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144 Cards in this Set
- Front
- Back
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Where do lymphocytes develop from?
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- Bone marrow (B cells)
- Thymus (T cells) |
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Where are mature lymphocytes located? How do they get there?
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- Peripheral lymphoid tissues: lymph nodes, spleen, tonsils, adenoids, and Peyer's patches
- Home to these locations by cytokines and chemokines |
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What are the most widely distributed and easily accessible lymphoid tissues?
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Lymph nodes
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What is the clinical significance of lymph nodes?
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- They are frequently examined for diagnostic purposes
- Trivial injuries and infections induce subtle changes - Significant infections produce nodal enlargement and sometimes leave residual scarring |
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What is the structure of lymph nodes?
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- Discrete, encapsulated structures
- Contain well-organized B-cell and T-cell zones - Richly invested w/ phagocytes and APCs |
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What morphological changes occur in lymph nodes after activation of the resident immune cells?
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- Within several days, primary follicles enlarge and transform into pale-staining germinal centers; this allows B cells to make high-affinity Abs
- Paracortical T-cell zones undergo hyperplasia |
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What are the types of Lymph Node inflammation?
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Acute and Chronic Non-specific Lymphadenitis
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What is the most common cause of acute lymphadenitis in the cervical region?
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Microbial drainage from infections of the teeth or tonsils
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What is the most common cause of acute lymphadenitis in the axillary or inguinal region?
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Microbial drainage from infections in the extremities
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What is the most common cause of acute lymphadenitis in the mesenteric region?
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Acute appendicitis (also can be caused by other self-limited infections that can mimic acute appendicitis)
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What is the most common cause of acute generalized lymphadenitis?
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Systemic viral infections (particularly in children) and bacteremia
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What is the morphological appearance of lymph nodes during acute non-specific lymphadenitis?
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- Swollen and engorged
- Grey-red - Large, reactive germinal centers contain mitotic figures - Macrophages contain particulate debris from dead bacteria or necrotic cells - Pyogenic organisms cause centers of follicles to necrose and they may contain pus |
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What happens to the lymphoid sinuses during acute non-specific lymphadenitis?
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- Endothelial cells lining the sinuses undergo hyperplasia
- Scattered neutrophils infiltrate about the follicles and accumulate within the lymphoid sinuses |
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What are the symptoms of Acute Non-specific Lymphadenitis?
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- Nodes are enlarged and painful
- If abscess formation is extensive, the nodes become fluctuant - Overlying skin is red - Sometimes, suppurative infections penetrate through the capsule of node and track to skin to produce draining sinuses - Healing of these lesions may lead to scarring |
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What happens if suppurative infections penetrate through the capsule of the node?
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- They may track to the skin to produce draining sinuses
- Healing of these lesions may lead to scarring |
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What are the types of Chronic Non-Specific Lymphadenitis?
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- Follicular Hyperplasia
- Paracortical Hyperplasia - Reticular Hyperplasia (aka sinus histiocytosis) |
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What is the morphological appearance of lymph nodes during chronic non-specific lymphadenitis with Follicular Hyperplasia?
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- Large oblong germinal centers (secondary follicles) - contain dark zone and light zone
- Surrounded by collar of small resting naive B cells (mantle zone) |
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What causes follicular hyperplasia in chronic non-specific lymphadenitis?
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Stimuli that activate humoral immune responses
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What is the appearance of germinal centers in Chronic Non-specific Lymphadenitis? What makes up these areas?
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- Large and oblong
- Surrounded by collar of small resting naive B cells = mantle zone Polarized into two distinct regions: - Dark zone: contains blast-like B cells (centroblasts) - Light zone: contains B cells w/ irregular or cleaved nuclear contours (centrocytes) In between the germinal B centers is a network of antigen-presenting follicular dendritic cells and macrophages ("tingible-body macrophages") that contain nuclear debris of B cells |
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Where are centroblasts found?
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Proliferating blast-like B cells found in the dark zone of the germinal center (secondary follicle) of lymph nodes during Chronic Non-specific Lymphadenitis
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Where are centrocytes found?
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B cells with irregular or cleaved nuclear controus found in the light zone of the germinal center (secondary follicle) of lymph nodes during Chronic Non-specific Lymphadenitis
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What is found between the germinal B centers in Chronic Non-specific Lymphadenitis? What are their characteristics?
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- Network of antigen-presenting follicular dendritic cells and macrophages (referred to as "tingible-body" macrophages)
- Contain nuclear debris of B cells - undergo apoptosis if they fail to produce an antibody w/ a high affinity for antigen |
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What happens to B cells that fail to produce antibody with a high affinity for antigen?
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Undergo apoptosis, phagocytosed by follicular dendritic cells and "tingible-body" macrophages
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What does this image show?
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- Reactive follicle and surrounding mantle zone
- The dark-staining mantle zone is more prominent adjacent to the germinal-center light zone in the left half of the follicle - The right half of the follicle consists of the dark zone |
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What are the causes of Follicular Hyperplasia in Chronic Non-Specific Lymphadenitis?
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- Rheumatoid arthritis
- Toxoplasmosis - Early stages of infection w/ HIV |
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What neoplasm is Follicular Hyperplasia similar to?
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Follicular Lymphoma
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What features favor a reactive (non-neoplastic) follicular hyperplasia?
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- Preservation of the lymph node architecture (including interfollicular T-cell zones and sinusoids)
- Marked variation in shape and size of follicles - Presence of frequent mitotic figures, phagocytic macrophages, and recognizable light and dark zones (which are absent in neoplastic follicles) |
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What is the morphological appearance of lymph nodes during chronic non-specific lymphadenitis with Paracortical Hyperplasia?
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- T-cell regions contain immunoblasts, activated T cells 3-4x the size of resting lymphocytes, have round nuclei, open chromatin, several prominent nucleoli, and moderate pale cytoplasm
- May efface B cell follicles - Often hypertrophy of sinusoidal and vascular endothelial cells |
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What causes Paracortical Hyperplasia?
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Stimuli that trigger T-cell mediated immune responses (eg, acute viral infections like infectious mononucleosis)
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What happens in Paracortical Hyperplasia?
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- Immunoblasts expand
- Expanded T-cell zone may efface B-cell follicles - Hypertrophy of sinusoidal and vascular endothelial cells - Sometimes infiltrating macrophages and eosinophils |
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What are immunoblasts? What do they look like? When do you see them?
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- Activated T cells 3-4x the size of resting lymphocytes
- Round nuclei - Open chromatin - Several prominent nucleoli - Moderate pale cytoplasm - Found in paracortical hyperplasia in T-cell regions |
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What needs to be done when immunoblasts are so numerous that they efface the B-cell follicles?
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Need to do special studies to exclude a lymphoid neoplasm
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What does Sinus Histiocytosis refer to?
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- AKA reticular hyperplasia
- Increase in number and size of cells that line lymphatic sinusoids |
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When is Sinus Histiocytosis more prominent?
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In lymph nodes draining cancers such as carcinoma of the breast
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What is the morphological appearance in Sinus Histiocytosis?
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- Lining lymphatic endothelial cells are markedly hypertrophied
- Macrophages are greatly increased in numbers - Results in expansion and distension of sinuses |
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What are the symptoms of Chronic Non-specific Lymphadenitis?
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Lymph nodes are non-tender because nodal enlargement occurs slowly over time
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Where is Chronic Non-specific Lymphadenitis common? Why?
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- Inguinal and axillary nodes
- Drain relatively large areas of body (extremities) and are challenged frequently |
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What is the difference between leukemias and lymphomas?
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- Leukemia: neoplasms in bone marrow and peripheral blood
- Lymphoma: neoplasms that arise as discrete tissue masses (these terms reflect the usual tissue distribution of each disease at presentation) |
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What are the sub-groups of lymphomas?
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- Hodgkin Lymphoma
- Non-Hodgkin Lymphomas (NHLs) - Plasma Cell Neoplasms |
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Where do Plasma Cell Neoplasms arise?
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- Arise in BM
- Only infrequently involve the lymph nodes or the peripheral blood |
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How common are Lymphomas?
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100,000 new cases diagnosed in US/year
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What is the typical clinical presentation of all Hodgkin lymphomas and 2/3 of non-Hodgkin lymphomas?
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- Enlarged non-tender lymph nodes (often >2cm)
- Hodgkin's Lymphoma often associated w/ fever related to release of inflammatory cytokines |
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What is the typical clinical presentation of the remaining 1/3 of non-Hodgkin lymphomas?
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Symptoms related to the involvement of extra-nodal sites (eg, skin, stomach, or brain)
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What is the typical clinical presentation of the lymphocytic leukemias?
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Signs and symptoms related to the suppression of normal hematopoiesis by tumor cells in the bone marrow
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What is the typical clinical presentation of Plasma Cell Neoplasms? Most common type?
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- Multiple Myeloma
- Causes bony destruction of the skeleton and often presents with pain d/t pathological fractures - Some plasma cell neoplasms cause symptoms via secretion of circulating factors (Eg, whole antibodies or Ig fragments) |
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What are the categories of Lymphoid Neoplasms?
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- I: Precursor B-Cell Neoplasms
- II: Peripheral B-Cell Neoplasms - III: Precursor T-Cell Neoplasms - IV: Peripheral T-Cell and NK-Cell Neoplasms - V: Hodgkin Lymphoma |
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What are the types of Class II Lymphoid Neoplasms?
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Peripheral B-Cell Neoplasms:
- Lymphoplasmacytic Lymphoma - Splenic and Nodal Marginal Zone Lymphomas - Extra-Nodal Marginal Zone Lymphoma - Mantle Cell Lymphoma - Follicular Lymphoma - Marginal Zone Lymphoma - Diffuse Large B-cell Lymphoma - Burkitt Lymphoma |
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What are the types of Class IV Lymphoid Neoplasms?
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Peripheral T-Cell and NK-Cell Neoplasms:
- Peripheral T-Cell Lymphoma, un-specified - Anaplastic Large-Cell Lymphoma - Extra-nodal NK/T-Cell Lymphoma |
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What are the types of Class V Hodgkin Lymphomas?
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Classical Sub-types:
- Nodular sclerosis - Mixed cellularity - Lymphocyte-rich - Lymphocyte-depletion Lymphocyte predominance |
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What is necessary for diagnosis of Lymphoid Neoplasia?
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Histologic examination of lymph nodes or other involved tissues is required for diagnosis although it can be suspected based on clinical features
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What are the features of all daughter cells derived from the malignant progenitor in lymphoid neoplasia? Why?
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Monoclonal population of lymphocytes:
- All share the same antigen receptor gene configuration and sequence - All synthesize identical antigen receptor proteins (Igs or T-cell receptors) - Because antigen receptor gene rearrangement precedes transformation |
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What are the features of daughter cells in normal immune responses (that distinguish them from daughter cells in lymphoid neoplasms)?
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Polyclonal populations of lymphocytes that express many different antigen receptors
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How can you distinguish lymphoid neoplasms from normal immune response cells?
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- Analyze antigen receptor genes and their protein products
- Distinguish reactive (polyclonal) and malignant (monoclonal) lymphoid proliferations |
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What is the basis of detecting if there are any residual malignant lymphoid cells after therapy?
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Each antigen receptor gene rearrangement produces a unique DNA sequence that constitutes a highly specific clonal marker (analyze antigen receptor genes and protein products to check for residual malignant lymphoid cells after therapy)
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What kind of cells cause lymphoid neoplasms?
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- 85-90% are of B-cell origin
- Most of remainder are of T-cell origin - Rarely of NK-cell origin - Most lymphoid neoplasms resemble some recognizable stage of B- or T-cell differentiation |
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What abnormalities are lymphoid neoplasms associated with? Implications?
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Immune Abnormalities:
- Loss of protective immunity (susceptibility to infection) - Breakdown of tolerance (auto-immunity) - Individuals w/ inherited or acquired immunodeficiency are at risk of developing certain lymphoid neoplasms, particularly those caused by oncogenic viruses (eg, EBV) |
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How do neoplastic B and T cells relate to normal B and T cells?
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- Neoplastic B and T cells tend to mimic the behavior of their normal compartments
- Eg, they home to certain tissue sites, leading to characteristic patterns of involvement |
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Where do follicular lymphomas occur?
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Germinal centers of lymph nodes (B cells)
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Where do cutaneous T cell lymphomas occur?
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Skin
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What governs the homing of the neoplastic lymphoid cells to certain areas of the body?
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- Adhesion molecules and chemokine receptors help home cells to normal locations
- They can also recirculate through the lymphatics and peripheral blood to distant sites (as a result most are widely disseminated at time of diagnosis) |
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Which types of lymphoid neoplasms are not as widely disseminated at time of diagnosis?
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- Hodgkin Lymphomas: sometimes restricted to one group of lymph nodes
- Marginal Zone B-Cell Lymphomas: often restricted to sites of chronic inflammation |
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How does Hodgkin Lymphoma spread? Vs. Non-Hodgkin Lymphoma? How does this affect staging information?
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- HL: spreads in orderly fashion
- NHL: spread widely early in course in less predictable fashion - While lymphoma staging provides general prognostic information, it is more useful for Hodgkin lymphoma (d/t predictable spread) |
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What are the neoplasms of Mature B Cells?
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- Burkitt Lymphoma
- Diffuse Large B-Cell Lymphoma - Extra-Nodal Marginal Zone Lymphoma - Follicular Lymphoma - Mantle Cell Lymphoma |
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Burkitt Lymphoma:
- Cell of origin - Genotype - Clinical features |
- Originates in germinal-center B cells
- t(8;14) - c-MYC and Ig loci - Some are EBV-associated - Affect adolescents or young adults with extranodal masses - Uncommonly presents as "leukemia" - Aggressive |
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Diffuse Large B-Cell Lymphoma:
- Cell of origin - Genotype - Clinical features |
- Originates in germinal-center or post-germinal-center B cells
- Diverse chromosomal arrangements, most often BCL6 (30%), BCL2 (10%), or c-MYC (5%) - All ages affect, more commonly in adults - Appears as rapidly growing mass - 30% extra-nodal - Aggressive |
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Extra-Nodal Marginal Zone Lymphoma:
- Cell of origin - Genotype - Clinical features |
- Originates in memory B cells
- t(11;18) - MALT1-IAP2 fusion gene - t(1;14) - BCL10-IgH fusion gene - t(14;18) - MALT1-IgH fusion gene - Arises at extra-nodal sites in adults w/ chronic inflammatory diseases - May remain localized - Indolent |
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Follicular Lymphoma:
- Cell of origin - Genotype - Clinical features |
- Originates in germinal-center B cells
- t(14;18) creates BCL2-IgH fusion gene - Older adults with generalized lymphadenopathy - BM involvement - Indolent |
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Mantle Cell Lymphoma:
- Cell of origin - Genotype - Clinical features |
- Originates in naive B cells
- t(11;14) creates CyclinD1-IgH fusion gene - Older males w/ disseminated disease - Moderately aggressive |
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Which type of Lymphoma presents in adolescents or young adults with aggressive extra-nodal masses? Cell of origin? Genotype?
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Burkitt Lymphoma
- Originates in germinal-center B cells - t(8;14) - c-MYC and Ig loci - Can be associated w/ EBV |
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Which type of Lymphoma presents in all ages (especially adults) with aggressive, rapidly growing mass (30% extranodal)? Cell of origin? Genotype?
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Diffuse Large B-Cell Lymphoma
- Originates in germinal-center B cells or post-germinal-center B cells - Diverse chromosomal rearrangements, most often BCL6 (30%), BCL2 (10%), or c-MYC (5%) |
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Which type of Lymphoma presents with indolent, localized, extra-nodal lesions in adults with chronic inflammatory diseases? Cell of origin? Genotype?
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Extra-Nodal Marginal Zone Lymphoma:
- Originates in memory B cells - t(11;18) - MALT1-IAP2 fusion gene - t(1;14) - BCL10-IgH fusion gene - t(14;18) - MALT1-IgH fusion gene |
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Which type of Lymphoma presents with indolent, generalized lymphadenopathy in older adults and involves the BM? Cell of origin? Genotype?
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Follicular Lymphoma
- Originates in germinal-center B cells - t(14;18) creates BCL2-IgH fusion gene |
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Which type of Lymphoma presents with moderately aggressive, disseminated disease in older males? Cell of origin? Genotype?
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Mantle Cell Lymphoma
- Originates in naive B cells - t(11;14) creates CyclinD1-IgH fusion gene |
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What are the neoplasms of Mature T or NK Cells?
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- Peripheral T-Cell Lymphoma, Unspecified
- Anaplastic Large-Cell Lymphoma - Extranodal NK/T-Cell Lymphoma |
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Peripheral T-Cell Lymphoma, Unspecified:
- Cell of origin - Genotype - Clinical features |
- Originates in Helper or Cytotoxic T cells
- No specific chromosomal abnormality - Mainly older adults - Usually presents w/ lymphadenopathy - Aggressive |
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Anaplastic Large-Cell Lymphoma:
- Cell of origin - Genotype - Clinical features |
- Originates in cytotoxic T cells
- Rearrangements of ALK - Children and young adults - Usually with lymph node and soft-tissue disease - Aggressive |
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Which type of Lymphoma presents with aggressive lymphadenopathy mainly in older adults? Cell of origin? Genotype?
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Peripheral T-Cell Lymphoma, Unspecified:
- Originates in Helper or Cytotoxic T cells - No specific chromosomal abnormality |
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Which type of Lymphoma presents with aggressive lymph node and soft tissue disease in children and young adults? Cell of origin? Genotype?
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Anaplastic Large-Cell Lymphoma
- Originates in cytotoxic T cells - Rearrangements of ALK |
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Extra-Nodal NK/T-Cell Lymphoma:
- Cell of origin - Genotype - Clinical features |
- Originates in NK cells (common) or Cytotoxic T cells (rare)
- EBV associated - No specific chromosomal abnormality - Adults with destructive extranodal masses - Most commonly sinunasal - Aggressive |
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Which type of Lymphoma presents in adults with destructive, aggressive extra-nodal masses, most commonly sinonasal? Cell of origin? Genotype?
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Extranodal NK/T-Cell Lymphoma
- Originates in NK cells (common) or Cytotoxic T cells (rare) - EBV associated - No specific chromosomal abnormality |
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What is the most common form of indolent Non-Hodgkin Lymphoma in US?
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Follicular Lymphoma
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What kind of cells does Follicular Lymphoma arise from? What do they express on their surface?
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- Germinal center B cells
- Express CD19, CD20, and CD10 |
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What genetic abnormality is Follicular Lymphoma associated with? Implications?
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- t(14;18) creating BCL2-IgH fusion gene
- Leads to over-expression of BCL2 - BCL2 antagonizes apoptosis and promotes survival of follicular lymphoma cells - Normally, germinal centers contain numerous B cells undergoing apoptosis, therefore this is bad |
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What are the morphological characteristics of Follicular Lymphoma?
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- Centrocytes and centroblasts are predominant cell types
- Centrocytes (irregular w/ cleaved nuclear contours) predominate - BM involvement in 85% of cases, takes form of paratrabecular lymphoid aggregates |
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What are the symptoms of Follicular Lymphoma? Treatment prognosis?
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- Indolent course
- Incurable - Median survival 7-9 years (not improved by aggressive therapy) |
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How do you treat Follicular Lymphoma?
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- Low-dose chemotherapy or immunotherapy (eg, anti-CD20 antibody) when they become symptomatic
- Usually is an indolent disease and therapy does not improve survival time |
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What is the most common form of Non-Hodgkin Lymphoma in US?
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Diffuse Large B-Cell Lymphoma (DLBCL)
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What are the morphological characteristics of Diffuse Large B-Cell Lymphoma (DLBCL)?
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- Large cell size (4-5x diameter of small lymphocyte)
- Open chromatin and prominent nucleoli - Diffuse pattern of growth |
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What are the clinical features / prognosis of Diffuse Large B-Cell Lymphoma (DLBCL)?
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- Rapidly enlarging mass at nodal or extra-nodal site (can arise anywhere in body)
- Aggressive tumors - Fatal without treatment |
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What are the types of Burkitt Lymphomas?
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- African (endemic) Burkitt Lymphoma
- Sporadic (non-endemic) Burkitt Lymphoma - Subset of aggressive lymphomas occurring in individuals infected w/ HIV |
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What are the morphological characteristics of Burkitt Lymphoma?
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- Diffuse infiltrate of intermediate-sized lymphoid cells
- High mitotic index - Contains numerous apoptotic cells * Starry sky pattern d/t macrophages being surrounded by abundant clear cytoplasm |
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What kind of cells are affected by Burkitt Lymphoma? What surface markers do they have?
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- Mature germinal center B cells
- Express IgM, CD19, CD20, CD10, and BCL6 * Almost always fail to express anti-apoptotic protein BCL2 |
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What is the genetic abnormality in Burkitt Lymphoma? Implications?
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- Translocations of the c-MYC gene on chromosome 8
- Usually with IgH locus, t(8;14) - May also be t(2;8) with Igκ of t(8;22) with γ ligh chain loci - These translocations all drive c-MYC (oncogene) expression |
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What is a feature of essentially all endemic Burkitt Lymphomas, 25% of HIV-associated Burkitt Lymphomas, and 15-20% of sporadic cases of Burkitt Lymphoma?
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Latent infections with EBV
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How common are latent infections of EBV in the types of Burkitt Lymphoma?
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- ~100% of endemic tumors
- ~25% of HIV-associated tumors - ~15-20% of sporadic tumors |
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Who is affected by Burkitt Lymphoma?
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Children or young adults
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How does endemic Burkitt Lymphoma present?
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- Mass involving the mandible
- Shows unusual predilection for involvement of abdominal viscera, particularly the kidneys, ovaries, and adrenal glands - BM and PB involvement is uncommon - Very aggressive but responds well to chemotherapy |
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How does sporadic Burkitt Lymphoma present?
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- Most often appears as a mass involving the ileocecum and peritoneum
- BM and PB involvement is uncommon - Very aggressive but responds well to chemotherapy |
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What happens to the neoplastic cells in Lymphplasmacytic Lymphoma?
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- Undergo differentiation to plasma cells
- Secrete monoclonal IgM, often in amounts to cause a hyperviscosity syndrome known as Waldenström macroglobulinemia |
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What type of lymphoma is associated with Waldenström macroglobulinemia? What happens in this?
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- Lymphoplasmactic Lymphoma
- Differentiate into plasma cells that secrete monoclonal IgM that causes a hyperviscosity syndrome |
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What is the morphological presentation of Lymphoplasmactic Lymphoma?
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Marrow contains diffuse sparse-to-heavy infiltrate of lymphocytes, plasma cells, and plasmacytoid lymphocytes in varying proportions
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How do you treat Lymphoplasmactic Lymphoma?
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Plasmapheresis to alleviate the hyperviscosity and hemolysis caused by the high IgM levels
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What kind of lymphoma has tumor cells that resemble the normal mantle zone B cells that surround germinal centers?
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Mantle Cell Lymphoma
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What are the morphological features of Mantle Cell Lymphoma?
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- Majority have generalized lymphadenopathy filled with cells that resemble the normal mantle zone B cells that surround germinal centers
- 20-40% have peripheral blood involvement |
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What is the genetic abnormality in Mantle Cell Lymphoma? Implications?
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- t(11;14) involves IgH locus (14) and CyclinD1 locus (11)
- Causes CyclinD1 over-expression - Promotes G1- to S-phase progression during cell cycle |
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What do the naive B cells express in Mantle Cell Lymphoma?
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- CD19
- CD20 - Moderately high levels of surface Ig (usually IgM and IgD w/ κ or γ light chain) - Usually CD5+ and CD23- (which helps distinguish it from CLL/SLL |
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How can you distinguish Mantle Cell Lymphoma from CLL/SLL?
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Mantle Cell Lymphoma is CD5+ and CD23-
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What is the prognosis for Mantle Cell Lymphoma?
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Poor - median survival is only 3-4 years
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Where do Marginal Zone Lymphomas arise?
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- Lymph nodes
- Spleen - Other extra-nodal tissues (MALT) |
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What kind of cells are affected in Marginal Zone Lymphoma?
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Memory B-cells that often show evidence of somatic hyper-mutation
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What did Marginal Zone Lymphomas at extra-nodal sites used to be called?
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Maltomas (mucosa-associated lymphoid tumors)
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What are the features of Marginal Zone Lymphomas at extra-nodal sites?
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- Often arise within tissues involved by chronic inflammatory disorders of auto-immune or infectious etiology (eg, salivary glands in Sjögren disease, thyroid gland in Hashimoto thyroiditis, and stomach in Helicobacter gastritis)
- Remain localized for prolonged periods - May regress if inciting agent (eg, H. pylori) is eradicated |
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Which type of lymphomas often arise within tissues involved by chronic inflammatory disorders of auto-immune or infectious etiology (eg, salivary glands in Sjögren disease, thyroid gland in Hashimoto thyroiditis, and stomach in Helicobacter gastritis)?
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Marginal Zone Lymphomas
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Which type of lymphomas are more common in Asia / Far East as opposed to US and Europe?
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- Peripheral T-cell tumors: 5-10% of NHLs in US and Europe, but more common in Asia
- NK cell tumors: rare in West but more common in Far East |
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What do Peripheral T-Cell Lymphoma, Unspecified do to lymph nodes? Types of cells?
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- Efface lymph nodes diffusely
- Typically composed of pleomorphic mixture of variably sized malignant, mature T cells |
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How do patients with Peripheral T-Cell Lymphoma, Unspecified present?
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- Generalized lymphadenopathy
- Sometimes eosinophilia, pruritus, fever, and weight loss |
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What is the prognosis of Peripheral T-Cell Lymphoma, Unspecified?
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Significantly worse prognosis than comparably aggressive mature B-cell neoplasms
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What genetic abnormality is associated with Anaplastic Large-Cell Lymphoma? Implications?
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Rearrangements in ALK gene on chromosome 2p23
- Breaks the ALK locus and leads to formation of chimeric genes - Encode ALK fusion proteins, constitutively active tyrosine kinases, which trigger a number of signaling pathways including JAK/STAT pathway |
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What are the morphological characteristics of Anaplastic Large-Cell Lymphoma?
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- Large anaplastic cells
- Some contain hallmark cells (horse-shoe shaped nuclei and voluminous cytoplasm) |
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Who is usually affected by Anaplastic Large-Cell Lymphoma? Prognosis?
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- Children or young adults
- Very good prognosis (unlike other aggressive peripheral T cell neoplasms) |
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What kind of lymphoma presents most commonly as a destructive nasopharyngeal mass?
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Extra-Nodal NK/T-Cell Lymphoma
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What is the most common presentation of Extra-Nodal NK/T-Cell Lymphoma?
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- Destructive nasopharyngeal mass
- Leads to extensive ischemic necrosis |
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What is Extra-Nodal NK/T-Cell Lymphoma associated with?
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EBV
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What is the prognosis of Extra-Nodal NK/T-Cell Lymphoma?
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- Highly aggressive neoplasm, responds well to radiation but not to chemotherapy
- Poor prognosis in advanced disease |
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Which type of lymphomas are characterized by the presence of a tumor giant cell, the Reed-Sternberg Cell?
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Hodgkin Lymphoma
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What kind of cell is this? What is it characteristic of?
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- Reed-Sternberg (RS) Cell
- Characteristic of Hodgkin Lymphoma |
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What is the distribution of cancer cells in Hodgkin Lymphoma?
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Typically spreads in a step-wise fashion to anatomically contiguous nodes
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What is the origin of cells affected by Hodgkin Lymphoma?
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B cell origin
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What are the morphological characteristics of Hodgkin Lymphoma? What cell surface markers do they have?
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Reed-Sternberg (RS) cells (owl eye appearance) - express CD15 and CD30 (but not CD45)
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What is the most common form of Hodgkin Lymphoma?
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Nodular Sclerosis Hodgkin Lymphoma
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What lymph nodes are most commonly affected by Nodular Sclerosis Hodgkin Lymphoma? Who gets it? Prognosis?
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- Lower cervical, supraclavicular, and mediastinal lymph nodes
- Most are adolescents or young adults - Excellent prognosis |
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What are the cellular characteristics of Nodular Sclerosis Hodgkin Lymphoma?
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Presence of a variant of the Reed-Sternberg cells - Lacunar Cells (single multi-lobate nucleus that lies in empty space where cytoplasm tore away = lacunae)
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What are the morphological characteristics of Nodular Sclerosis Hodgkin Lymphoma?
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Collagen bands that divide the lymphoid tissue into nodules
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What is the most common type of Hodgkin Lymphoma in patients over 50 years of age (25% overall)?
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Mixed-Cellularity Hodgkin Lymphoma
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Which type of Hodgkin Lymphoma expresses B cell markers (CD20) but usually fails to express CD15 and CD30?
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Lymphocyte-Predominance Hodgkin Lymphoma
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What are the clinical differences between Hodgkin and Non-Hodgkin Lymphomas in regards to the location?
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- HL: more often localized to a single axial group of nodes (cervical, mediastinal, para-aortic)
- NHL: more frequent involvement of multiple peripheral nodes |
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What are the clinical differences between Hodgkin and Non-Hodgkin Lymphomas in regards to the spread?
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- HL: orderly spread by contiguity
- NHL: Non-contiguous spread |
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What are the clinical differences between Hodgkin and Non-Hodgkin Lymphomas in regards to the Mesenteric nodes and Waldeyer ring?
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- HL: rarely involved
- NHL: commonly involved |
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What are the clinical differences between Hodgkin and Non-Hodgkin Lymphomas in regards to extranodal involvement?
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- HL: uncommon
- NHL: common |
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How do you stage Hodgkin and Non-Hodgkin Lymphoma?
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- I: Involvement of a single lymph node region (I) or involvement of a single extralymphatic organ or tissue (IE)
- II: Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or with involvement of limited contiguous extralymphatic organs or tissue (IIE) - III: Involvement of lymph node regions on both sides of the diaphragm (III), which may include the spleen (IIIS), limited contiguous extralymphatic organ or site (IIIE), or both (IIIES) - IV: Multiple or disseminated foci of involvement of one or more extralymphatic organs or tissues with or without lymphatic involvement |
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What are the characteristics of stage I Hodgkin and Non-Hodgkin Lymphoma?
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Involvement of a single lymph node region (I) or involvement of a single extralymphatic organ or tissue (IE)
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What are the characteristics of stage II Hodgkin and Non-Hodgkin Lymphoma?
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Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or with involvement of limited contiguous extralymphatic organs or tissue (IIE)
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What are the characteristics of stage III Hodgkin and Non-Hodgkin Lymphoma?
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Involvement of lymph node regions on both sides of the diaphragm (III), which may include the spleen (IIIS), limited contiguous extralymphatic organ or site (IIIE), or both (IIIES)
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What are the characteristics of stage IV Hodgkin and Non-Hodgkin Lymphoma?
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Multiple or disseminated foci of involvement of one or more extralymphatic organs or tissues with or without lymphatic involvement
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