Lymphomas Flashcards
How do lymphomas begin?
by the “malignant transformation” of a lymphocyte in the lymphatic system
What is the cause of follicular lymphoma?
overexpression of BCL-2 (gene that blocks programmed cell death)
Where does it start?
Lymphoma
Leukemia
Lymphoma- starts in the lymph nodes or lymphatic system
Leukemia- tumor or cancer of blood cells or bone marrow
MOA
Lymphoma
Leukemia
Lymphoma invades other organs by unnecessary and uncontrollable growth of a lymphoid tissue
Leukemia produces abnormal white blood cells which inhibits the function of the immune system and platelet function
How does each present?
Lymphoma
Leukemia
Lymphomas- present with signs of swollen lymph nodes in the neck, armpits, or groin which are frequently painful
Leukemia-have sx typical of other cancers and may have no early telling symptoms, especially if chronic
Hodgkin Lymphoma Incidence characterized by who gets this (incidence) How does it spread? (orderly vs. not) Involves what cells
Incidence- 1% of cancers, 11% of lymphomas
characterized by- Reed-sternberg cells
who gets this (incidence)-bimodal, young adults and elderly
How does it spread? (orderly vs. not)- Orderly
Involves what cells- abnormal B cells
Types of HL
Nodular sclerosis (60-75%) **predominant Mixed cellularity (5-15%) Lymphocyte rich (5%) Lymphocyte depletion (5%)
Non-Hodgkin Lymphoma Incidence characterized by who gets this (incidence) How does it spread? (orderly vs. not) Involves what cells
Incidence- 4% cancers, 89% lymphomas
characterized by- Nothing special. This just includes anything without the Reed-Sternberg cells.
who gets this (incidence)- adults, ~60yo
How does it spread? (orderly vs. not)- Disorderly fashion
Involves what cells- abnormal B cells (most common) or T cells
Types of Non-hodgkin lymphoma
Over 60 different types
Indolent -follicular lymphoma -MALT lymphoma Agressive -diffuse large B-cell lymphoma -AIDS-associated lymphoma -Burkitt's lymphoma
Symptoms of HL
- Painless enlargement of lymph nodes, slow growth usually
- swelling from edema
- SOB, fatigue
- weight loss, muscle weakness
- Pel-Ebstein fever (very variable fever), night sweats
- Increased susceptibility to infections
Symptoms of NHL
- RAPID, painless enlargement of lymph nodes
- progressive swelling of legs, swelling of face
- pale skin or dark, itchy patches of skin
- difficulty breathing, chest pain
- pleural effusion, causing persistent cough
- bloating, cramping, diarrhea (MALT)
- abd pain or distention, constipation
- fever, weight loss
- increased susceptibility to infections
What is the Ann Arbor system used for?
Staging of lymphoma
- classifies tumors into categories based on where the tumor is present
- useful in determining specific treatments, prognosis
Diagnostic testing for lymphomas
- CXR
- CT scans*
- Bone marrow aspiration/biopsy*
- PET scan
- Gallium scan
- MRI
- Lumbar puncture
Ann Arbor Staging System
staging and site involvement
sub staging and description
Stage 1- single LN region or extralymp organ or site
Stage 2- Cancer in 2 or more groups of LN on same side of diaphragm
Stage 3- Caner in groups of LN both above and below diaphragm
Stage 4- Cancer has spread to one or more organ or tissure site
A- No systemic symptoms present at dx
B- fever, night sweats, weight loss
E (extranodal)-cancer in an area or organ other than LN
S (spleen)- Cancer spread present in the spleen
When is Grading used?
in NHL
-describes how quickly how quickly cancer is growing
Grading
grade and description
Low- slow growing, incurable (follicular, waldenstroms)
Intermediate- rapid growing, potentially curable
High-fastest growing, potentially curable (Burkitt’s lymphoma)
can also use
Indolent-grow very slowly, need little or no treatment until sx appear. tend to recur after treatment
Aggressive-grow quickly, symptomatic and tx required immediately, can be treated with intense chemo
Risk factors for HL
- h/o mononucleosis increases risk of young-adult HL
- sibling dx w/HL
Risk factors for NHL
- Toxic compounds in atmosphere
- EBV after organ transplant
- h. pylori can cause MALT lymphoma
- other viruses such as Hep C
- Genetic predisposition
Risk factors for both HL and NHL
- HIV
- AIDS
- HTLV (human T-cell virus)
- Suppressed immune function
- S. japan, caribbean, S. America, Africa
Clinical features of HL
- Asymptomatic lymphadenopathy (70%)
- Splenomegaly
- systemic symptoms: B symptoms- fever, puritis, night sweats
- Non-specific: alcohol induced pain in nodes, nephrotic syndrome
- disease spread in contiguity with lymph system
What would the results of a HL CBC be?
anemia, eosinophilia, leukocytosis, platelets increased the decreased in advanced disease
What would you order for HL (labs and diagnostic)?
- CBC
- LFT, ESR, LDH
- CXR, CT
- Cardiac function assessment
- excisional lymph biopsy confirms dx
- bone marrow bx (if B sx present)
Stage is often more important than the type for prognosis. True or false
True
What do the Reed-Sternberg cells look like and what disease might they be present in?
“Owl eyes” and HL
HL treatment methods
- surgery
- radiation
- chemo
- biotherapy
- peripheral blood stem cell transplant (PBSCT)
Treatment HL
Stage 1-2
Stage 3-4
Relapse after tx
Stage 1-2: chemo followed by XRT
Stage 3-4:Chemo wiith XRT for bulky disease
Relapse after tx: high dose chemo, bone marrow transplant, -PET to follow up tx response
HL treatment complications
- cardiac disease and hypothyroidism post XRT
- Pulmonary disease
- Infertility (2ndary to damage)
- secondary malignancy
What is the most common hematological cancer?
Non-hodgkin’s lymphoma (malignant proliferation of lymphoid cells of progenitor or mature b/t cells)
-distinguished from HL by proliferation of only malignant B-cell
Types of NHL neoplasms
B-cell
- Precoursor
- Mature (Hairy cell leukemia)
T-cell
- Precoursor
- Mature
Adult risk factors of NHL
- being older, male
- autoimmune disease
- HIV/AIDS
- other viruses
- h. pylori
- long term use of immunosuppressant drugs
- pesticides
- past treatment for hodgkin’s lymphoma or with radiation
NHL Clinical features
- Painless superficial LAD
- widespread disease
- constitutional symptoms uncommon
- cytopenia (anemia, neutro, thrombocytopenia) with bone marrow involvement
- abdominal signs: hepatosplenmegaly, retropertoneal and mesenteric involvement
- sore throat and obstructive apnea
- CNS involvement in HIV pts
What is essential for diagnosing NHL?
lymph node bx
What is the diagnostic process for NHL
- Biospy**
- CBC (normocytic, normochromic anemia, autoimmune hemolytic anemia, thrombocytopenia, neutropenia)
- Uric acid (elevated), LDH- poor prognosis, LFTs abnormal)
- CXR, CT chest, abdomen, pelvis
- gallium scan (monitor response to tx)
NHL Tx
- follow up frequently
- CD20 (Rituximab) (new biotherapy)
- autologous or allogeneic transplantation
- totaly nodal irradiation
-everyone gets chemo. Burkitt lymphoma is 90% chemo.
NHL
complications
prognosis
complications
- hypersplenism
- infection
- autoimmune hemolytic anemia and thrombocytopenia
- vascular obstructions
- tumor lysis syndrome (aggressive lymphoma)
Prognosis
-diffuse B cell lymphoma… 5 year survival (25-73%)
Which virus is a member of the herpes family, infects B cells and epithelial cells of the oropharynx, and causes a positive heterophil rxn?
EBV
What disease would you suspect if you saw a histologic section with numerous mfgs that have a “starry sky” appearance?
Burkitt lymphoma
NHL associated conditions
immunodeficiency (HIV) autoimmune disease (SLE) infections (EBV)
