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DD & BL Unit 2 > Lymphomas & Plasma Cell Neoplasms > Flashcards

Lymphomas & Plasma Cell Neoplasms Flashcards

1
Q

BCL6 and CD10

A

Markers of germinal B cells (and derived lymphomas)

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2
Q

CD20

A

B-cell specific marker positive in both mantle zone and germinal center B cells

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3
Q

CD3

A

T-cell specific marker positive in paracortical T cells

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4
Q

Chronic lymphocytic leukemia (CLL) and Small Lymphocytic Lymphoma (SLL) - Definition

A

Both characterized by lymphocytosis of monoclonal B cells with mature immunophenotype

Peripheral lymphocytosis > 5 x 10^9 / L over 3 months

CLL is a liquid presentation originating in the bone marrow

SLL is a solid presentation usually presenting in a lymph node

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5
Q

CLL & SLL - Immunophenotype

A

Positive for B cell markers CD19, CD23 and T cell marker CD5

Weakly positive for CD20 and sIg (mature B cell markers)

Negative for CD10 (expressed on germinal B cells)

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6
Q

CLL & SLL - Epidemiology

A

CLL is the most common leukemia in the Western world and accounts for 30% of all leukemias

SLL accounts for 7% of Non-Hodgkin Lymphoma

Median age of diagnosis - 65 years, most elderly patients with CLL are asymptomatic

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7
Q

Follicular Lymphoma - Epidemiology

A

40% of adult lymphomas in the US; 20% worldwide

Median age of diagnosis - 60 years

Stages III and IV are common at diagnosis; however, patients are often asymptomatic except for lymphadenopathy

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8
Q

Follicular Lymphoma - Immunophenotype, and Cytogenetics

A

Lymphoma of germinal B cell origin

B cell marker positive - CD19, CD20
Germinal B cell marker positive - BCL6, CD10
Positive for BCL2, which is negative in normal, reactive germinal center B cells

t(14;18) causes constitutive expression of BCL2 under the IGH promotor; BCL2 functions to suppress apoptosis and is normally down-regulated in normal germinal center B cells, allowing cells to die during selection

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9
Q

Mantle Cell Lymphoma - Epidemiology

A

Comprises 3-10% of Non Hodgkin Lymphoma

Median age of diagnosis = 60 years

Presentation in stages III and IV with hepatosplenomegaly and marrow involvement is common; moderately aggressive disease

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10
Q

Mantle Cell Lymphoma - Immunophenotype & Cytogenetics

A

B cell marker positive (CD19, CD20)
Positive for CD5 but negative for CD23 (differentiates from CLL/SLL)
Negative for germinal center B-cell markers (CD10, BCL6)
Positive for cyclin D1 (BCL1) - specific for Mantle Cell Lymphoma

t(11;14) induces constitutive activation of the BCL1 gene under the IGH promotoer, leading to over-expression of cyclin D1 and unchecked progression through the cell cycle

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11
Q

Burkitt’s Lymphoma (BL) - 3 Types

A

Endemic BL - typically seen throughout equatorial Africa in children 4-7 years old; often presents in the jaw or abdomen; 95% EBV+

Sporadic BL - typically seen in children or young adults; often presents in the ileocecal area; 30% EBV +

Immunodeficiency-associated BL - primarily seen in HIV+ patients; 25-40% EBV+

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12
Q

Burkitt’s Lymphoma - Immunophenotype & Cytogenetics

A

Positive for B cell markers (CD19, CD20)
Positive for germinal B-cell markers (CD10, BCL6)
Positive for MYC

t(8;14) causes constitutive activation of the MYC transcription factor under the IGH promoter

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13
Q

Plasma Cell Neoplasms - Definition

A

A clonal proliferation of plasma cells that secrete a singe class of Ig or a polypeptide subunit of a single Ig, detectable as a monoclonal protein (M protein) on serum or urine electrophoresis

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14
Q

Plasma Cell Myeloma (PCM, Multiple Myeloma) - Definition & Diagnosis

A

A bone-marow based plasma cell neoplasm associated with M protein in the serum or urine;

Diagnosed based on:

M protein present in serum or urine (most often IgG, IgA)

Monoclonal plasma cells present in the bone marrow, usually > 10% nucleated cells in the marrow

Related organ or tissue impairment: hypercalcemia, renal insufficiency, anemia, bone lesions (CRAB)

Rouleaux formation (stacked or linked RBCs) may be present on peripheral smear when M protein is significantly elevated

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15
Q

Monoclonal Gammopathy of Undetermined Significance (MGUS) - Definition and Diagnosis

A

Describes the presence of a monoclonal Ig in the serum or urine of a patient without other evidence of plasma cell myeloma or other disease known to produce monoclonal immunoglobulins

Diagnosis is based on:

Presence of M protein at less than myeloma levels

Marrow plasmacytosis < 10%

No lytic bone lesions or other myeloma-related symptoms (CRAB)

*MGUS is a precursor lesion of PCM with a malignant transformation rate of 1.5%/year

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16
Q

Solitary plasmacytoma of bone - definition & diagnosis

A

A localized tumor of the bone composed of clonal plasma cells

Diagnosis is based on:

Presence of a single bone lesion consisting of monoclonal plasma cells

Absence of PCM-related symptoms (CRAB)

Absent or low serum or urine M protein

17
Q

Extraosseous plasmacytoma

A

Localized plasma cell tumors arising in tissues outside of the bone marrow, most often in the upper respiratory system

18
Q

Hodgkin’s Lymphoma - 5 sub types

A 
Classical Hodgkin's Lymphoma 
Nodular Sclerosis Hodgkin's Lymphoma
Lymphocyte-rich Hodgkin's lymphoma
Mixed cellularity Hodgkin's lymphoma
Lymphocyte-depleted Hodgkin's lymphoma
19
Q

Reed-Sternberg (RS) cells

A

Diagnostic for Classical Hodgkin’s Lymphoma

Morphology:

Large
Multiple nuclei or single, multi-lobated nuclei
Single large, eosinophilic nucleolus
Ample cytoplasm

20
Q

Classical Hodgkin’s Lymphoma

A

Germinal B cell lymphoma

Diagnosis is based on the identification of Reed-Sternberg cells; malignant cells represent only <2% of total cellular population

21
Q

CHL - Immunophenotype

A

Positive for CD30 and CD15

Negative for CD10 and BCL6 (usually positive in germinal center B cells)

22
Q

Nodular Sclerosis Classic Hodgkin Lymphoma

A

Most common subtype of CHL; accounts for 50-80% of all CHLs

Predominates in young adults

Morphology includes a thickened lymph node capsule and nodular areas of lymph node surrounded by broad bands of collagen

Lacunar cells present - large tumor cells surrounded by a prominent clear space, an artifact of formalin fixation

Positive for CD15 and CD30

23
Q

Mixed Cellularity CHL

A

Second most frequent sub-type of CHL, 20-30%

Frequently seen in stages III and IV with B symptoms (fever, fatigue, night sweats)

Lack of broad bands of collagen seen in nodular sclerosis CHL

75% EBV+

24
Q

Lymphocyte Rich CHL

A

Accounts for 5% of CHLs

Frequently presents in stages III and IV with presence of B symptoms; lack of broad bands of collagen seen in nodular sclerosis CHL

Classic RS cells very rare

Most favorable prognosis

25
Q

Lymphocyte-depleted CHLs

A

Rarest subtype of CHL - 1%

Numerous scarce RS cells, scarce lymphocytes

DD & BL Unit 2 (7 decks)

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