Lymphoma Flashcards
Lymphomas are categorized into two major types:
- Hodgkin Lymphoma (HL)
- Non-Hodgkin Lymphoma (NHL)
is a unique type of lymphoma that is defined by the presence of Reed-Sternberg cells, which are large, abnormal B cells with two or more nuclei that resemble “owl eyes” when viewed under a microscope.
Hodgkin lymphoma
is a diverse group of lymphomas that lack Reed-Sternberg cells.
It can originate from either B-cells or T-cells and tends to be more aggressive or indolent (slow-growing) depending on the subtype.
Non-Hodgkin lymphoma
• Cell of origin (B-cell vs. T-cell/NK-cell).
• Aggressiveness (indolent vs. aggressive).
• Genetic mutations and molecular markers.
NHL
NON-HODGKIN’S LYMPHOMA
Among the T-cell lymphomas,______ is a unique and highly aggressive subtype strongly associated with Human T-cell Lymphotropic Virus Type 1 (HTLV-1) infection.
Adult T-Cell Leukemia/Lymphoma (ATLL)
NON-HODGKIN’S LYMPHOMA
is a post-thymic neoplastic disorder of mature T-cells that results from chronic HTLV-1 infection.
It primarily affects adults in endemic regions such as Japan, the Caribbean, South America, and Africa.
ATLL
NON-HODGKIN’S LYMPHOMA
is a retrovirus that infects CD4+ T cells, leading to genetic mutations and uncontrolled cell proliferation over decades.
HTLV-1 (Human T-Cell Lymphotropic Virus Type 1)
NON-HODGKIN’S LYMPHOMA
Only 5% of HTLV-1 carriers develop_____ during their lifetime, usually after long-term viral latency (20–50 years post-infection)
ATLL
NON-HODGKIN’S LYMPHOMA
Modes of HTLV-1 Transmission
HTLV-1 is transmitted through:
- Transplacental transmission (mother-to-child).
- Breastfeeding (the most common route).
- Blood transfusion (infected lymphocytes in transfused blood).
- Sexual intercourse (unprotected sex with an infected partner).
NON-HODGKIN’S LYMPHOMA
ATLL presents in four distinct subtypes, each with varying severity and prognosis:
Acute ATLL
Lymphomatous ATLL
Chronic ATLL
Smoldering ATLL
(Most common) ATLL
• Aggressive, extensive extranodal involvement of peripheral blood and skin
Acute ATLL
NON-HODGKIN’S LYMPHOMA
• Leukemic phase
• Marked leukocytosis
• “Flower cell”
ATLL
NON-HODGKIN’S LYMPHOMA
• CD3+, CD4+, CD25+, CCR4+
• CD7-, CD8-
ATLL
NON-HODGKIN’S LYMPHOMA
Treatment
- Acute subtype: poor prognosis
• Median survival 8 mos
- Indolent forms: better prognosis (disease is progressing owly)
- Interferon-alpha, azidothymidine (AZT)
- Anti-CCR4 Ab (mogamulizumab)
- HSCT
Adult T-cell Leukemia/Lymphoma (ATLL)
NON-HODGKIN’S LYMPHOMA
- Aggressive malignancy of mature B cells
• Rapid tumor growth
Burkitt Lymphoma (BL)
NON-HODGKIN’S LYMPHOMA
BL (3 subtypes)
Endemic
Sporadic
Immunodeficiency-associated
NON-HODGKIN’S LYMPHOMA
BL
• children; equatorial Africa; extranodal involvement (orbit, mandible);
(+) EBV genome
Endemic
NON-HODGKIN’S LYMPHOMA
BL
• western regions;
abdominal disease;
BM infiltration in 70%; CNS involvement in 1/3 of patients
Sporadic
NON-HODGKIN’S LYMPHOMA
BL
• HIV, post-transplantation, congenital immunodeficiency;
primarily involves the blood and bone marrow
Immunodeficiency-associated
NON-HODGKIN’S LYMPHOMA
•______ phase
• BL cells with finely clumped chromatin, deeply basophilic cytoplasm, with distinct vacuoles
- BM/LN biopsy: “starry sky” pattern
Leukemic phase Burkitt Lymphoma
NON-HODGKIN’S LYMPHOMA
• Mature B cell markers, IgD+,
LCR+, CD10+
• CD34-, TdT-
BL
NON-HODGKIN’S LYMPHOMA
Treatment
• Highly responsive to chemotherapy
• Curable in >90% with early disease, 60-80% with advanced disease
• Risk for tumor lysis syndrome
Burkitt Lymphoma (BL)
NON-HODGKIN’S LYMPHOMA
• Disease of middle aged to older adults
• Generally incurable
• Follicular Lymphoma (FL)
NON-HODGKIN’S LYMPHOMA
- Neoplastic disorder of germinal B cells
• Second most common NHL
• Follicular Lymphoma (FL)
