Myelomas & Lymphomas Flashcards
What are 6 lymphoid organs?
Bone marrow Thymus Lymph nodes Spleen Tonsils, adenoids Digestive tracts
Where is the origin of MOST lymphomas?
Most Lymphomas are of B Cell Origin. B Stands for Bowel or Bursa. B Cells are the Immunoglobulin Antibody Producing Cells.
What are the 2 classes of lymphomas?
Non-Hodgkin’s Lymphoma (NHL)
Hodgkin’s Disease (H.D.)
which lymphoma is composed of larger cells?
which of smaller cells?
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)
All Lymphomas are Characterized by
Lymphadenopathy and the Histology of the Lymph Node is the Most Fundamental Way the Different Lymphomas are Characterized
Lymphadenopathy in NHL spreads how?
Spreads Non-Contiguously from Peripheral Lymph Node Groups Inward Toward the Central Lymph Node Groups.
Lymphadenopathy in H.D. spreads how?
Contiguous from Central Lymph Node Groups Outward
In H.D. what are the majority of the cells?
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
In the NHL what are the majority of the cells?
Most of the Cells in the Diagnostic Lymph Node are Malignant
Chronic Lymphocytic Leukemia; Small Lymphocytic Lymphoma Epidemiology
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
2 MC symptoms of Chronic Lymphocytic Leukemia; Small Lymphocytic Lymphoma
Fatigue and Lymphadenopathy.
If the Patient with CLL has a Fever, Assume Infection.
3 Lab finding for Chronic Lymphocytic Leukemia (CLL); Small Lymphocytic Lymphoma (SLL)
Lab findings besides Lymphocytosis include Anemia and Thrombocytopenia. Most Patients have decreased IgG
Staging, The Rai System for CLL
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
Treatment of CLL/SLL
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
Hairy Cell Leukemia (HCL)
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
Splenic Marginal Zone Lymphoma/Leukemia
Has Splenomegaly and Lymphocytosis like HCL without Lymphadenopathy
Has a Clinical Course like CLL & is Not Curable with Purine Analogues
Marginal Zone Lymphomas (MZL) and the Mucosal-Associated Lymphoid Tissue Lymphomas (MALT Lymphomas)
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
Marginal Zone Lymphomas (MZL) and the Mucosal-Associated Lymphoid Tissue Lymphomas (MALT Lymphomas) Treatments*
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
The Follicular Lymphomas (FL)
FL is the 2nd most Common of the Adult Lymphomas, ~ 30%.
They are usually Considered Low Grade
Staging is Based on the Follicular Lymphoma International Prognostic Index (FLIPI)
One Point for Each Characteristic: Age > 60 y.o. increased LDH Ann Arbor Stage III or IV Hgb < 12 Extra-nodal Disease
Follicular Lymphomas: Characterized by?
Positive B Cell Markers
Follicular lymphoma treatment?
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
Diffuse Large B Cell Lymphoma (DLBCL)
Most Common of the Adult Lymphomas ~35%
An Aggressive Lymphoma that Frequently Involves Extra-nodal Sites e.g. the GI Track
International Prognostic Index for Aggressive High Grade Lymphomas
1 Point for Each: Age > 60 increased LDH PS 2-4 Extra-nodal Sites Ann Arbor Stage 3 or 4
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
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
Burkitt’s Lymphoma Treatment
Burkitt’s is treated with High Dose Chemotherapy
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
T Cell Lymphomas
rare
Tendency to Involve the Skin and Subcutaneous Tissue
Small Cell Varieties are Indolent and Not Curable
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
Peripheral T Cell Lymphoma Not Otherwise Specified (PTCL NOS) Treatment
Treatment is Generally with CHOP but is Unsatisfactory with a 56% CR
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
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
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)
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.
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
What are the Four Subtypes of Classical HD
Lymphocyte Rich
Nodular-Sclerosis
Mix Cellularity
Lymphocyte Depleted
Lymphocyte Rich
Mostly a Disease of Mediastinal LNs in Older Males
Relatively Good Prognosis
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
Mix Cellularity
Tend to Present as Stage III or IV Disease with B Sx in Older Men
The LN is Diffusely Involved
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
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
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
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
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
Multiple Myeloma the Immunoglobulin is
Usually IgG
Waldenstrom’s Macroglobulinemia the Immunoglobulin is?
is IgM
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
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
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
Monoclonal Gammopathy of Unclear Significance: MGUS
Monoclonal Protein < 3 g/dL
< 10% Plasma Cells in B.M.
No End Organ Damage
Presenting symptoms of multiple myeloma
Fatigue SOB Bone Pain, Particularly Back Pain Pathological Fracture Infections Particularly Pneumonias Hypercalcemia: Lethargy, Polydipsia & Polyuria
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
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
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
What is a standard part of MM treatment?
HSCT
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.
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
Waldenstrom Macroglobulinemia treatment
Plasmapharesis is frequently Necessary to Control Levels of IgM till Chemotherapy Can Be Effective
supportive care
