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59 Cards in this Set

  • Front
  • Back
What is a lymphoma?
A lymphomas is a proliferation of lymphocytes which typically presents with enlargement of the spleen and/or lymph nodes.
What organs are commonly infiltrated by lymphoma?
Liver, lung and bowel
What type of lymphoma is on the rise?
Incidence of Non-Hodgkin's Lymphoma has been on the rise for 60 years.
How is the diagnosis of lymphoma made?
Lymphoma Dx is based on excisional biopsy of a centrally-involved lymph node.
How is Hodgkin's Lymphoma staged?
By the degree of dissemination of the disease.
Stage 1 - Single node
Stage 2 - Multiple nodes, same side of diaphragm
Stage 3 - Multiple nodes, both sides of diaphragm
Stage 4 - Disseminated disease with extra-lymphatic involvement
What is the characteristic histologic finding in Hodgkin's Lymphoma?
The "Reed-Sternberg" Cell
What composes the bulk of a Hodgkin's tumor?
Unlike other malignancies, most cells in a Hodgkin's tumor are normal. Only a few are the binucleate "Reed-Sternberg" cells.
What the two broad sub-types of Hodgkin's Lymphoma?
1) Classical Hodgkin's
2) Nodular Lymphocyte Predominant Hodgkin's Disease (NLPHD)
What are the four sub-types of Classical Hodgkin's Disease?
1) Lymphocyte Rich (rare - good prog.)
2) Nodular Sclerosis (50% - good prog.)
3) Mixed Cellularity (40% - med. prog.)
4) Lymphocyte Depletion (5% - worst prog.)
What are B-Symptoms in Hodgkin's Disease?
Constitutional symptoms: fever, night sweats, unexplained weight loss of more than 10% in the past 6 months.
What imaging technique can differentiate an active HD tumor?
PET Scan
What is the primary goal in the treatment of Hodgkin's Disease?
HD is treated for CURE, so accurate staging and chemotherapy is critical.
What is the therapy for Hodgkin's Disease? What are the cure rates?
Limited Chemo + Limited Radiation. Cure rates are 70-90%
What is the modern chemo regimen for Hodgkin's Disease?
Hodgkin's: ABVD
A - Adriamycin
B - Bleomycin
V - Vinblastine
D - DTIC
What are the older chemo regimens for Hodgkin's Disease?
MOPP
How is prognosis and therapy determined for Non-Hodgkin's Lymphomas (NHLs)?
The histologic pattern seen in involved lymph nodes.
What are the two most important lymph features in NHL?
1) Overall architecture (diffuse or clustered)
2) Individual tumor cell appearance (large vs. small cells)
From what cell lines are NHL tumors derived?
85% from B-Cells
15% from T-Cells
What are the two types of NHL classifications for this course?
"Indolent/Low Grade" and "Aggressive/Intermediate & High Grade"
Describe the Classification, Histology, Mean survival, and Chemo for NHL.
What is the typical clinical presentation of NHL?
Painless Adenopathy.
Which lymphomas are curable?
Aggressive lymphomas are often more curable than indolent ones.
What chemotherapy drug seems to be key in curing aggressive lymphoma?
Doxorubicin (Adriamycin)
What are the treatment options for INDOLENT NHL?
Stage 1-2 Indolent: radiotherapy alone (50% cure)
Stage 3-4: no therapy, or chlorambucil, CVP and/or rituximab (CD20)
What does Rituximab recognize?
The CD20 antigen
What's CVP, and what disease is it used for?
CVP is a chemo regimen for Indolent Stage 3-4 NHL
* Cyclophosphamide
* Vincristine
* Prednisone
What is the standard chemo for an Aggressive Non-Hodgkin's Lymphoma?
CHOP:
* Cyclophosphamide
* Doxorubicin (Hydroxydaunorubicin)
* Vincristine (Oncovin)
* Prednisone
How often can remission be induced with CHOP therapy for NHL?
60-80% of aggressive NHL patients will go into remission
What is a Plasma Cell Dyscrasia?
Disorders of the plasma, or B-Cells, that often generate extra antibodies. Examples are multiple myeloma and Waldenstrom's macroglobulinemia.
Describe the following Serum Protein Electrophoresis (SPEP) curves:
1) Normal Pattern
2) Polyclonal Gammopathy
3) Monoclonal Gammopathy
4) Hypogammaglobulinemia
What are the four major Plasma Cell Dyscrasias?
1) Multiple Myeloma
2) Waldenstrom's Macroglobulinemia
3) Primary Amyloidosis
4) Monoclonal Gammopathy of Undetermined Significance (MGUS)
What are the four clinical manifestations of the plasma cell disorders?
1) the tumor
2) Paraproteins made by the tumor
3) Decreased normal Ig proteins
4) Other factors produced by the tumor
What is Mutiple Myeloma?
A malignant disorder characterized by the monoclonal proliferation of plasma, or "myeloma," cells.
How does MM typically present?
MM commonly presents as "bone pain," esp. in the vertebrae, ribs and long bones. Anemia, thrombocytopenia and neutropenia are common.
What are focal collections of myeloma cells called? How might they present?
Plasmacytomas, which might present as subcutaneous nodules.
Why are lytic lesions common in MM?
MM tumor cells produce TNF-Beta, which activates osteoclasts and increases bone resorption.
What is the most common lab abnormality in MM?
A hypo-proliferative anemia, along with "rouleax" RBCs.
What is a "rouleaux" finding?
RBCs stacking on the peripheral smear. Increased serum IgG binds to RBCs, decreasing the normal ionic repulsion between them.
What is the key diagnostic tool in MM?
Serum Protein Electrophoresis (SPEP). A prominent MONOCLONAL M-Spike will show.
What classic urine finding is associated with MM?
Bence Jones Proteinuria. About 10% of MM will produce the Ig Light Chain proteins, which are small enough to be excreted in the urine.
What are the marrow findings with MM? Is marrow biopsy a required test with MM?
MM requires marrow biopsy. Typical findings include 10-30% malignant plasma cells. Easier to detect on biopsy than aspirate.
What are the 4 diagnostic characteristics of MM?
1) >10% abnormal plasma marrow cells
2) Serum or urine monoclonal gammopathy
3) Lytic bony lesions
4) Plasmacytomas

Not all features are required to make the diagnosis.
What are the common clinical complications of MM?
1) Renal Failure
2) Pathologic Fractures
3) Paraprotein assoc. with hyperviscosity
What are the issues commonly associated with high levels of paraprotein in MM?
1) Hyperviscosity leading to vascular stasis
2) Interference with platelet function leading to mucosal bleeding
3) Suppression of normal plasma cell growth and decreased normal Ig
Is there a curative therapy for MM?
No, only palliative therapies and stem cell transplant.
What is a suggested palliative chemotherapy for MM?
1) Thalidomide & Dexamethasone
2) Lenalidomide & Dexamethasone
3) Lenalidomide & Bortezomib (relapse)
How might local MM plasmacytomas be managed?
Local radiation therapy may be used to control painful tumors.
What can be done about bone resorption in MM?
Bisphosphonates reduce bone resorption in MM, and may even be toxic to tumor cells.
What is Waldenstrom's Macroglobulinema?
A hyperviscous plasma disease similar to MM.
What proteins are produced in WM? Is this clinically important?
IgM proteins. Because they are so large, complications of hyperviscosity are more common on WM.
What are the symptoms of hyperviscosity syndrome?
1) Headache
2) Confusion
3) Visual disturbances
4) Difficulty hearing
5) Coma
What is the "Classic Triad of Hyperviscosity?"
1) Bleeding (epistaxis, ecchymosis)
2) Visual changes (blurred)
3) Neurologic changes (HA, dizziness)
What are the laboratory findings in WM?
Marked increase in IgM in Serum Plasma Electrophoresis. Pancytopenia is possible with extensive marrow replacement
How is WM treated?
There is no cure for WM. Drugs include:
* Chlorambucil
* Fludarabine
* Rituximab
What is the median survival time for WM?
Approx. 5 years
How is hyperviscosity managed acutely?
Plasmapheresis
What are the clinical features of Amyloidosis?
Fatigue, weakness, weight loss, dyspnea, purpura and EDEMA.
How is Amyloidosis diagnosed?
Isolation of amyloid protein with apple-green birefringence in tissue samples. Bone marrow biopsy is also performed
What does Congo Red staining in the marrow indicate?
Amyloidosis