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HEM/ONC | Section 19: Non-Hodgkin Lymphomas
I. Non-Hodgkin Lymphoma: Epidemiology
A. A diverse group of tumors of lymphoid tissue
B. The mean age of onset varies with subtype
C. Grades
1. Low grade
2. Intermediate grade
3. High grade
4. Miscellaneous lymphomas
II. Non-Hodgkin Lymphoma: Pathophysiology
A. Malignant transformation of B or T lymphocytes
B. B cell lymphomas (85%)
C. T cell lymphomas (15%)
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III. Non-Hodgkin Lymphoma: Risk Factors
FIGURE 14.4.121 - Diffuse Large B-Cell Lymphoma
A. HIV/AIDS
B. Organ transplant recipients
C. Viral infections (EBV, HTLV-1)
D. Helicobacter pylori
E. Autoimmune diseases
“Burkitt lymphoma, H&E” bye uthman is licensed under CC BY 2.0
FIGURE 14.4.122 - Starry Sky Appearance in Burkitt
“Small Lymphocytic Lymphoma in Marrow” by euthman is licensed under CC BY 2.0
Lymphoma
FIGURE 14.4.120 - Small Lymphocytic Lymphoma in Bone
Marrow
IV. Non-Hodgkin Lymphoma: Diagnosis
A. Lymph node biopsy (> 1 cm)
B. Chest X-ray and CT scan
C. Bone marrow biopsy
D. Bone involvement: elevated ALP
E. Liver involvement: elevated ALP, LFTs, bilirubin
F. Marrow involvement: anemia, leukopenia,
thrombocytopenia
V. Non-Hodgkin Lymphoma: Treatment
A. Low-grade lymphomas: observation, chemotherapy,
and radiation
B. Intermediate or high-grade lymphomas: combination
chemotherapy and radiation
“File:SkinTumors-PC181146.jpg” by LWozniak&KWZielinski is licensed under CC BY-SA 3.0
1. High dose chemotherapy with “CHOP”:
Cyclophosphamide, Hydroxydaunomycin FIGURE 14.4.123 - Mycosis Fungoides
(doxorubicin), Oncovin (vincristine), and
Prednisone
C. Rituximab
D. Bone marrow transplantation
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Grade Type Key Features Prognosis
- Widespread lymph
- Closely related to CLL; more
- Small lymphocytic node involvement with
Low Grade common in elderly patients
lymphoma dissemination to liver, spleen,
- Indolent course
and bone marrow
- Most common form of NHL
- Mean age of onset is 55
- May transform into diffuse,
- Most patients with localized
large cell
disease can be cured with
- Follicular lymphoma - Associated with
radiotherapy
translocation: t(14;18)
- Median survival 10 years
- Indolent course
- Presents with painless,
peripheral lymphadenopathy
- Predominantly B-cell origin
- Middle-aged and elderly - 85% cure rate with CHOP
- Diffuse, large cell
Intermediate patients therapy
lymphoma
- Locally invasive; presents as
large extranodal mass
TABLE 14.4.14 - Low-Grade and Intermediate Non-Hodgkin Lymphomas
Grade Type Key Features Prognosis
- T-cell lymphoma
- Aggressive with rapid
- More common in children
High Grade - Lymphoblastic lymphoma spread, but may respond to
- May progress to T-ALL
chemotherapy
- B symptoms common
- B-cell lymphoma
- More common in children
- African (endemic): maxilla
- Poor prognosis unless
and jaw; associated with EBV
- Burkitt lymphoma aggressively treated with
infection
chemotherapy
- Sporadic: abdominal organs
- Associated with
translocation: t(8;14)
- T-cell lymphoma of the skin
- Eczematous skin lesions
- Prognosis depends on the
that progress to generalized
degree of dissemination
erythroderma
- Potentially curable with
Miscellaneous lymphomas - Mycosis fungoides - Cribriform shape of
radiation and topical
lymphocytes
chemotherapy if limited to
- Disseminates to lymph
the skin
nodes, blood, and other
organs
TABLE 14.4.15 - High-Grade and Miscellaneous Non-Hodgkin Lymphomas
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REVIEW QUESTIONS ?
1. What are the differences between the endemic and • The correct answer is option B.
sporadic variants of Burkitt lymphoma? Is it a B or a • This young boy with a past medical history of EBV
T-cell lymphoma?’ infection presents with intermittent vomiting and
an unintentional 12-kg weight loss over the past
• The African (endemic) variant most commonly
6 months.
involves the maxilla or jaw, whereas the
• His vital signs are significant for mild hypotension
American (sporadic) variant involves abdominal
with a blood pressure of 80/60 mmHg.
organs.
• This suggests vomiting-induced dehydration.
• Burkitt lymphoma is a B cell lymphoma.
• Dehydration is further supported by the findings
2. An oncologist prescribes cyclophosphamide, of sunken eyes and decreased skin turgor on
doxorubicin, vincristine, and prednisone for the physical exam.
treatment of a patient who has been recently • The patient also has hepatosplenomegaly.
diagnosed with a lymphoma. Is the patient’s • Stool culture, ova and parasite test, and fecal
pathology more likely to be Hodgkin’s lymphoma or fat studies are negative, making infection and
non-Hodgkin’s lymphoma? malabsorption unlikely.
• The abdominal ultrasound reveals a hypoechoic
• This patient most likely has non-Hodgkin mass in the duodenal wall.
lymphoma. • Biopsy of the mass shows small basophilic cells
• Cyclophosphamide, hydroxydaunomycin (also and evenly dispersed macrophages leading to a
known as doxorubicin), oncovin (also known starry sky appearance.
as vincristine), and prednisone make up the • These findings in a patient with a previous EBV
combination “CHOP” chemotherapy regimen infection are classic for Burkitt lymphoma.
used in intermediate and high-grade non-Hodgkin • Burkitt lymphoma can occur in the jaw as well
lymphomas. as the abdomen. Burkitt lymphoma of the jaw
is more common in African patients, while that
3. A 9-year-old boy is brought to the physician due to of the abdomen is more prevalent in the United
intermittent vomiting and an unintentional 12-kg States.
weight loss over the past 6 months. He has a past • Option A is incorrect because Hodgkin lymphoma
medical history of EBV infection. He was born at term is characterized by binuclear Reed-Sternberg
without any perinatal or postnatal complications. cells, which are absent on this patient’s biopsy.
His vital signs include temperature of 36 °C (96.8 • Option C is incorrect because mycosis fungoides
°F), blood pressure of 80/60 mmHg, heart rate of is a T-cell lymphoma that commonly presents as
91 beats/min, and respiratory rate of 15 breaths/ an eczematous skin rash. Additionally, the nuclei
min. The physical examination shows sunken eyes, of the lymphocytes have a cribriform shape in
decreased skin turgor, and hepatosplenomegaly. mycosis fungoides.
Stool culture, ova and parasite testing, and fecal • Option D is incorrect because follicular lymphoma
fat studies are negative. The abdominal ultrasound is characterized by a follicular arrangement of
shows a hypoechoic mass in the duodenal wall. lymphocytes rather than a starry sky appearance
Biopsy of the mass shows small basophilic cells and on the biopsy.
evenly dispersed macrophages with a starry sky
appearance. Which of the following is the most likely
diagnosis in this patient?
A. Hodgkin lymphoma
B. Burkitt lymphoma
C. Mycosis fungoides
D. Follicular lymphoma
