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Heme/ Lymph > Peripheral B Cell malignancies > Flashcards

Peripheral B Cell malignancies Flashcards

1
Q

Most common leukemia of adults in western world

A

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic LTerminology & Epidemiology
Same morphology and immunophenotype of malignant cells but with two different clinical presentations

CLL if initially presents with >5000 abnormal B cells/uL blood
Same features but count < 5000 = monoclonal B-cell lymphocytosis
SLL if presents initially as malignant lymphadenopathy
ymphoma (SLL)

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2
Q

Small Lymphocytic Lymphoma

A

Diffusely effaced lymph node architecture

  • Proliferation centers -prominent mitotically active foci with prolymphocytes
      • Pathognomonic for SLL

Small (6-12 micron) lymphocytes with irregular nuclear chromatin
Variable numbers of prolymphocytes & rare large immunoblasts

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3
Q

Chronic Lymphocytic Leukemia

A
  • Irregular nuclear chromatin
  • Smudge cells

Peripheral blood and bone marrow lymphocytosis with small lymphocytes
Hemolytic anemia and/or thrombocytopenia in some patients

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4
Q

soccer ball/ petoskey stone/ turtle shell pattern

A

small lymphocytic lymphoma

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5
Q

CBC reveals anemia with anisocytosis, spherocytes, doublets, schistocytes, a mildly elevated WBC count due to lymphocytosis with lymphocytes with irregular lymph chromatin and an elevated erythrocyte sedimentation rate.
Follow up reveals a low serum haptoglobin level.

What is this?

A

Chronic lymphocytic leukemia (CLL)

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6
Q

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma Immunophenotype

A

+ for CD19 and CD20

Classic finding is presence of T-cell marker CD5 on cells and also expresses CD23

Some CD38+

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7
Q

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma Cytogenetics/Molecular Genetics

A

Chromosomal translocations are rare but can have
Poor prognosis- trisomy 12q and deletions of 11q, 12q,17p
Good prognosis- deletion 13q (LUCKY 13)

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8
Q

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma Clinical Features

A

Onset > age 50 (median age 60)

Male predominance 2:1

Often asymptomatic

May have hypogammaglobulinemia (↑bacterial infections)

May have small monoclonal Ig peak by electrophoresis

CLL/SLL disrupts normal immune function (infections)

*** 10-15% patients have autoantibodies produced by non-neoplastic cells to red cells and/or platelets

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9
Q

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma Prognosis

A

50-60% have mutated IGVH* (memory-post GC)
– live >24 years
ZAP 70-, CD38-
40-50% have unmutated IGVH* (naive-pre GC)
– live mean of 7 years
ZAP 70+, CD38+

Indolent clinical course
Responds poorly to treatment
Diffuse involvement of marrow and/or high serum Beta-2 microglobulin - worse prognosis

  • Immununoglobulin variable region heavy chain
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10
Q

Prolymphocytic Transformation (Leukemia) (CLL/ SLL)

A

a rare transformation with worsening of cytopenias

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11
Q

Richter Transformation (Syndrome) (CLL/ SLL)

A

into Diffuse Large B-cell Lymphoma (3%) or EVB+ Hodgkin Lymphoma (0.5%) with more aggressive course

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12
Q

Follicular Lymphoma frequency

A

“most common form of indolent lymphoma” = most common of the lymphomas that will not kill you quickly

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13
Q

Follicular Lymphoma Two cell types:

and grading

A

Centrocytes - small cleaved
Centroblasts - Larger cell with open nuclear chromatin & ↑cytoplasm

Grade given based on number of centroblasts (grade 3: >15 centroblasts/HPF)
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14
Q

Follicular Lymphoma Immunophenotype:

A

+ for CD19, CD20, CD10 (CALLA) and sIg

+ for BCL2 (apoptosis antagonist), BCL6

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15
Q

Follicular Lymphoma Clinical Features/Prognosis

A

extranodal sites uncommon

Median survival 7 – 9 years (incurable)
Transformation occurs in 30-50% of patients
Usually to Diffuse Large B-Cell Lymphoma

	Following transformation, survival under 1 year
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16
Q

Diffuse Large B-cell Lymphoma Morphology

A

Large size neoplastic lymphocyte

Diffuse pattern of growth

Tumor cells have large nuclei, open chromatin, and prominent nucleoli

17
Q

Diffuse Large B-cell Lymphoma Immunophenotype

and Cytogenetics/ molecular genetics

A

+ for CD19, CD20, sIg
Variable expression germinal center markers (CD10, BCL6)

** dysregulation BCL6, a DNA-binding zinc-finger
transcriptional regulator inhibits expression of factors that regulate differentiation
inhibits p53 activity

18
Q

Diffuse Large B-cell Lymphoma Clinical Features

A

Typically present with rapidly enlarging often symptomatic mass at a single nodal or extranodal site
Oropharynx (Waldeyer ring, tonsils, adenoids) common site

19
Q

Diffuse Large B-cell Lymphoma Important Clinical Subtypes and prognosis

A

Mediastinal large B-cell lymphoma- young women with involvement of viscera and CNS

Immunodeficiency-associated large B-cell lymphoma – occurs in end-stage HIV infection or bone marrow transplantation plus Epstein-Barr Virus

Body cavity large cell lymphoma (Primary effusion lymphoma): associated with KSHV/HHV-8 in HIV patients

Prognosis: 60-80% remission (~50% of these cured)

20
Q

Burkitt Lymphoma Three types

A

(different demographics - same histologic findings)

African

  • EBV positive
  • Jaw and viscera involvement

Sporadic US (nonendemic)

  • Most EBV negative
    • Abdominal masses (ileocecal involvement most frequent)
  • Bilateral breast, ovaries

Immunodifficiency-associated

  • In association with HIV infection
  • May be initial manifestation of AIDS
  • Highly aggressive lymphoma
21
Q

Burkitt Lymphoma Morphology

A

** “Starry Sky”

Involved tissues effaced by diffuse infiltrate neoplastic lymphocytes
Intermediate-sized lymphocytes (10-25 micron in diameter) and moderate amount of amphophilic or basophilic cytoplasm
High mitotic rate (Warburg effect with aerobic glycolysis and high biosynthesis)
Apoptotic tumor cell death (“Starry Sky” pattern)
Marrow and blood rarely involved

22
Q

Burkitt Lymphoma Immunophenotype

A

Hypermutated mature follicular center B-cells with surface IgM, kappa or lambda light chains and + for IgM, BCL6, CD19, CD20 and CD10

23
Q

Mantle Cell Lymphoma morphology

A

Onset 5th to 6th decade

Morphology: Homogenous population small cleaved lymphocytes resembling the normal mantle B-cells that surround the follicular center

24
Q

Mantle Cell Lymphoma Cytogenetics and Molecular Genetics and immunophenotype:

A

t(11:14) translocation involving the Cyclin D1 (cell cycle regulator promoting G1→S phase) locus on 11 and IgH locus on chromosome 14

Classical nodal (90%) 
Leukemic nonnodal (10%) - Hypermutated cell

Immunophenotype: * Cyclin D1, * CD5,

(CD19, CD20, BCL2, and moderately high levels of surface immunoglobulin (IgM or IgD), but CD23-, CD10-)

25
Q

Mantle Cell Lymphoma Clinical Features

A

Presents with painless lymphadenopathy +/- peripheral blood involvement
Signs/symptoms of splenomegaly

Extranodal GI involvement commonly seen
Sometimes presents as ***lymphomatoid polyposis

Prognosis: Aggressive, Median survival only 3-4 years

26
Q

Marginal Zone Lymphoma terminology, location, pathogenesis

A

(MALT Lymphoma when in GI Tract, BALT when in bronchial tissue)

Terminology: Initially recognized in mucosal sites and referred to as MALTomas or mucosal-associated lymphoid tissue lymphomas

Body Location and Morphology: Extranodal (GI & Spleen) and/or lymph nodes

Remain localized for long periods, spreading systemically only late in course
If gastric, may regress if inciting agent (H. pylori) is removed

27
Q

Marginal Zone Lymphoma Immunophenotype

A

[nothing here really helps us]

Positive CD19, CD79a, BCL2
Negative CD5, CD10, CD23, cyclin D1

28
Q

Marginal Zone Lymphoma genetics and prognosis

A

No translocations initially but later can develop;
t(11;18) MALT1; BIRC2 (API2);

Prognosis
Excellent if inciting agent removed (80% 15 year survival)
If translocations occur then tumor does not regress with antibiotic treatment
May transform into diffuse large B-cell lymphoma

29
Q

Hairy Cell Leukemia Immunophenotype

A

CD25, PAX5, CD11c , CD103
**TRAP (tartrate resistant acid phosphatase)

[CD19, CD20, CD79a, sIg, CD22, ]
also CD123, HC2, FMC7 and annexin A1

30
Q

Hairy Cell Leukemia Clinical Features and prognosis

A

Massive * splenomegaly common

  • Pancytopenia with increased susceptibility to infection
    Increased atypical mycobacterial infections

Prognosis:
Indolent course
Tumor cells **“exceptionally sensitive” to chemotherapy
Long-lasting remission in majority of patients

Heme/ Lymph (15 decks)

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