of multiple myelomas
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
Testing Required if MM is Suspected
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
Treatment of Multiple Myeloma (MM)
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
What is a standard part of MM treatment?
HSCT
57
Waldenstrom Macroglobulinemia
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
What are the main symptoms of Waldenstrom Macroglobulinemia
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
Waldenstrom Macroglobulinemia treatment
Plasmapharesis is frequently Necessary to Control  Levels of IgM till Chemotherapy Can Be Effective supportive care