17. Lymphomas and Myeloma Flashcards
Differentiate between lymphoma and myeloma.
What % of different cell types are responsible for lymphomas?
Describe the 2 types of lymphoma.
What are the features of lymphoma?
Lymphoma: blood cell tumours, develop from lymphocytes. 2 main categories = Non Hodgkin’s (NHL) and Hodgkin’s (HL).
Myeloma: cancer of plasma cells, produce abnormal abs -> kidney problems and thick blood.
90% B cell, 10% T and NK cell
NHL: indolent/low grade, aggressive/high grade. All lymphomas without Reed-Sternberg cells, mainly from B cell lines.
HL: nodular sclerosing, mixed cellularity, lymphocyte rich, lymphocyte deplete, nodular lymphocyte predominant. Reed-Sternberg cells (mirror-image nuclei)
Lymphadenopathy (pic - preauricular, submandibular), fever, night sweats, weight loss, fatigue, itching (puritis), pain on drinkin g alcohol (HL). Underlined = B symptoms - help determine grade of lymphoma.
Where should you look in an examination of the lymphatic system?
What is the Ann Arbor staging system for lymphomas?
Neck, axillae, abdomen, groin.
[PIC]
Stage 1: cancer located in single region, usually 1 LN, often no outward symptoms
Stage 2: in 2 seperate regions, both confined to 1 side of the diaphragm (above/below)
Stage 3: spread to both sides of diaphragm, including 1 organ/area near LNs or spleen
Stage 4: diffuse/disseminated involvement of 1/more extralymphatic organs, incl. liver/BM/nodular involvement of lungs
Letters A/B can be added to some stages. A = no associated symptoms; B = unexplained fever, night sweats and weight loss >10% in last 6 months. May suggest high grade lymphoma
What is the only way a lymphoma can be diagnosed?
What might you see in a lymphoma biopsy?
What are the cells indicated here, and what does it suggest?
Biopsy. Types: fine needle (poor dx, for neck LNs), core biopsy or excision biopsy (gold std). Histological appearance and immunostaining. Need to pick up CD20 antigen found on B cells associated with this type of lymphoma.
Lots of centriblasts (big, blastoid like, nucleolus), centrocytes (smaller, blue). [Pic]
Reed Sternbeck cells - Hodgkin’s lymphoma
Label A-D of this biopsy.
A: adipose tissue
B: lymphoid follicles
C: germinal centre
D: capsule
What are A and B in this biopsy?
What can you see?
What is Burkitt’s lymphoma?
A: Tumor cells
B: lung tissue
Monomorphic distrubtion of cells, packed LN in respiratory tissue, amorphous cell mass.
High grade NHL that develops from B cells, one of the fastest growing - so more likely to be cured than other types of lymphoma, affects children and adults, sometimes linked to EBV (causes glandular fever).
What are the characteristic translocations of the following types of lymphomas?
- *a) Follicular lymphoma**
b) Burkitt lymphoma - *c) DLBCL (diffuse large B cell lymphoma)**
d) Mantle cell lymphoma
- *a) t(14:18) - BCL2**
b) t(8:14), t(8:22) - c-MYC
c) t(14:18) or t(3:14) - BCL2
d) t(11:14) - CCND1
For DLBCL and Follicular lymphoma (both NHL), and for Hodgkin’s lymphoma, compare the:
a) Age
b) Percentage of cases
c) Most common race found in
d) Majority stage?
e) B symptoms?
f) Agressive or slow?
g) Extranodal involvement?
h) Curable?
What other conditions may be assocaited with lymphoma?
What other investigations would you do?
What is the FLIPI score?
Viral infections (EBV, HIV, Hep C), bacterial infections (H. pylori, chlamydia psittaci), inflammatory conditions (Coeliac disease, Sjogren’s syndrome), industrial/medical exposure (ionising radiation, benzene, immunosupression)
CT PET (can give radiolabelled glucose and find ares of high metabolic intake e.g. brain and heart, and lymphoma), FBC (if lymphoma advances can be apparent in blood - increased WCC, if affects bone marrow = reduced plt), virus test, basic biochemistry (e.g. kidney function b/c can be affected by retroperitoneal masses, LDH - overview of cancer turnover). BM involvement?
Follicular lymphoma International Prognostic Index, based on several factors: age, stage, Hb, LDH and number of LN areas involved. Pts that score 3 or more = decease rapidly.
How is lymphoma treated if established or non-established?
What is mycosis fungoides?
Lymphomas are a diverse group of related cancers characterised by lymphadenopthy +/- B symptoms. What are the broad devisions?
If established: watch and wait (early causes), steroids (treatment backbone), radiotherapy (can have toxic SEs), chemo +/- monoclonal abs (e.g. Rituximab - binds to CD20 and helps kill B cells) Hodgkin’s don’t have CD20 so use ABVD regime, [R-CHOP], SC translation (if v aggressive).
If non-established: pathway inhibitors, engineered CAR-T cells (modified T cells recognise and kill cancer - pic)
Most common form of cutaneous T-cell lymphoma, generally affects skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin.
NHL, HL, high grade vs low grade, B vs T cell
What is a myeloma?
Describe the pathophysiology of myeloma.
Malignant neoplasm of plasma cells, may be preceded by MGUS (when an abnormal protein (M protein) is in the blood, produced by abnormal plasma cell. Average age 70yrs. Most have paraprotein in serum/monoclonal band - seen on serum/urine electrophoresis. So e.g. if it’s an IgG producing abnormal cancerous cell would see IgG when measure blood - see monoclonal band with supression of all other ab producing cells -> become B immunodeficiency
Myeloma -> marrow infiltration (anaemia) -> release of cytokines -> bone destruction -> bone pain and hypercalcaemia.
Myeloma -> monoclonal protein -> renal failure, hyperviscosity, amyloidosis, LCDD
Myeloma -> immune deficiency -> infection
How is myeloma diagnosed?
OLD CRAB is a mnemonic for what symptoms of multiple myeloma?
How is myeloma treated?
Urine and serum electrophoresis, bone marrow examination, skeletal survey (look for bone lesions), bloods (look at all proteins in blood/serum, should see them in 5 diff bands (green), but in myeloma = spike in gamma region (pic). Do immunology test to know which Ig it is).
OLD age, Calcium elevated (hypercalcemia), Renal failure, Anaemia, Bone lytic disease.
Newly diagnosed and trasplant candidate -> induction therapy -> auto transplant -> maintenance options?
Newly diagnosed and not transplant candidate -> Melphalan-based regimens -> observation
New treatments: thalidomide, lenalidomide, bortezomib, monoclonal abs. Chemotherapy. Autologous SC transplant. Radiotherapy (radiate lesions -> pain relief).
