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LPD > B cell LPD > Flashcards

B cell LPD Flashcards

1
Q

GCB immunophenotype

A

CD10+

CD10-/BCL6+/MUM1-

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2
Q

ABC immunophenotype

A

CD10-/BCL6+/MUM1+
CD10-/BCL6-
(2 pos or 2 neg)

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3
Q

IPI for DLBCL

A 
(SLOPE)
Stage III or IV
LDH
Old age > 60
Performance status 2 or more
Extranodal sites > 1
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4
Q

Immunophenotype of Burkitt lymphoma

Include Endemic subtype and pertinent negative IHC

A
  • Moderate to strong membrane IgM with light chain restriction
  • B cell antigens - CD19, CD20, CD79a and PAX5
  • Germinal centre markers are positive: BCL6 and CD10
  • Ki67 approaches 100% (unless apoptosis causes it to be falsely low)
    Negative = TdT, CD5, CD23, CD30, CD138, BCL2
    Endemic: almost all are EBV positive and CD21+ (receptor for EBV)
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5
Q

Subtypes of Burkitt lymphoma

A

Endemic (African) - facial/jaw bone; EBV driven
Sporadic - abdominal mass and ascites
Immunodeficiency - lymphadenopathy

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6
Q

Features of Burkitt lymphoma

A 
Highly aggressive B cell lymphoma
Rapidly enlarging mass
Very high LDH
Spontaneous tumour lysis
Characterised by MYC translocations
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7
Q

Cytogenetics and molecular findings of BL

A
  • Hallmark is translocation of MYC at 8q24:
    t(8;14) = IgH (80%)
    t(2;8) = kappa (15%)
    t(8;22) = lambda (5%)
  • 70% have TCF3 or its negative regulator ID3 mutations which activate the BCR signalling pathway.
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8
Q

Morphology and immunophenotype of ALK+ large B cell lymphoma

A

Immunoblastic or plasmablastic
Plasma cell phenotype.
ALK+, EMA+, Pan-B negative, CD138+, cIg+, light chain restriction, often CD45-
CD30-

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9
Q

Plasmablastic lymphoma

A

CD30+
CD138+, CD38+, CD20-
cIg with light chain restriction
CD56+/-

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10
Q

Presentation of Hairy Cell Leukaemia

A

Splenomegaly, B symptoms
Pancytopenia with monocytopenia
Opportunistic infections

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11
Q

Pathogenesis of HCL?

A

Post GC B cell
BRAF mutation = constitutive action of MAPK
Prolonged cell survival is mediated by BRAF and BCL2 over-expression (anti-apoptosis)
Hairy cells secrete FGF = reticulin fibrosis
Hairy cells secrete TGF-beta = inhibits normal haematopoiesis

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12
Q

Immunophenotype of HCL?

A

CD19, CD20, CD22, CD11c and sIg (all bright)
CD25, CD103 and CD123, CD200
Annexin A1, BRAF

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13
Q

Morphology of HCL

A

Intermediate sized lymphocytes
Oval or kidney shaped nuclei
Spongy, homogenous nuclear chromatin pattern with ABSENT NUCLEOLI
Abundant cytoplasm, grey blue hairy projections
TRAP positive.
Trephine = Fried egg appearance due to widely spaced lymphocytes with abundant cytoplasm and prominent cell borders.

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14
Q

Compare and contrast HCLv with HCL

A

Clinical: HCLv = leukocytosis, normal monocyte count
Morphology: HCLv = int. lymphocytes with hairy projections but prominent nucleoli and less spongy appearance of chromatin. BM may show intrasinusoidal or subtle interstitial infiltrate, no fried egg appearance
Immunophenotype: HCLv = CD11c and CD103 positive but BRAF, CD25 and CD123 negative. DBA.44 is positive.
Management: Splenectomy or R-chemo, resistant to cladribine.

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15
Q

Clinical features of LPL/Waldenstrom’s

A 
Anaemia
Cold agglutinin disease
Cryoglobulinaemia
Hyperviscosity
Amyloidosis
Coagulopathy
Neuropathy (MAG antibodies)
Bing Neel syndrome (CNS involvement)
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16
Q

Causes of coagulopathy in Waldenstrom’s

A
  1. Impaired platelet function - paraprotein non-specifically binds to platelets
  2. Paraprotein interferes with fibrin clot formation
  3. Acquired VWD
  4. Acquired factor VIII deficiency
  5. Amyloidosis causing acquired factor X deficiency
17
Q

Manifestations of hyperviscosity syndrome?

A

Triad:

  1. Mucosal bleeding (epistaxis, gum bleeding)
  2. Visual - retinal haemorrhages
  3. Neurological - headaches, dizziness, rarely can get coma
18
Q

What are cryoglobulins?

A

Immunoglobulins that precipitate on cooling (and redissolve on re-warming)

19
Q

Describe how you would test for cryoglobulins

A

Take blood into pre-warmed syringe and put into pre-warmed test tube free of anti-coagulant
Allow to clot at 37 degrees
Centrifuge at 37 degrees and collect serum
Cool serum to 4 degrees and store for 7 days
Measure amount of ppt compared to original volume of serum.
N.B. pre-warming is essential to avoid false negatives - otherwise cryoglobulins may ppt into clotted blood.

20
Q

Morphologic findings in LPL?

A

Small mature lymphocytes + plasmacytoid lymphocytes + plasma cells (may be dispersed or seen in clusters)
Interstitial, nodular, diffuse +/- paratrabecular infiltrate

21
Q

Immunophenotype of LPL?

- Include B lymphocytes and plasma cells

A

CD19+, CD20+, CD22+, CD79b positive, sIg+
CD5- and CD10-
Plasma cells - CD138+, CD19+ (unlike MM) and often CD45+

22
Q

Morphologic variants in Mantle cell lymphoma

A 
Classic = fish mouth
Small cell
Marginal zone like
Blastic (resembles ALL)
Pleomorphic
23
Q

Prognostic factors in Mantle cell lymphoma (MIPI + 2 more)

A 
WBCs > 10
ECOG 2-4
Age >60
Ki67 >30%
LDH >ULN
Pleomorphic and blastic morphology
17p deletion
24
Q

What is leukaemic non-nodal MCL?

A

More indolent form of MCL
PB, BM +/- splenic involvement but not nodal.
CCND1+ but SOX11 negative
IgHV hypermutated (goes through germinal centre)
Can progress to more aggressive form upon acquisition of TP53 mutation.

25
Q

Diagnostic Criteria for HLH

A

A. Molecular diagnosis consistent with HLH
B. 5/8 or more of the following criteria:
1. Fever >38.5
2. Splenomegaly
3. Increased TGs or low fibrinogen
4. Cytopenias (2 out of Hb <100, Plts <100, Neuts <1.0)
5. Haemophagocytosis in BM, LNs, spleen or liver
6. High ferritin (>500)
7. Increased sCD25
8. Decreased or absent NK cell function (flow cytometry for NK cell perforin)

26
Q

Pathogenesis of HLH

A
  1. Immune activation (stimulation from infection e.g. EBV or immune deficiency causing lack of down regulation of activated macrophages)
  2. Excessive activation of macrophages
    - cytokine storm
    - HLH
  3. Impaired NK and T cell cytotoxic response meaning activated macrophages aren’t eliminated.
27
Q

Revised ISS for myeloma

A

B2MG and albumin (3.5, 35, 5.5)
Cytogenetic abnormalities detected by FISH (17p, 4;14, 14;16)
LDH (normal or high)

28
Q

Immunophenotype of plasma cell myeloma

A

CD19-/CD56+ or CD19-/CD56-
Aberrant markers common e.g. CD117, CD20, CD56
Loss of CD45, CD38 but brighter CD138

29
Q

Plasma cell leukaemia - cytogenetics, immunophenotype

A

Can be primary (2/3) or secondary

More commonly associated with t(11;14) and CD20+

30
Q

What factor deficiency can result from amyloidosis?

A

Factor X (factor binds to amyloid)

31
Q

Types of amyloidosis

A

AL - light chain
AA - amyloid associated protein from systemic inflammation
ATTR - either WT (senile) or MT (familial)
B2MG - chronic dialysis

32
Q

Is CD10 positive or negative in primary CNS DLBCL

A

Almost always negative. If CD10+ then must search very hard for systemic disease.

33
Q

Immunophenotype of ALK+ large B cell lymphoma

A

ALK+, EMA+, CD138+, CD45+/-,

Pan B antigens negative

34
Q

Lymphomas with plasmablastic morphology and immunophenotype (CD138+, CD38+, Pan-B neg, often CD45-)

A
  1. Plasmablastic lymphoma (LNs, oral mass, immunodeficiency related)
  2. Plasmablastic myeloma (CRAB)
  3. Primary effusion lymphoma
  4. ALK+ large B cell lymphoma
35
Q

What is the most specific marker for HCL

A

Annexin A1 (not seen in any other B cell LPDs)

36
Q

Name 4 Germinal Centre-derived lymphomas

A
  1. FL
  2. DLBCL
  3. BL
  4. cHL
37
Q

Name a pre-GC derived lymphoma

A

Mantle cell lymphoma

38
Q

Name 5 post-GC derived lymphomas

A
  1. CLL
  2. MZL
  3. HCL
  4. ABC-type DLBCL
  5. LPL
39
Q

DDx of LPD with nucleoli

A 
MCL
B-PLL and T-PLL (smaller and less distinct nucleoli)
HCLv
Atypical CLL
Sometimes SMZL and LPL

LPD (3 decks)

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