HEMA 2 LEC - Mature Lymphoid Neoplasms

Question 1

• monoclonal proliferation of terminally differentiated B cells (i.e., plasma cells)

Answer 1

plasma cell neoplasms

Question 2

• multifocal accumulation of malignant plasma cells in the bone marrow presenting as lytic bone lesions

Answer 2

plasma cell neoplasms

Question 3

• encompass the entire patient population with clonal serum immunoglobulin and only mild marrow plasmacytosis.

Answer 3

Monoclonal gammopathy of undetermined significance (MGUS):

Question 4

a localized form of plasma cell neoplasm, may present as a solitary bone lesion or involve an extraosseous or extramedullary site, most commonly the nasopharynx, oropharynx, or sinuses

Answer 4

Plasmacytoma

Question 5

• marked bone marrow plasmacytosis.

* Large aggregates and sheets of plasma cells,

Answer 5

plasma cell neoplasms

Question 6

high nuclear-to-cytoplasmic ratio, dispersed chromatin pattern, and distinct nucleoli

Answer 6

plasma cell neoplasms

Question 7

: for cases > 20% circulating plasma cells or plasma cell counts exceeding 2 109/L.

Answer 7

Plasma cell leukemia

Question 8

• what cells are positive for CD138 (syndecan 1), high-density CD38 antigen, and monoclonal cytoplasmic immunoglobulins

Answer 8

Plasma cells

Question 9

clinical features
•	Bone pain and pathologic fractures 
•	Renal insufficiency 
•	Cytopenias 
•	Depressed normal immunoglobulin levels

Answer 9

plasma cell neoplasms

Question 10

• Tissue deposits of clonal immunoglobulins is called

Answer 10

amyloidosis

Question 11

• High levels of serum immunoglobulins → interfere with the coagulation cascade → impair circulation through an increase in serum viscosity

what condition

Answer 11

plasma cell neoplasms

Question 12

• Defining feature: large cell size

Answer 12

Diffuse Large B-Cell Lymphoma

Question 13

• Shows a diffuse proliferation of large lymphoid cells replacing the normal lymph node architecture

Answer 13

Diffuse Large B-Cell Lymphoma

Question 14

• Cells at least twice the size of normal small lymphocytes and show single or multiple nucleoli and ample cytoplasm

Answer 14

Diffuse Large B-Cell Lymphoma

Question 15

• Characterized by medium- sized, highly proliferating lymphoid cells with basophilic vacuolated cytoplasm

Answer 15

Burkitt lymphoma

Question 16

3 variants of Burkitt lymphoma WHO classification lists

Answer 16

o	Endemic (occurring predominantly in Africa)
o	Sporadic
o	Immunodeficiency associated

Question 17

• Lymphoid proliferation: diffuse, shows a prominent “starry sky” pattern (low magnification) imparted by numerous tingible body macrophages

Answer 17

Burkitt lymphoma

Question 18

• Hallmark of Burkitt lymphoma =

Answer 18

high proliferation rate.

Question 19

how many percent of Burkitt lymphoma cells are actively proliferating

Answer 19

100%

Question 20

constitutive expression of MYC gene (cell cycle gatekeeping gene) secondary to its translocation under the promoter of immunoglobulin heavy or light chain genes, t(8;14), t(2;8), t(8;22)

Answer 20

Burkitt Lymphoma

Question 21

gene expressed in Burkitt

Answer 21

MYC gene

Question 22

variant of Burkitt lymphoma in young children (4 to 7 years of age), most commonly as a jawbone mass

Answer 22

Endemic

Question 23

variant of Burkitt lymphoma >seen in the United States and Europe, occurs in children and young adults most commonly as an abdominal mass

Answer 23

Sporadic

Question 24

variant of Burkitt lymphoma

presents most often as nodal disease; occurs predominantly in HIV- positive patients

Answer 24

Immunodeficiency-associated

Question 25

variant of Burkitt lymphoma
Gastrointestinal tract and abdominal lymph nodes are often involved however, other extranodal sites, such as gonads and breasts, can be a site of primary disease

Answer 25

Sporadic

Question 26

commonly involves the central nervous system, bone marrow, and peripheral blood (Burkitt leukemia).

Answer 26

Burkitt leukemia

Question 27

virus present in Burkitt Lymphoma

Answer 27

Epstein-Barr virus (EBV)

Question 28

Much less common

Shows geographic and ethnic variability

Answer 28

Mature T-Cell and Natural Killer
 Cell Lymphomas


Question 29

• T-cell neoplasms occur more frequently in

Answer 29

extranodal sites

Question 30

Phenotype (B or T cell) of mycosis fungoides

Answer 30

T cell

Question 31

most common skin lymphoma

Answer 31

mycosis fungoides

Question 32

Small to medium-sized lymphoid cells with irregular nuclear outlines (cerebriform nuclei)
Show a predilection for the epidermis (epidermotropism) and dermis

Answer 32

mycosis fungoides

Question 33

presents as a disseminated disease with widespread skin involvement (erythroderma), lymphadenopathy, and circulating lymphoma cells (Sézary cells with characteristic cerebriform nuclei)

Answer 33

Sézary syndrome

Question 34

In mycosis fungoides, the colonization of epidermis by neoplastic lymphocytes is seen with small aggregates of lymphoma cells forming so-called

Answer 34

Pautrier microabscesses

Question 35

important immunophenotypic feature of mycosis fungoides

Answer 35

absence of CD7 antigen

Question 36

How many sezary cells to be classified as sezary syndrome

Answer 36

at least 1000 Sezary cells/mm^3

Question 37

CD4 to CD8 ratio to be classified as sezary syndrome

Answer 37

more than 10

Question 38

aggressive lymphoma with skin and lymph node involvement

Answer 38

sezary syndrome

Question 39

Morphologically heterogeneous group of lymphomas with mature T-cell phenotype

Answer 39

Peripheral T-Cell Lymphoma, Unspecified

Question 40

• Lymph node involvement: usually diffuse with a prominent vascular proliferation
• Cytologic features are variable with medium-sized to large cells with atypical and occasionally pleomorphic nuclei

Answer 40

Peripheral T-Cell Lymphoma, Unspecified

Question 41

• Generalized lymphadenopathy

* Constitutional symptoms: fever, night sweats, weight loss, and pancytopenia

Answer 41

Peripheral T-Cell Lymphoma, Unspecified