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RHS > L21- WBC Pathology II > Flashcards

L21- WBC Pathology II Flashcards

1
Q

classify the precursor lymphoid neoplasms (according to WHO)

A

B-lymphoblastic leukemia/lymphoma, 85%:

  • ” “, NOS* (not otherwise specified)
  • ” “, w/ recurrent genetic abnormalities

T-lymphoblastic leukemia/lymphoma, 15%

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2
Q

(1)/(2) are the most common childhood malignancy with overlapping presentations. Classification of (1)/(2) is based on (3) and (4). Causes of (1)/(2) are unknown, but it is suspected to be associated with (5).

A

1/2- ALL, acute lymphoblastic leukemia / lymphoma
3- immunophenotyping (B/T cell differentiation)
4- presence of cytogenic abnormalities
5- ionizing radiation

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3
Q

ALL presents with a (slow/rapid) onset with (mild/severe) symptoms.

  • (2) and (3) symptoms are seen in all ALL types
  • (4) is seen in T-ALL
  • (5) is seen in B-ALL
A

1- rapid (abrupt), severe onset
2- (related to BM replacement) cytopenias, bone pain
3- lymph node enlargement and or organomegaly (liver, spleen)
4- thymic enlargement
5- testicular enlargement, CNS involvement (HAs, blurred vision, vomiting)

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4
Q

ALL, laboratory diagnostics:

  • (1) CBC results
  • (2) morphology on peripheral blood smear
A

1- leukocytosis (possibly >100,000)

2- blasts seen in peripheral blood smear and bone marrow aspirate/biopsy: blasts with scanty (agranular) basophilic cytoplasm, delicate stippled (condensed) chromatin and small nucleoli

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5
Q

ALL, laboratory diagnostics:

  • (1) specific histochemical stains
  • (2) methods of immunophenotyping
  • (3) common results of cytogenetic investigations
A

1- myeloperoxidase and Sudan black negative // some PAS positivity

2- (important for T-ALL v B-ALL) flow cytometry, immunohistochemistry

3- (90% abnormal) translocations, deletions, hyperdiploidy are possibly present

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6
Q

Flow Cytometry in ALL:

  • (1) are expressed on pre-B lymphoblasts and indicate B-ALL
  • (2) may also be expressed on mature B-cells
A

(B-cells)
1- CD10, CD19 (pan-B-cell marker)
2- TdT (terminal deoxynucleotidyl-transferase), CD22

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7
Q

ALL Tx:

  • (1) list the phases
  • (2) and (3) are the common components of each phase
  • (4) include remission and fully cured statistics
  • (5) Tx is reserved for relapsed cases
A

1:

i) remission induction
ii) consolidation therapy
iii) maintenance therapy

2- multi-agent chemotherapy
3- testicular, CNS prophylaxis

4- 90% remission, 65% cured overall (higher in children)
5- BMT (bone marrow transplant)

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8
Q

list and describe the 5 factors that affect the prognosis of ALL

A

1) age
2) WBC count in peripheral blood smear (lower the better)
3) immunophenotype (T cell worse than B cell)
4) cytogenetics
5) residual disease after chemotherapy

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9
Q

ALL prognosis:

  • (1) describe age difference
  • (2) describe WBC count in peripheral blood smear
  • (3) describe residual disease after chemotherapy
A

1:
Good- age 2-10 y/o
Bad: <2 y/o, >10y/o

2:
Good- low WBC count
Bad- >100,000

3:
Good- undetectable
Bad- detectable

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10
Q

ALL prognosis:

  • (1) describe by immunophenotype
  • (2) describe by cytogenetics
A

1:
Good- B-cell
Bad- T-cell

2:
Good: hyperdiploidy, presence of t(12;21)
Bad: other ploidy, presence of t(9;22)

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11
Q

Non-Hodgkin’s Lymphoma = (1)

provide examples, (2)

A

= Mature (peripheral) B-cell Neoplasms

Exs:

  • B-cell chronic lymphocytic leukemia / small lymphocytic lymphoma (CLL/SLL),
  • B-cell prolymphocytic leukemia (B-PLL)
  • lymphoplasmacytic lymphoma (LPL)
  • marginal zone lymphomas (MZL)
  • mantle cell lymphoma (MCL)
  • follicular lymphoma (FL)
  • hairy cell leukemia (HCL)
  • diffuse large B-cell lymphomas (DLBL)
  • Burkitt lymphoma (BL)
  • plasma cell neoplasms
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12
Q

(1) then (2) are the most common B-cell lymphomas, aka (3)

A

1- DLBCL (diffuse large B-cell lymphoma)
2- FL (follicular lymphoma)
3- Non-Hodgkin’s lymphoma

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13
Q

(1) is the most common lymphoma in adults, with median age of presentation at (2), although it can occur (3).

A

1- DLBCL (diffuse large B-cell lymphoma)
2- 60 y/o
3- any age, 15% of childhood lymphomas

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14
Q

DLBCL, (1), is a (HL/NHL) with a(n) (aggressive/passive) history. DLBCL can be caused / classified by (4) or (5).

A

1- diffuse large B-cell lymphoma
2- non-hodgkin’s lymphoma
3- aggressive

4- primary, de novo
5- secondary, transformation of previous low grade lymphoma (mainly FL, but most small B-cell lymphomas have this capability)

Note- many subtypes with variable prognoses

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15
Q

DLBCL, (1), presents with (2) as the main symptom in (3) or (4) location, and usually presents in (5) stage. Other common symptoms include this triad, (6).

A 
1- diffuse large B-cell lymphoma
2- rapidly enlarging lymphoid mass(es)
3- lymph nodes, 60%
4- extra-nodal mass, 40%
5- stage I, II (w/o Bone Marrow involvement)
6- fever, weight loss, night sweats
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16
Q

DLBCL has a (diffuse/patchy) growth pattern with (small/large) (B/T)-cells. Nuclei are described as (4). Immunophenotyping will look for (5).

A

(diffuse large B-cell lymphoma)
1- diffuse
2- large, 3-4x normal size
3- B-cells
4- medium/large, round/oval nuclei with dispersed chromatin and several small nucleoli
5- expressed B cell Ags (typically includes CD20)

17
Q

DLBCL (diffuse large B-cell lymphoma) is a(n) (aggressive/passive) cancer that is always (curable/fatal). Treatment includes (3) and (4). (5) are the typical results from treatment.

A

1- aggressive
2- fatal if untreated, often curable

3- combination chemotherapy
4- anti-CD20 immunotherapy
5- 60-80% remission –> 50% of which remain cured

18
Q

(1) lymphoma is derived from germinal center B-cells, mainly affecting (2) age group. (3, describe) is the most common cytogenetic abnormality seen as it results in (4).

A

1- FL, follicular lymphoma
2- 50-60 y/o
3- (>85%) t(14;18) –> translocation fuses BCL2 gene on chr.18 next to igH locus on chr.14
4- overexpression of BCL2 protein –> major apoptosis inhibitor

19
Q

FL, (1), is a (rapid/slow) growing tumor. It often presents in (3) stage with (4) involvement.

A

1- follicular lymphoma
2- slow (indolent) growth
3/4- stage IV; bone marrow involvment (80% cases)

20
Q

list the 3 main manifestations of FL

A

(follicular lymphoma)
-painless localized to generalized lymphadenopathy

  • 40% have tumor cells in blood (resembling CLL)
  • frequently involves spleen/liver –> hepatosplenomegaly
21
Q

FL, follicular lymphoma, has a (1) or (2) growth pattern. Packed follicles contains the following two types of cells (3) and (4), more increased levels on (3/4) indicate a tumor with more aggressive behavior, likely transformation into (6).

A

1- nodular, >75% follicular
2- nodular and diffuse, 25-75% follicular

3- small cleaved cells w/o nucleoli –> Centrocytes
4- large cells w/ moderate cytoplasm, multiple nucleoli –> Centroblasts

5- Centroblasts (4)
6- DLBCL

22
Q

describe using Immunophenotyping to differentiate the causes of enlarged lymph node (lymphadenopathy)

A

Mark for BCL2 in follicle:
-very little staining in follicle (dark brown BCL2 in mantle cell zone) = Reactive Lymph Node

-dark brown BCL2 staining in follicle = FL

23
Q

FL diagnosis:

  • (1) is necessary
  • (2) describe immunophenotyping
  • (3) characteristic cytogenic change
A

1- characteristic lymph node morphology

2- by flow cytometry OR immunohistochemistry for CD19, CD20, CD10

3- t(14;18), 90% of cases

24
Q

FL prognosis:

  • mostly (1) characteristic
  • 40% transform into (2)
  • (3) is the median survival of (2)
A

1- incurable (except for cases with large cells / high grade)

2- DLBCL

3- <1 yr

25
Q

CLL and SLL are mostly identical, but differ in…..

A

(chronic lymphocytic leukemia, small lymphocytic lymphoma)

peripheral blood involvement

26
Q

describe characteristics of CLL on PB (in comparison to SLL)

A

(chronic lymphocytic leukemia on peripheral blood smear)

  • lymphocyte count > 5000 cells/µL
  • bone marrow involvement
  • monoclonal B cell count > 5000 cells/µl
27
Q

describe characteristics of SLL on PB (in comparison to CLL)

A

(small lymphocytic lymphoma, non-leukemic cases on peripheral blood smear)

  • monoclonal B cell count < 5000 cells/µL
  • enlarging lymph nodes
28
Q

(1) is used to discover CLL/SLL in early diagnosis.

In late diagnosis of CLL/SLL:

  • (2) is evident diffusely
  • (3) is often observed
  • some patient develop (4) autoimmune diseases or increased infections due to (5)
  • eventually (6) symptoms are evident due to progressive bone marrow replacement
A

1- CBC showing leukocytosis

2- generalized lymphadenopathy
3- hepatosplenomegaly, 50-60%
4- autoimmune hemolytic anemia or thrombocytopenia
5- acquired hypogammaglobinemia
6- pancytopenia
29
Q

CLL/SLL morphology and diagnosis:

  • (1) cells are seen on peripheral blood smear
  • (2) is noted on bone marrow biopsy
  • (3) is noted on lymph node biopsies
  • immunophenotyping is completed by (4) for the following CDs, (5)
A

1- small mature appearing lymphocytes = ‘smudge cells’ (inc fragility)
2- interstitial nodules –> diffuse replacement by small lymphocytes
3- diffuse replacement by small round lymphocytes w/ few larger cells concentrated in pale proliferation centers
4- flow cytometry, immunohistochemical staining
5- CD19, CD20, CD5, CD23, CD43

30
Q

CLL/SLL is noted to be (1) in treatment. Mean survival rate is (2), and can reach up to (3) if diagnosed early.

A

1- incurable (although indurant)
2- 4-7 yrs
3- 10 yrs

31
Q

CLL/SLL can lead to death through the following mechanisms,….

A
  • progressive pancytopenia –> infections, bleeding

- transformation into aggressive neoplasm, PLL (20%, prolymphocytic leukemia) OR DLBCL (5-10%)

32
Q

Extra-nodal marginal zone lymphoma = (1). It is a (rapid/slow) growing tumor mostly composed of (3) cells commonly in (4) locations. (4) locations lack abundant lymphoid tissue, but acquire it via (5), mostly commonly the (6) site- include cause for (6)

A

1- MALT lymphoma (MALToma)
2- slow growth, indolent (Note- remains localized for long periods, often recur or spread to other mucosal sites)
3- small mature appearing lymphocytes
4- extra-nodal / mucosal sites
5- chronic infection / autoimmune disease
6- stomach w/ superimposed H. pylori gastritis

33
Q

(1) infections of (2) area can progress into extra-nodal marginal zone lymphoma. (3) treatment has been shown to regression some early MZL (2) tumors.

Other common sites for MZL are (4) and (5).

A

1- H. pylori (=> chronic inflammation)
2- stomach
3- antibiotic Tx for H. pylori gastritis

4- salivary glands
5- thyroid glands (autoimmune inflammation via Hashimoto’s thyroiditis)

RHS (35 decks)

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