94 General Considerations of Lymphomas: Incidence Rates, Etiology, Diagnosis, Staging, and Primary Extranodal Disease Flashcards
Lymphoma is more common in (men, women)
Men
The disease is more common in men with incidence rates of 23.9 and 16.2 in men and women, respectively.
The highest reported incidence rate is in
Israel
It is lowest of all in Asia
There are more cases of NHL in Asia than in the United States.
Incidence of different subtypes of NHL in different countries and continents
Follicular lymphoma: United States and Europe
Extranodal lymphoma: Japan
Burkitt lymphoma: sub-Saharan Africa
T-cell leukemia/lymphoma: southwest Japan, the southeastern United States, northeastern South America, and the Caribbean basin
An exponential increase in incidence in NHL among men and women occurs with increasing age ECXEPT for:
Lymphoblastic lymphoma: most commonly in children
Burkitt lymphoma : 20- to 64-year-old age group
Primary mediastinal B-cell lymphoma: median age of 35 years
Occupations with association with lymphoma
Crop farming but not animal farming, women’s hairdressers, cleaners, spray painters, carpenters and textile workers
Environmental factors and lymphoma
Cigarette smoking: NHL, follicular lymphoma
BMI above 30 kg/m2: DLBCL
Radiation exposure
Surprisingly, some studies have suggested an inverse relationship between an individual’s exposure to ultraviolet light and lymphoma incidence (especially DLBCL)
Infectious Agent: HTLV1
C-type RNA tumor virus
An acquired retrovirus that is not related to other known animal retroviruses
Also leads to a neurologic disorder called tropical spastic paraparesis
Adult T-cell leukemia/lymphoma (ATLL)
Infectious Agent: Epstein-Barr Virus
DNA virus in the herpesvirus family
- Burkitt lymphoma
- Posttransplantation lymphoma
- HIV-associated lymphomas (immunodeficiency-related Burkitt lymphoma, primary CNS lymphoma, primary effusion lymphoma, the immunoblastic-plasmacytoid type of DLBCL, and oral cavity plasmablastic lymphoma)
- Extranodal NK/T-cell lymphoma, nasal type
EBV binds to the _______
CD21 antigen (also the receptor for the C3d component of complement) on B lymphocytes
A three-step process in the development of Burkitt lymphoma has been proposed:
(a) EBV initiates a polyclonal proliferation of B cells;
(b) malaria or other infections further stimulate the proliferating B-cells; and
(c) the transforming B cells incur specific reciprocal translocations of chromosome 8 with chromosome 2, 14, or 22, resulting in a clonal expansion of B lymphocytes
Infectious Agent: Human Herpesvirus-8
In the homosexual population (mainly in the United States and Europe), HHV-8 is principally transmitted during repeated sexual contacts, whereas in Africa, it is mainly transmitted from mother to child and among siblings.
Saliva seems to play a major role in HHV-8 transmission.
- Kaposi sarcoma
- Castleman disease
- Primary effusion lymphoma
- Posttransplantation primary effusion lymphoma
Infectious Agent: Hepatitis B
DLBCL and follicular lymphoma
Infectious Agent: Hepatitis C
Associated with immunopathologic reactions, such as cryoglobulinemia, and frequently, clonality of infected B lymphocytes
DLBCL
Marginal zone lymphoma (MZL)
Lymphoplasmacytic lymphoma
Infectious Agent: Helicobacter pylori
Major cause of peptic ulceration and gastric cancer
Marginal zone mucosa-associated lymphoid tissue (MALT) lymphomas of the stomach
Transformation of a MALT or de novo DLBCL
Infectious Agent: Chlamydophila psittaci
Ocular adnexal lymphomas
The majority of ocular adnexal lymphomas are extranodal MALT lymphomas
Treated with doxycycline
Infectious Agent:Campylobacter jejuni
Immunoproliferative disease of the small intestine
Infectious Agent:Borrelia burgdorferi
B-cell lymphoma of the skin
TRUE OR FALSE
With the exception of common variable immunodeficiency and the syndromes present in childhood, many of the inherited immune deficiencies are X-chromosome linked; as a result, males are affected more commonly than females.
TRUE
With the exception of common variable immunodeficiency and the syndromes present in childhood, many of the inherited immune deficiencies are X-chromosome linked; as a result, males are affected more commonly than females.
This germline predisposition syndrome does not have an immunodeficiency phenotype. Rather, it is a nonsyndromic familial cancer syndrome transmitting susceptibility to mutations in p53. The cancers that occur in these families include lymphoma.
Li-Fraumeni syndrome
There is increased incidence of NHL in patients with rheumatological diseases receiving _______________ inhibitors
Tumor necrosis factor (TNF) inhibitors
The OR is 1.93 (95% CI, 1.16–3.20), and it is possible that the risk is higher with the TNF fusion protein etanercept than it is with inhibitory monoclonal antibodes.
Autoimmune disoreders: Sjögren syndrome
Parotid gland MZL (1000-fold increased risk)
Autoimmune disoreders: Hashimoto thyroiditis
Thyroid MALT lymphoma
MZL
Autoimmune disoreders: Lupus
MZL and DLBCL
Autoimmune disorders: Autoimmune hemolytic anemia
DLBCL
Autoimmune disorders: celiac disease, inflammatory bowel disease, and eczema
T-cell lymphoma
TRUE OR FALSE
The presence of such “B symptoms” has unfavorable prognostic significance in HL and NHL
FALSE
The presence of such “B symptoms” has unfavorable prognostic significance in HL, but these symptoms do not have independent prognostic significance for NHL
Recurrence of these symptoms after treatment may herald disease relapse of HL
Fatigue, rash, pruritus, and alcohol-induced pain
TRUE OR FALSE
During the physical examination, all enlarged lymph nodes (>1.5 cm) should be recorded.
Involved nodes typically are nontender, firm, and rubbery.
TRUE
During the physical examination, all enlarged lymph nodes (>1.5 cm) should be recorded.
Involved nodes typically are nontender, firm, and rubbery.
The single most important test in a lymphoma patient
Biopsy of affected tissue
Excisional lymph node biopsy of one of the largest nodes available, or generous incisional biopsy of an extranodal site.
When is core biopsy indicated
When the only sites of disease involvement are deep in the thorax or pelvis
TRUE OR FALSE
Narrow-diameter core biopsies or fine-needle aspiration alone should never be used as the sole method of establishing the initial diagnosis of lymphoma.
TRUE
Narrow-diameter core biopsies or fine-needle aspiration alone should never be used as the sole method of establishing the initial diagnosis of lymphoma.
FDG-avid lymphomas
HL, DLBCL, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, anaplastic large-cell lymphoma, and most subtypes of PTCL
ABDE(man-tEl)F-HP
Non FDG-avid lymphomas
Uses Contrast-enhanced CT
MZLs, chronic lymphocytic leukemia/small lymphocytic lymphoma, lymphoplasmacytic lymphoma/Waldenström macroglobulinemia, angioimmunoblastic T-cell lymphoma, mycosis fungoides, and cutaneous B-cell lymphomas
Preferred imaging for disease affecting the brain
Magnetic resonance imaging should be performed as well as a lumbar puncture
PET/CT scans are of limited value for the detection of disease affecting the brain
TRUE OR FALSE
Although marrow aspiration and biopsy have been standard in lymphoma staging in the past, the high sensitivity of PET/CT for marrow involvement has rendered these procedures less essential for patients with any kind of lymphoma who have negative PET/CT imaging of the bones and marrow.
FALSE
Although marrow aspiration and biopsy have been standard in lymphoma staging in the past, the high sensitivity of PET/CT for marrow involvement has rendered these procedures less essential for patients with HL and DLBCL who have negative PET/CT imaging of the bones and marrow.
Required diagnostics for full staging of other subtypes of lymphoma (aside from HL and DLBCL)
Single 2.5-cm core biopsy with flow cytometry and cytogenetics
Important serum prognostic markers that should be assessed at baseline for most lymphomas
Lactate dehydrogenase and β2-microglobulin
“B symptoms”
- Fevers greater than 38.3 °C
- Drenching night sweats
- Unexplained weight loss of more than 10% of body mass over 6 months
Bulky disease for HL
A nodal mass of ≥10 cm, or greater than one-third of the transthoracic diameter at any level of thoracic vertebrae
Bulky disease for NHL
There is no consensus on the size of “bulk” for NHL with a suggestion that 6 cm may be optimal for follicular lymphoma.
Sizes between 6 cm and 10 cm have been advocated to define bulk for diffuse large B-cell lymphoma
Indicate metabolic activity in tumor sites less than in the mediastinal blood pool, signifying complete metabolic response and CR
Deauville scores of 1–2
1- No significant FDG uptake in tumor site(s)above background
2- FDG uptake in tumor site(s) less than that in the mediastinal blood pool
Indicate residual abnormal metabolic activity, representing treatment failure
Deauville scores of 4 or 5
4- FDG uptake in tumor site(s) moderately higher than in the liver
5- FDG uptake in tumor site(s) markedly higher than that in the liver and/or new FDG-avid lesions likely to be lymphoma
Indicates metabolic activity greater than the mediastinum but less than the liver, it is indeterminant
Deauville score of 3
Most patients with HL or DLBCL who have a Deauville score of 3 at the end of treatment have a good outcome, but careful follow-up of such patients is required.
Recommended follow-up for HL and DLBCL
Visits every 3 months during the first 2 years are recommended and then reduced to every 6 months for the next 3 years and annually thereafter
Recommendation for followup imaging
NCCN: Contrast-enhanced CT imaging should not be performed more frequently than every 6 months for 2 years after the end of therapy for DLBCL and HL and then be discontinued
Lugano IWG guidelines: Discourage any surveillance CT imaging for asymptomatic patients with HL and DLBCL in CR at the end of therapy
TRUE OR FALSE
There is general agreement that surveillance PET/CT imaging should not be carried out in asymptomatic patients in CR
TRUE
There is general agreement that surveillance PET/CT imaging should not be carried out in asymptomatic patients in CR
Patients with incurable histologies require long-term follow-up and are usually observed every _____________
3–6 months
Extranodal involvement that occurs as the only initial evidence of lymphoma after staging procedures
Primary extranodal lymphoma
The histopathology of primary extranodal lymphoma is usually
MZL of MALT or DLBCL
Usual treatment for Primary extranodal lymphoma
- Immuno-chemotherapy multidrug regimen
- Rituximab–cyclophosphamide, hydroxydaunorubicin, vincristine (Oncovin), and prednisone (RCHOP) or rituximab and bendamustine
Use of radiotherapy in Primary extranodal lymphoma
Selected cases of extranodal lymphoma, other stage I–II lymphomas and for consolidation therapy of bulky adenopathy
Primary CNS lymphomas are uncommon and almost always are of an aggressive histologic subtype, usually __________
DLBCL
There is a relatively high incidence of MYD88/CD79B mutations
Treatment for Primary CNS lymphomas
Multiagent chemotherapy is usually given in younger fit patients with drugs that cross the blood–brain barrier such as a combination of methotrexate, thiotepa, cytarabine, and rituximab (MATRix)
Consolidation is often given with either an autologous stem cell transplant or whole-brain radiotherapy
The most common orbital malignancy
Ophthalmic lymphoma
The most frequent subtype of Ophthalmic lymphoma
MZL of MALT
The most common site of ocular lesions
Periorbital soft tissues, particularly the conjunctival mucosal surfaces and the area surrounding the lacrimal gland
The therapy for orbital MZL
Radiotherapy
The therapy for orbital DLBCL
R-CHOP chemotherapy either alone or combined with local radiotherapy
Intraocular lymphomas are a rare presentation of lymphoma of the eye. Most cases are
DLBCL
Lymphoma arising in the lacrimal sac was usually DLBCL
The diagnosis of intraocular DLBCL is established by
Vitrectomy
The therapy for intraocular DLBCL
High-dose methotrexate-based chemotherapy such as MATRix with or without intrathecal treatment and/or whole-brain and eye radiotherapy
Localized NHL involving the nasal cavity and/or paranasal sinuses may be
DLBCL, T-cell lymphoma, or NK/T-cell lymphoma
The predominant site of involvement in T-cell and NK/T-cell lymphoma
Nasal cavity
Nasal NK/T lymphomas are typically treated with
L-asparaginase plus radiotherapy
Patients with primary sinus lymphoma usually have
DLBCL
The three main types of cutaneous B-cell lymphomas
Primary cutaneous marginal zone B-cell lymphoma
Primary cutaneous follicular center lymphoma
Primary cutaneous large B-cell lymphoma (leg type)
Cutaneous B-cell lymphomas that are indolent types with an excellent prognosis
Treated primarily with nonaggressive therapies, including simple excision, glucocorticoid injections, or local radiotherapy.
Primary cutaneous marginal zone B-cell and primary cutaneous follicle center lymphoma
A Cutaneous B-cell lymphoma with diffuse dermal infiltrate of neoplastic B-cells with extension to both the papillary dermis and the subcutaneous fat
Aggressive lymphoma that should be treated primarily with aggressive chemotherapy
Primary cutaneous large B-cell lymphoma (leg type)
Primary pulmonary lymphoma histopathology is usually
Marginal zone B-cell lymphoma of the MALT or DLBCL
A rare occurrence pulmonary lymphoma that may follow lung transplantation
Primary endobronchial lymphoma
Most common location of lymphomatous masses in the heart
Right atrium
Most cases of Primary cardiac lymphoma are
B-cell lymphoma
The most common form of extranodal lymphoma, accounting for one-third of cases
Gastrointestinal lymphoma
Most commonly involved site of the GI tract
Stomach
Followed by the small bowel, ileum, cecum, colon, and rectum
In the bowel, the small intestine, rectum, and colon may be involved, in that order of frequency
The intestinal location most often involved is the ileocecal region followed by small bowel, large bowel, and multiple intestinal sites.
Diagnosis of Gastrointestinal lymphoma is made by
Endoscopic biopsy
TRUE OR FALSE
GI Lymphomas: MALT lymphoma is common, but DLBCL also may arise de novo or may be found in the background of a MALT lymphoma.
If both subtypes of lymphoma are present, the treatment should be directed in treating MALT
FALSE
GI Lymphomas: MALT lymphoma is common, but DLBCL also may arise de novo or may be found in the background of a MALT lymphoma.
If both subtypes of lymphoma are present, the treatment should be directed at the large B-cell lymphoma
The intestinal location most often involved is
Ileocecal region
The most common disease location in the colon
Cecum
TRUE OR FALSE
Primary lymphoma of the testes typically presents as a painless enlargement of the testis in an older man
TRUE
Primary lymphoma of the testes typically presents as a painless enlargement of the testis in an older man
The histologic type usually of testicular lymphoma is
DLBCL
Relapses of testicular lymphoma usually occurs in
Either the CNS or contralateral testicle
The current international standard of care for testicular lymphoma
Orchiectomy, R-CHOP chemotherapy given every 21 days for six cycles, intrathecal methotrexate, and locoregional radiotherapy to the contralateral testicle
Characteristic of primary renal lymphoma:
Bilateral enlargement of the kidneys without obstruction and other organ or nodal involvement and absence of other causes of renal failure
When primary to the spleen, the lymphoma may be principally confined to the
Red pulp
Histopathology of Primary splenic lymphoma
DLBCL or MZL
Primary bone lymphoma may involve any bone but usually affects the
Long bones
Histopathology of breast lymphoma
DLBCL
B-cell lymphoma 2 (BCL-2) gene expression is frequently present in the tumor cells.
Breast lymphoma that may be the consequence of breast implants, especially those with a “rough” surface
T-cell anaplastic lymphoma of the breast
