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Boards.CP.Heme > MZL,HCL,PLL,LPL,Heavy Chain > Flashcards

MZL,HCL,PLL,LPL,Heavy Chain Flashcards

-Marginal Zone Lymphoma -Hairy Cell Leukemia -PLL -LPL -Heavy Chain disease

1
Q

Marginal Zone Lymphoma - Splenic Morphology:

  • Expansion of what Pulp?
  • Lymph Node involvement
  • Liver involvement?
  • Peripheral Blood involvement?
A
  • Expansion of WHITE pulp
  • Splenic Hilar nodes exclusively
  • Liver sinusoids (often involved)
  • P. blood - splenic lymphoma with villous lymphocytes (SLVL)
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2
Q

What is typically NOT involved in Hairy Cell Leukemia?

A

Lymph Nodes

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3
Q

What is the most common form of Heavy Chain disease>

A

alpha H chain disease

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4
Q

MZL: Associated Antienic Stimulation:

-Cutaneous MALT

A

B. burgdorferi

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5
Q

Immunophenotype of mature B cell neoplasms composed of small lymphoctyes:
-CD5 Positive (2)

A
  • CLL

- Mantle Cell

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6
Q

Hairy Cell Leukemia - Tissue Morphology:

-Histochemistry

A

TRAP (Tartrate Resistant Acid Phosphatase)

-Strong TRAP is specific; Weak TRAP is nonspecific

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7
Q

Marginal Zone Lymphoma - Extranodal (MALT) Morphology:

  • Proliferation
  • Tumor Cells
  • Ass. lesions
  • Germinal centers?
A
  • Variably destructive and/or tumefactive
  • Monocytoid B cells with clonal plasma cells (Dutcher bodies)
  • Lymphoepithelial lesions
  • Reactive (polyclonal) germinal centers may be present
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8
Q

Hairy Cell Leukemia - IHC:

-Negative for (1)*

A

-CD43

  • ~10% are positive for CD10 (do not differ clinically)
  • CD5, CD23
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9
Q

Immunophenotype of mature B cell neoplasms composed of small lymphoctyes:
-CD23 Positive (2)

A
  • CLL

- Follicular Lymphoma

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10
Q

Hairy Cell Leukemia - Presentation. (3)

A
  • New onset neutropenia, moncytopenia, or aplastic anemia
  • Splenomegaly
  • 4:1 male:female ratio
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11
Q 
Lymphoplasmacytic lymphoma (LPL) IHC:
-Negative or weak for (1)*
A
  • CD43

* CD5, CD10, CD23

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12
Q

Hairy Cell Leukemia - Ultrastructural Morphology.

A

Ribosome lamellar complexes

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13
Q

Marginal Zone Lymphoma - IHC:

-CD43

A

Negative in MZL generally

-Positive in 30% of MALT lymphoma

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14
Q

Hairy Cell Leukemia - Blood Smear Morphology:

  • size
  • nuclei
  • chromatin
  • nucleoli
  • characteristic
A
  • Large (2X normal lymphs)
  • Round to reniform nuclei with smooth contour
  • Ground glass chromatin
  • Indistinct/absent nucleoli
  • Circumferential frayed cytoplasmic edge (Hairy projections)
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15
Q 
Lymphoplasmacytic lymphoma (LPL) IHC:
-Positive for (2)*
A
  • CD38
  • cIg (plasma cells)

*sIg(BRIGHT), CD19, CD20

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16
Q

Marginal Zone Lymphoma - IHC:

-Positive for (4)*

A
  • CD21
  • CD79a
  • FMC7
  • blc-2

*sIg (IgM), CD19, CD20

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17
Q

Prolymphocytic Leukemia (PLL) Presentation. (4)

A
  • WBC (>100,000)
  • B symptoms
  • Cytoplenias
  • Splenomegaly
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18
Q

What is Immunoproliferative Small Intestine Disease (IPSID) and what infection is it associated with?

A

AKA Mediterranean lymphoma

  • form of MALT lymphoma
  • Campylobacter jejuni infection
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19
Q

Marginal Zone, Hairy Cell, PLL and LPL share the following IHC profiles:

  • Positive for (3)
  • Negative for (3)
A

Positive for:

  • CD19
  • CD20
  • sIG

Negative for:

  • CD5
  • CD10
  • CD23
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20
Q

Lymphoplasmacytic lymphoma is associated with what other conditions?

A
  • HCV
  • Cryoglobulinemia

*These cases may respond to antiviral treatment

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21
Q 
Lymphoplasmacytic lymphoma (LPL):
-Molecular/Cytogenetics
A

t(9;14)

-PAX5 gene and C region of IgH

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22
Q

MZL: Associated Antienic Stimulation:

-Thyroid MALT

A

Hashimoto

23
Q

Peripheral blood invovlement of Splenic MZL (SLVL):

-Cells resemble Hairy cell leukemia (HCL), but SLVL is more likely to display what?

A
  • Nucleoli

- Polar villous projections

24
Q

What cytopenia is most commonly associated with HCL?

A

Monocytopenia

25
Q

Marginal Zone Lymphoma - Molecular & Cytogenetic:

-Stomach and Lung

A

t(11;18)

-Rearrangement of API2 and MALT1 genes

26
Q

Lymphoplasmacytic Lymphoma (LPL): Diagnosed when which other lymphomas with plasmacytic features are excluded? (3)

A
  • CLL/SLL
  • Mantle Cell
  • Marginal Zone
27
Q

MZL: Associated Antienic Stimulation:

-Gastric MALT

A

H. pylori

28
Q

What are the 3 types of Marginal Zone Lymphomas (seperated by clinical presentation)?

A
  • Nodal Marginal Zone B cell Lymphoma
  • Extranodal Margin Zone B cell Lymphoma of MALT
  • Splenic Marginal Zone Lymphoma
29
Q

Hairy Cell Leukemia - General Tissue Morphology. (4)

A
  • “fried egg”
  • Reticulin fibrosis
  • Blood lakes
  • Mast Cells frequent
30
Q

MZL: Associated Antienic Stimulation:

-Salivary MALT

A

Sjogren syndrome

31
Q

Prolymphocytic Leukemia (PLL):

  • Diagnosis criteria
  • Cell mophology
A
  • > 55% prolymphocytes

- Prominent Nucleoli and moderate cytoplasm

32
Q

In both Nodal and Extranodal MZL, what shape do B cells classically adopt?

A

Monocytoid

33
Q

MZL: Associated Antienic Stimulation:

-Ocular MALT

A

C. psittaci

34
Q

Immunophenotype of mature B cell neoplasms composed of small lymphoctyes:
-CD10 Positive (1)

A

Follicular Lymphoma

35
Q

Marginal Zone Lymphoma - IHC:

-Negative for (3)*

A
  • CD11c
  • CD103
  • Annexin A1

*CD5, CD10, CD23

36
Q

Lymphoplasmacytic lymphoma Cell Morphology.

A
  • Spectrum of small lymphs to Plasma cells

- Dutcher bodies may be present

37
Q

Marginal Zone Lymphoma - Molecular & Cytogenetic:

-Ocular, Parotid, Cutaneous

A

t(14;18)

-MALT1-IgH Fusion

38
Q

Hairy Cell Leukemia - IHC:

-Positive for (7)*

A
  • CD11c (bright)
  • CD22
  • CD25 (bright)
  • CD103
  • cyclin D1 (dim, nuclear)
  • Annexin A1
  • DBA.44

*sIG, CD19, CD20

39
Q

MZL: Associated Antienic Stimulation:

-IPSID (alpha-chain disease)

A

C. jejuni

40
Q

Hairy Cell Leukemia Molecular findings:

A

No reproducible molecular findings.

-bcl-1 overexpression NOT associated with structureal rearrangements of BCL1

41
Q 
Prolymphocytic Leukemia (PLL) IHC:
-Negative for (2)*
A
  • CD11c
  • CD25

*CD5, CD10, CD23

42
Q

Heavy Chain Disease definition.

A

A lymphoproliferative disorder in which immunoglobulin Heavy (H) chains only are produced

43
Q

Marginal Zone Lymphoma - IHC:

-Plasma cells

A

Monoclonal cytoplasmic Light Chains

44
Q

What is Franklin H chain disease?

A

gamma (y) heavey chain disease found in some cases of LPL

45
Q

Immunophenotype of mature B cell neoplasms composed of small lymphoctyes:
-CD25 (2)

A
  • Hairy Cell Leukemia

- SLVL

46
Q

Immunophenotype of mature B cell neoplasms composed of small lymphoctyes:
-FMC7 Negative (1)

A

-CLL

47
Q 
Prolymphocytic Leukemia (PLL) IHC:
-Positive for (2)*
A
  • CD79a
  • FMC-7

*CD19, CD20, sIg

48
Q

Marginal Zone Lymphoma - Nodal Morphology:

  • Proliferation
  • Tumor cells:
  • -size
  • -nuclei
  • -cytoplasm
  • -additional cell types
A

-Nodular or diffuse proliferation

Tumor cell morphology

  • Small lymphocytes
  • rounded to indented nuclei
  • abundant pale cytoplasm (monocytoid)
  • Clonal Plasma cells (rare Dutcher bodies)
49
Q

Immunophenotype of mature B cell neoplasms composed of small lymphoctyes:
-CD11c (3)

A
  • Hairy Cell Leukemia (+++)
  • MZL/SLVL (+)
  • CLL (+/-)
50
Q

Lymphoplasmacytic lymphoma Lymph Node Morphology

A

Architecture may be preserved or effaced

  • LPL in interfollicular spaces and sinuses
  • PAS+ material in sinuses (+/-)
51
Q

Waldenstrom macroglulinemia is LPL with what additional features?

A
  • IgM monoclonal gammopathy

- Bone marrow involvement

52
Q

MZL: Associated Antienic Stimulation:

-Splenic MALT

A

HCV

53
Q

Hairy Cell Leukemia - Tissue Morphology:

  • Spleen
  • Liver
A
  • Infiltrate RED pulp in spleen

- Infiltrate Sinusoids in liver

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