HEMA 2 LEC - Mature Lymphoid Neoplasms Flashcards Preview

► Med Misc 05 > HEMA 2 LEC - Mature Lymphoid Neoplasms > Flashcards

Flashcards in HEMA 2 LEC - Mature Lymphoid Neoplasms Deck (41):
1

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

plasma cell neoplasms

2

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

plasma cell neoplasms

3

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

Monoclonal gammopathy of undetermined significance (MGUS):

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

Plasmacytoma

5

• marked bone marrow plasmacytosis.
• Large aggregates and sheets of plasma cells,

plasma cell neoplasms

6

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

plasma cell neoplasms

7

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

Plasma cell leukemia

8

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

Plasma cells

9

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

plasma cell neoplasms

10

• Tissue deposits of clonal immunoglobulins is called

amyloidosis

11

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

what condition

plasma cell neoplasms

12

• Defining feature: large cell size

Diffuse Large B-Cell Lymphoma

13

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

Diffuse Large B-Cell Lymphoma

14

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

Diffuse Large B-Cell Lymphoma

15

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

Burkitt lymphoma

16

3 variants of Burkitt lymphoma WHO classification lists

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

17

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

Burkitt lymphoma

18

• Hallmark of Burkitt lymphoma =

high proliferation rate.

19

how many percent of Burkitt lymphoma cells are actively proliferating

100%

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)

Burkitt Lymphoma

21

gene expressed in Burkitt

MYC gene

22

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

Endemic

23

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

Sporadic

24

variant of Burkitt lymphoma
presents most often as nodal disease; occurs predominantly in HIV- positive patients

Immunodeficiency-associated

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

Sporadic

26

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

Burkitt leukemia

27

virus present in Burkitt Lymphoma

Epstein-Barr virus (EBV)

28

Much less common
Shows geographic and ethnic variability

Mature T-Cell and Natural Killer
 Cell Lymphomas


29

• T-cell neoplasms occur more frequently in

extranodal sites

30

Phenotype (B or T cell) of mycosis fungoides

T cell

31

most common skin lymphoma

mycosis fungoides

32

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

mycosis fungoides

33

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

Sézary syndrome

34

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

Pautrier microabscesses

35

important immunophenotypic feature of mycosis fungoides

absence of CD7 antigen

36

How many sezary cells to be classified as sezary syndrome

at least 1000 Sezary cells/mm^3

37

CD4 to CD8 ratio to be classified as sezary syndrome

more than 10

38

aggressive lymphoma with skin and lymph node involvement

sezary syndrome

39

Morphologically heterogeneous group of lymphomas with mature T-cell phenotype

Peripheral T-Cell Lymphoma, Unspecified

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

Peripheral T-Cell Lymphoma, Unspecified

41

• Generalized lymphadenopathy
• Constitutional symptoms: fever, night sweats, weight loss, and pancytopenia

Peripheral T-Cell Lymphoma, Unspecified

Decks in ► Med Misc 05 Class (295):