Term
| What are the 3 different groups of Non-Hodgkin's Lymphomas? |
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Definition
Based on aggressiveness of course
1) Indolent NHL- slow growing but high recurrence and resistant to therapy
- Follicular
- Small lymphocytic
- Marginal zone
- Mycosis fungoides/Sezary syndrome
2) Aggressive NHL- fast growing but respond to chemo
- Diffuse large B cell
- Mantle cell
- Anaplastic large cell
3) Highly aggressive- very fast growing and respond well, but can spread to CNS
- Burkitts
- Precursor B or T cell leukemia/lymphoma
- Adult T cell leukemia/lymphoma |
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Term
Which of the following is NOT a major risk factor for Lymphoma?
1. SCID
2. Organ transplant
3. HH8 infections
4. RDS
5. Agent orange
6. Herbicide exposure |
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Definition
4.
1/2. Immune deficiency IS (AIDS, Hypogamma, CVID, SCID, organ transplant, autoimmune)
3. Infection is common
5. Chemicals like agent orange are associated |
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Term
| What are the classic clinical features of Lymphoma? |
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Definition
1) Poor appetite/weight loss
2) Lymphadenopathy, splenomegaly
3) Fever, chills, sweats (B SYMPTOMS)
4) Fatigue
5) Pruritis after alcohol use (Hodgkin's) |
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Term
Patient presents complaining of fever, fatigue and lack of appetite. He says that he has been feeling "itchy" after drinking alcohol.
On PE, you notice hepatosplenomegaly, lymphadenopathy and a skin rash.
What do you expect to find in labs and what tests should you order? |
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Definition
1) Labs
- Elevated blood counts
- elevated LFTs (liver) and serum creatinine (renal)
- Elevated LDH and UA with aggressive forms
- Abnormal cells of flow cytometry
2)
- Order CBC, liver and renal tests, LDH, uric acid
- Peripheral blood flow
- Lymph node biopsy and bone marrow
- |
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Term
| Why might you give a patient with suspected lymphoma allopurinol, IVF and bicarbonate? |
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Definition
Tumor Lysis Syndrome!
- Sudden breakdown of tumor cells with hyperuricemia, hyperkalemia, hyperphosphatemia, uric acid crystalization in urine and renal failure. |
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Term
| How are lymphomas staged clinically? |
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Definition
Ann Arbor staging
Modifiers
A or B (absence of presence of symptoms)
E (extra-nodal disease)
X (bulky disease)
S (splenic spread)
Stage 1- single region, usually one group of LN
Stage 2- 2 or more separate regions on one side of diaphragm
Stage 3- Both sides of diaphragm
Stage 4- Extra-lymphatic involvement (marrow, liver, lungs) |
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Term
Describe what is meant by each of the following lymphoma grades.
1. Grade 2B
2. Grade 3AX
3. Grade 3BE |
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Definition
1. tumor involves more than 2 groups of LN, but is restricted to one side of diaphragm. "B" means symptoms are present.
2. Asymptomatic, "bulky" tumor that involves both sides of diaphragm
3. Symptomatic tumor with extra-nodal spread on both sides of diaphragm.
"S" means splenic involvement |
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Term
| How is prognosis of lymphoma patients determined? |
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Definition
International Prognostic Index (IPI)
1 point for each:
1) Age (> 60?)
2) Stage III or IV
3) Elevated LDH
4) Poor performance status
5) >1 extra-nodal side
0-1: Low risk (5 year 73%)
2: low-intermediate
3: high intermediate
4-5: high (5 year 26%) |
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Term
| What is the prognosis of a patient with lymphoma who is 73 years old, with Stage IIIE disease, normal LDH and performance status. |
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Definition
Age > 60 is 1 point
Stage III/IV disease is 1 point
E of > 3 sites is 1 point
Normal LDH and performance is 0 points
Score on IPI is "3", meaning High-intermediate risk, wish 5-year survival at 43% |
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Term
What type of lymphoma is described by each of the following?
1. Lymphoma of 20-30 year olds and >50 year olds that is associated with EBV
2. Characterized by Bcl-2 translocation next to heavy chain, near promotor, t(14:18), causing indolent disease
3. Accumulation of monoclonal lymphocytes in peripheral blood of elderly patients with concern of Richter's transformation
4. Lymphoma associated with chronic infection and inflammation that is treated with antibiotics and occasionally, rituximab and chemo.
5. Characterized by microabscesses and medium/large lymphocytes with ceribriform nuclei and scant cytoplasm. |
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Definition
1. Hodgkin's Lymphoma= Reed Sternberg cells
- Rare and 95% cured with radiation, chemo or combination
2. Follicular Lymphoma
- Common (40%), B cell lymphoma from follicles of LN, treated (80%) with Rituximab in combination with chemo
- Bcl-2 is anti-apoptotic gene
3. Small cell lymphoma/chronic lymphocytic lymphoma
- common and indolent in elderly population and commonly asyptomatic at presentation
4. Marginal zone lymphoma
- Indolent lymphoma treated with antibiotics (infection) and rituximab/chemo occassionally.
- Can be nodal, extra-nodal or splenic (Hep C, monoclonal gammopathy and autoimmune HA)
5. Mycosis fungoides/ Sezary syndrome
- Indolent, cutaneous T cell lymphoma with skin involvement (Pautrier's microabscesses and Sezary cells). |
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Term
Which of the following regarding Hodgkin's lymphoma is FALSE?
1. Associated with HSV infection
2. Characterized with Reed-Sternberg cells
3. Found in 20-30 year olds and those > 50
4. Cured with radiation and/or chemo in 95% of cases |
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Definition
| 1. 40% association with EBV! |
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Term
Which of the following associations with Marginal Zone lymphoma in INCORRECT?
1. Gastric MALT- H. pylori
2. Ocular-adnexal MALT- Chlamydia psittaci
3. Small intestine MALT- Treponema pallidum
4. Cutaneous MALT- Borrelia burdorferi |
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Definition
8-10% of HNL associated with chronic infection and inflammation (e.g. splenic zone with **Hep C**, monoclonal gammopathy and autoimmune HA)
3- Small intestine MALT related marginal zone lymphoma is associated with C. jejuni, not Syphilis. |
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Term
Which of the following characteristics is true of Mycosis fungoides lymphoma?
1. Aggressive course
2. Pautrier's microabscesses
3. Associated with Hepatitis C
4. Bcl-2 translocation
5. Reed sternberg cells |
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Definition
2. Mycosis fungoides lymphoma is cutaneous T-cell lymphoma that produces indolent disease associated with Pautrier's microabscesses and Sezary cells (large lymphocytes with ceribriform nuclei and scant cytoplasm)
2. Aggressive lymphomas include Diffuse large B cell, Anaplastic large cell and Mantle cell.
3. Hep C is associated with Marginal Zone lymphoma at splenic margin (also hypogammaglobulinema and autoimmune HA)
4. Bcl-2 translocation occurs in Follicular NHL, t(14:18), which leads to high levels of anti-apoptotic gene.
5. Reed sterberg cells as found in Hodgkin's lymphoma, which is associated with EBV |
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Term
What type of lymphoma is described by each of the following?
1. Most common NHL, with rapidly enlarging tumors and associated with 3q27 abnormalities involving Bcl-6 gene
2. Characterized by t(11:14) involving Cyclin D1 oncogene in older males.
3. Highly aggressive lymphoma that spreads to CNS with "starry sky" biopsy pattern
4. Lymphoma associated with C-Myc gene translocation on chromosome 8 and tumor lysis syndrome
5. Characterized by microabscesses and medium/large lymphocytes with ceribriform nuclei and scant cytoplasm. |
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Definition
1. Diffuse Large B cell lymphoma
- Most common NHL and 20-30% of all lymphomas
- Good response to chemotherapy
2. Mantle Cell lymphoma (germinal centers)
- G1-S progression occurs with rapid progression
- Rituximab, with chemo and stem cell rescue is usually required (50% at 5 years)
3) Burkitt's lymphoma
- Highly aggressive (Endemic, Sporadic or Immunodeficient)
- Biopsy shows macrophages with ingested apoptotic tumor cells (starry sky)
- Associated with "C-Myc" transocation on chromosome 8 and tumor lysis syndrome
- CNS prophylaxis mandatory
4) ALSO BURKITT's
5) Mycosis fungoides lymphoma is cutaneous T-cell lymphoma that produces indolent disease associated with Pautrier's microabscesses and Sezary cells (large lymphocytes with ceribriform nuclei and scant cytoplasm) |
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Term
Which of the following is NOT characteristic of Burkitt's lymphoma
1. C-Myc translocation on chromosome 6
2. Numerous macrophages with ingested tumor cell fragments on biopsy.
3. Aggressive course
4. Treated with CNS prophylaxis and intensive chemotherapy
5. Associated with tumor lysis syndrome |
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Definition
1) Chromosome 8!
Burkitt's lymphoma
- Highly aggressive (Endemic/jaw in kids, Sporadic/young healthy adults with lymphadenopathy and splenomeagly, or Immunodeficient/HIV patient)
- Biopsy shows macrophages with ingested apoptotic tumor cells (starry sky)
- Associated with "C-Myc" transocation on chromosome 8 and tumor lysis syndrome
- CNS prophylaxis mandatory |
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Term
| Describe the 3 types of Burkitt's lymphoma. |
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Definition
Starry sky biopsy and C-MyC on chromosome 8
1) Endemic: jaw in kids
2) Sporadic: young healthy adults in US, with lymphadenopathy and splenomeagly
3) Immunodeficient: HIV patient or EBV association (distinguish from Hodgkin's) |
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Term
The gene commonly translocated in Burkitt's lymphoma is:
1) cMyc
2) Cyclin D1
3) Bcl-2
4) MLL |
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Definition
| 1: cMyc present on chromosome 8 is commonly translocated in Burkitt’s lymphoma. Cyclin D1 translocations are seen in Mantle cell lymphoma while Bcl-2 translocations are seen in follicular lymphoma. MLL re-arrangements are uncommon in lymphomas but are commonly seen in acute leukemia. |
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Term
Splenic marginal zone lymphoma is associated with which of the following infections:
1) Hepatitis B
2) Hepatitis C
3) C. jejeuni
4) Borrelia burgdorferi
5) H. Pylori |
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Definition
| 2: Hepatitis C is commonly associated with splenic marginal zone lymphoma, usually treatment of underlying infection leads to improvement in the lymphoma. C. jejeuni, borrelia and H pylori are associated with intestinal, cutaneous and gastric MALT lymphomas respectively. |
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Term
Hodgkin's disease is characterized by which of the following pathological findings:
1) Reed Sternberg cell
2) Diffuse large B cells
3) Small round sheets of lymphocytes
4) Popcorn cells |
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Definition
| 1: Reed Sternberg cell is pathognomonic of Hodgkin’s lymphoma and is characterized by abundant slightly basophilic cytoplasm and at least two nuclei. The nuclei have an eosinophilic nucleolus surrounded by a peri-nuclear halo. |
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Term
Richter's transformation involves:
1) change of an indolent lymphoma into a high grade, aggressive lymphoma
2) bleeding into lymphoma leading to enlargement
3) CNS spread of lymphoma causing symptoms
4) Hyperuricemia, hyperkalemia and renal insufficiency from breakdown of lymphoma cells |
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Definition
1: Involves transformation of an indolent lymphoma into a high grade, aggressive lymphoma like DLBCL. Characterized by sudden lymphadenopathy, new B symptoms and BM involvement.
Tumor Lysis syndrome is characterized by sudden breakdown of tumor cells which causes hyperuricemia, hyperkalemia, hyperphosphatemia, uric acid crystallization in urine and renal failure. |
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Term
| What are the major methods that are available and can be used to characterize lymphoid proliferation? |
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Definition
1) LN biopsy- H & E stain
2) Phenotype
- Flow cytometry
- Immunohistochemistry
3) Cytogenetics
- Classical cytogenetic studies
- Fluorescence in-situ hybridization (FISH)
4) Molecular studies
- Southern blot
- PCR |
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Term
| What features can help you distinguish between reactive lymphoid proliferation and a lymphoma? |
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Definition
1) Lymphomas tend to composed of a homogenous cell population, while reactive lymphoid proliferation tends to have many kinds (Histology)
2) Flow cytometry
- homogenous antigen population
- light chain types (e.g if y>>>>>>k, think monoclonal)
3) Cytogenic
4) Molecular |
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Term
What can the presence of each of the following CD markers tell you?
1) CD10
2) CD19
3) CD20
4) CD79a
5) kappa or lamda |
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Definition
1) Pre-B cells and mature germinal center B cells
2) Pre-B cells and mature B cells
3) Pre-B cells after CD19 and mature B cells, no plasma cells
4) Pre-B cells, Mature B cells AND plasma cells
5) Mature B cells, not plasma cells |
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Term
A 55 year old man noted a lump in his neck while shaving. He felt perfectly well otherwise. The mass was excised and sent fresh to Hematopathology.
On histology, you notice follicle proliferation with a homogenous population of lymphocytes.
On Flow, you see a homogenous population of CD10+ cells expressing only kappa and no lamba light chain
What might FISH analysis show? |
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Definition
This sounds like classic follicular lymphoma, a NHL with an indolent, difficult to treat course.
The classic cytological feature is a reciprical t(14:18) of the bcl2 gene (apoptosis inhibitor)
You should follow up with bone marrow examination with aspirates and biopsies (because the lymphoma might not aspirate well) for staging.
CT scans looking for adenopathy and other masses. PET/CT scans are also used to look for evidence of lymphoma |
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Term
True or False:
Transformed follicular lymphoma to diffuse large B cell lymphoma is more concerning than a primary diffuse large B cell lymphoma. |
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Definition
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Term
18 year old male presented with chest discomfort. A large anterior mediastinal mass was identified on CT scan as well as a pleural effusion.
What type of lymphoid neoplasm would you suspect in these circumstances? |
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Definition
H/x and age is classic for T-ALL
1) T-lymphoblastic leukemia/lymphoma
2) Hodgkin lymphoma
3) Mediastinal large B-cell lymphoma |
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Term
| What is the difference between "transformed" and "blastic cells"? |
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Definition
1) Blastic cells are recursor (primitive) cells including lymphoblasts and myeloid blasts (the types of cells seen in acute leukemias).
- features include medium sized, dispersed chromatin (very fine/delicate, like a fine haze), nucleoli not prominent, many mitotic figures.
2) Transformed cells are mature cells that have been stimulated in some fashion analagous to what happens with mitogen stimulation of normal lymphocytes. |
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Term
24 year old female presents with a very large mediastinal mass, fever and night sweats. She is also found to have supraclavicular lymphadenopathy on physical examination.
On histology, you discover a thickened, nodular appearing LN with collagen bands that are birefingent under polarized light
What do you expect to see on a blood smear and how do you treat? |
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Definition
Sounds like nodular sclerosing hodgkin's lymphoma, especially in young woman.
Could be more certain with flow, looking for CD30+ CD15+ cells lacking other typical B and T cell markers.
1) Look for numerous, small lymphocytes, eosinophils, and REED STERNBERG cells
- There is a high lymphocyte:RSC ratio, which indicates good prognosis.
2) |
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