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Lymphoid Malignancies
Hematology
45
Biology
11/30/2011

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Term
Lymphoid Malignancies
Definition
Leukemia within the tissues or the lymph system
Lymphomas
Develop in previously healthy people
Lymphadenopathies
Reactive Hyperplasia
Term
Causes of an increased risk for developing lymphoid malignancies
Definition
Altered immune function
Certain viruses and bacteria
Exposure to chemicals or toxins
Term
Lymphadenopathies
Definition
Enlarged lymph nodes
Benign or Malignant
Not necessarily a lymphoma
Term
Reactive Hyperplasia (RH)
Definition
Non-malignant lymphomas
4 Types - Follicular, Paracortical, Sinusoidal, and Mixed Pattern
Term
Follicular
Definition
Most common reactive hyperplasia
Seen in tonsils of children and adolescents
Reaction to infections
Term
Paracortical
Definition
Associated with virus reactions (Mono), drug reactions, and chronic skin disease
Reactive Hyperplasia Type
Term
Sinusoidal
Definition
Expanded subcapsular cortical medullary sinus in a lymph node
Reactive Hyperplasia Type
Term
Mixed Pattern
Definition
Seen in toxoplasma gandhii
Reactive Hyperplasia Type
Term
Low Grade Lymphoid Malignancies
Definition
Long, indolent course
Term
High Grade Lymphoid Malignancies
Definition
Clinically aggressive
Kill rapidly if not treated
Term
Ann Arbor Scheme
Definition
Suggests 4 stages of lymphomas
Term
Lymphoma Stage 1
Definition
One single node region
One single extralymphatic site
Condensed to one area
Term
Lymphoma Stage 2
Definition
2 or more lymph node regions of the same side of the diaphragm
Either lower body (below the diaphragm) or upper body (above the diaphragm)
Limited extralymphatic site
Term
Lymphoma Stage 3
Definition
Lymph node regions on both sides of the diaphragm, including the spleen
Limited contiguous extralymphatic site
Term
Lymphoma Stage 4
Definition
Multiple or disseminated
Involvement of 1 or more lymphatic organs or tissues
Term
Chronic Leukemic Lymphoid Malignancies
Definition
Grouped together because the malignant cells are mature lymphs
Primarily located in the blood and bone marrow
7 Types
Term
B-Cell Chronic Lymphocytic Leukemia
Definition
Chronic Leukemic Lymphoid Malignancy
Usually happens in older men
Usually aymptomatic
Lymphocytes are small with scanty cytoplasm
-Ginger Snaps
- >5x10^9
<10% prolymphocytes
Smudge Cells
CD5, CD19, CD23 with weak surface IgG
Anemia, Thrombocytopenia, and Neutropenia
Low grade
20% develop Richter's Transformation
Term
Prolymphocytic Leukemia (PLL)
Definition
Chronic Leukemia Lymphoid Malignancy
Aggressive leukemic disorder
2 Types - T-Cell and B-Cell
Term
T-Cell PLL
Definition
Symptoms include splenomegally, skin lesions, and lymphadenopathy
Increased lymphocyte count with varied appearance
CD2, CD5, CD7
Inv(14)q11q32
Anti-CD52 Monoclonal Antibody Therapy
Most patients live 7.5 months
Term
B-Cell PLL
Definition
Usually found in adult males
Increased lymphocytosis
>300 x 10^9
55% are prolymphocytes
-Large cells with moderate basophilic cytoplasm and moderately condensed chromatin and a single prominent nucleoli
Anemia
Decreased platelets
CD20, FMC-7 with strong surface immunoglobulin
Does not take well to treatment
Term
Hairy Cell Leukemia
Definition
Uncommon B-cell leukemia
Found in middle-aged people, mostly males
Lymphocytes have hairy projections on them and oval nuclei with fine chromatin
Pancytopenia
Massive splenomegally
TRAP positive
CD19, CD20, CD22, CD11c, CD25 with strong surface immunoglobulin
Bone marrow lymphocytes have a "Fried Egg" appearance
Dry Traps
Increased cytokines
Treatment is 2-chloro-deoxyadenosine, deoxycoformycine, a-interferons, or purine analogs
Term
Large Granular Lymphocytic Leukemia
Definition
2 Types: T-Cell and Natural Killer (NK)
Term
T-Cell Large Granular Lymphocytic Leukemia
Definition
Pancytopenia
Lymphocytes have abundant pale-staining cytoplasm with azurophilic granules and are positive for rheumatoid factor
CD2, CD3, CD8, CD16, CD56, CD57
The T-cell receptor is clonally rearranged
Indolent Course
80% of patients live 10 years
Term
NK Large Granular Lymphocytic Leukemia
Definition
More acute presentation
Very aggressive
Decreased WBC's and RBC's
CD2, CD3, CD4, CD8, CD16, CD56, CD57
Rapidly fatal
Term
Sezary's Syndrome
Definition
Malignant lymphocytes with regular, convoluted nuclear outlines and fine chromatin
Cutaneous T-cell leukemia
Slow progression
Localized treatment
Term
Sezary Cells
Definition
Malignant lymphocytes with regular, convoluted nuclear outlines and fine chromatin
Term
Hairy Cells
Definition
Lymphocytes with hairy projections on them, oval nucleus, and fine chromatin
Term
Circulating Lymphoma Cells
Definition
Result from Non-Hodgkin's Lymphoma
Irregular nuclear outlines with deep indentations
Seen in Follicular Lymphoma
aka Buttock Cells
aka Butt Cells
Term
Malignant Lymphomas
Definition
2 Types: Non-Hodgkin's and Hodgkin's
Term
Non-Hodgkin's
Definition
9 Types
-Small Lymphocytic Lymphoma
-Follicular
-Mantel Cell Lymphoma
-Mucosal Associated Lymphoid Tissue Lymphoma (MALT)
-Waldenstrom's Macroglobulinemia
-Burkitt's Lymphoma
-Lymphoblastic Lymphoma
-Peripheral T/NK-Cell Lymphoma
Term
Hodgkin's Lymphoma
Definition
2 Broad Categories:
-Nodular Lymphomas
-Classic Hodgkin's
Term
Small Lymphocytic Lymphoma (SLL)
Definition
Tissue form of CLL
Lymphadenopathy
10-year survival time
Small lymphoid cells with course chromatin, inconspicuous nucleoli, and scanty cytoplasm
Smudge cells
Bone marrow shows nodular, diffuse, or interstitial infiltrations of small lymphoid cells
WHO classifies this as a CLL
Term
Follicular
Definition
Originates from germal centers
Has numerous, closely-spaced follicles that replace the normal architecture
Medium-sized lymphoid cells with angular or indented nuclei
Found in people aged 50-60
Most common Non-Hodgkin's Lymphoma
Term
Mantel Cell Lymphoma (MCL)
Definition
Found in 60-year old men
Absence of the large cell component
Medium-sized lymphoid cells with irregular nuclear outlines
Main site of involvement is the lymph nodes
People live 3-5 years
Poor response to therapy
CD19, CD5, FMC7, slg
Term
Mucosal Associated Lymphoid Tissue Lymphoma (MALT)
Definition
Lympho-epithelial lesions infiltrate with neoplastic lymphocytes
Precede inflammatory disorders such as Helicobacter pylori, sjorins, and hashimotos
Go away when the inflammatory reactions are treated
3 Types: Nodal, Extranodal, and Splenic
Term
Waldenstrom's Macroglobulinemia
Definition
Lymphoid malignancy that causes an increased production of monoclonal IgM
Symptoms include lymphadenopathies, hepatomegally, splenomegally, and hyperviscous syndrome
No bone lesions
Treatment involves plasmaphoresis
Most people live 4 years
Term
Diffuse Large B-Cell Lymphoma (DLBCL)
Definition
Diffuse proliferation of large lymphoid cells that replace the lymph architecture
-Have single or multiple nucleoli
Usually found in 60-year olds
Localized and Aggressive
Sensitive to multi-agent chemotherapy
Term
Burkitt's Lymphoma
Definition
High grade
High incidence in Africa
Found outside Africa in people with compromised immune systems (eg: HIV)
Biopsy reveals a Starry Sky appearance
-Sky = Blue nucleoli of neoplastic lymphocytes
-Stars = Scattered pale-staining, chingable macrophages
t(8;14) distinguishes this from DLBCL
Treatment involves aggressive chemotherapy
May see epstein-barr
Term
Peripheral T/NK-Cell Lymphoma
Definition
Either have a mature T-cell or a mature NK cell
Associated with post-epstein-barr infections
Very aggressive
Chemotherapy can work
CD30
Seen in pediatric patients
Term
Nodular Lymphomas
Definition
Hodgkin's Lymphoma in which the B-cells are the neoplastic cells
Term
Classic Hodgkin's
Definition
Reed-Sternburg Cells
2+ nuclear lobes
Have inclusions that look like nucleoli
Have an area of nuclear clearing that looks like owl eyes
Term
Plasma Cell Neoplasms
Definition
Rarely involve lymph nodes
Secrete monoclonal immunoglobulin
3 Types: Multiple Myeloma, Plasmacytoma, Monoclonal Gammopathy of Undeteremined Significance (MGUS)
Term
Multiple Myeloma (MM)
Definition
Multi-focal
Can form multiple tumors and bone lesions throughout the body
Usually found in men about 65 years-old
Can be normal or abnormal plasma cells that contain Dutcher Bodies
Rouloux due to increased immunoglobulins
Bence Jones Proteins in the urine
M-Spike on the electrophoresis
Patients with 6 months without treatment
-3 years with it
-Longer with a bone marrow or stem cell transplant
Term
Plasmacytoma
Definition
Localized tumorous collection of monoclonal plasma cells
Develops into MM
Term
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Definition
Clonal serum immunoglobulin
Mild mirror of plasmacytoma
Will see a low level of serum monoclonal protein without neoplasms
Chance of this occurring increases with age
25% develop into MM