Marginal Zone Lymphoma - Splenic Morphology: - Expansion of what Pulp? - Lymph Node involvement - Liver involvement? - Peripheral Blood involvement?

- Expansion of WHITE pulp - Splenic Hilar nodes exclusively - Liver sinusoids (often involved) - P. blood - splenic lymphoma with villous lymphocytes (SLVL)

Marginal Zone Lymphoma - Extranodal (MALT) Morphology: - Proliferation - Tumor Cells - Ass. lesions - Germinal centers?

- Variably destructive and/or tumefactive - Monocytoid B cells with clonal plasma cells (Dutcher bodies) - Lymphoepithelial lesions - Reactive (polyclonal) germinal centers may be present

Hairy Cell Leukemia - Blood Smear Morphology: - size - nuclei - chromatin - nucleoli - characteristic

- Large (2X normal lymphs) - Round to reniform nuclei with smooth contour - Ground glass chromatin - Indistinct/absent nucleoli - Circumferential frayed cytoplasmic edge (Hairy projections)

MZL,HCL,PLL,LPL,Heavy Chain Flashcards by Filip Garrett

Marginal Zone Lymphoma - Splenic Morphology:

Expansion of what Pulp?
Lymph Node involvement
Liver involvement?
Peripheral Blood involvement?

Expansion of WHITE pulp
Splenic Hilar nodes exclusively
Liver sinusoids (often involved)
P. blood - splenic lymphoma with villous lymphocytes (SLVL)

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What is typically NOT involved in Hairy Cell Leukemia?

Lymph Nodes

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What is the most common form of Heavy Chain disease>

alpha H chain disease

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MZL: Associated Antienic Stimulation:

-Cutaneous MALT

B. burgdorferi

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

- Mantle Cell

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Hairy Cell Leukemia - Tissue Morphology:

-Histochemistry

TRAP (Tartrate Resistant Acid Phosphatase)

-Strong TRAP is specific; Weak TRAP is nonspecific

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Marginal Zone Lymphoma - Extranodal (MALT) Morphology:

Proliferation
Tumor Cells
Ass. lesions
Germinal centers?

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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Hairy Cell Leukemia - IHC:

-Negative for (1)*

-CD43

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

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

- Follicular Lymphoma

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Hairy Cell Leukemia - Presentation. (3)

New onset neutropenia, moncytopenia, or aplastic anemia
Splenomegaly
4:1 male:female ratio

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Lymphoplasmacytic lymphoma (LPL) IHC:
-Negative or weak for (1)*

CD43

* CD5, CD10, CD23

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Hairy Cell Leukemia - Ultrastructural Morphology.

Ribosome lamellar complexes

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Marginal Zone Lymphoma - IHC:

-CD43

Negative in MZL generally

-Positive in 30% of MALT lymphoma

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Hairy Cell Leukemia - Blood Smear Morphology:

size
nuclei
chromatin
nucleoli
characteristic

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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Lymphoplasmacytic lymphoma (LPL) IHC:
-Positive for (2)*

CD38
cIg (plasma cells)

*sIg(BRIGHT), CD19, CD20

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Marginal Zone Lymphoma - IHC:

-Positive for (4)*

CD21
CD79a
FMC7
blc-2

*sIg (IgM), CD19, CD20

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Prolymphocytic Leukemia (PLL) Presentation. (4)

WBC (>100,000)
B symptoms
Cytoplenias
Splenomegaly

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

AKA Mediterranean lymphoma

form of MALT lymphoma
Campylobacter jejuni infection

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

Positive for (3)
Negative for (3)

Positive for:

CD19
CD20
sIG

Negative for:

CD5
CD10
CD23

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Lymphoplasmacytic lymphoma is associated with what other conditions?

HCV
Cryoglobulinemia

*These cases may respond to antiviral treatment

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Lymphoplasmacytic lymphoma (LPL):
-Molecular/Cytogenetics

t(9;14)

-PAX5 gene and C region of IgH

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MZL: Associated Antienic Stimulation:

-Thyroid MALT

Hashimoto

Peripheral blood invovlement of Splenic MZL (SLVL):

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

Nucleoli

- Polar villous projections

What cytopenia is most commonly associated with HCL?

Monocytopenia

Marginal Zone Lymphoma - Molecular & Cytogenetic: | -Stomach and Lung

t(11;18) | -Rearrangement of API2 and MALT1 genes

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

- CLL/SLL - Mantle Cell - Marginal Zone

MZL: Associated Antienic Stimulation: | -Gastric MALT

H. pylori

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

- Nodal Marginal Zone B cell Lymphoma - Extranodal Margin Zone B cell Lymphoma of MALT - Splenic Marginal Zone Lymphoma

Hairy Cell Leukemia - General Tissue Morphology. (4)

- "fried egg" - Reticulin fibrosis - Blood lakes - Mast Cells frequent

MZL: Associated Antienic Stimulation: | -Salivary MALT

Sjogren syndrome

Prolymphocytic Leukemia (PLL): - Diagnosis criteria - Cell mophology

- >55% prolymphocytes | - Prominent Nucleoli and moderate cytoplasm

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

Monocytoid

MZL: Associated Antienic Stimulation: | -Ocular MALT

C. psittaci

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

Follicular Lymphoma

Marginal Zone Lymphoma - IHC: | -Negative for (3)*

- CD11c - CD103 - Annexin A1 *CD5, CD10, CD23

Lymphoplasmacytic lymphoma Cell Morphology.

- Spectrum of small lymphs to Plasma cells | - Dutcher bodies may be present

Marginal Zone Lymphoma - Molecular & Cytogenetic: | -Ocular, Parotid, Cutaneous

t(14;18) | -MALT1-IgH Fusion

Hairy Cell Leukemia - IHC: | -Positive for (7)*

- CD11c (bright) - CD22 - CD25 (bright) - CD103 - cyclin D1 (dim, nuclear) - Annexin A1 - DBA.44 *sIG, CD19, CD20

MZL: Associated Antienic Stimulation: | -IPSID (alpha-chain disease)

C. jejuni

Hairy Cell Leukemia Molecular findings:

No reproducible molecular findings. | -bcl-1 overexpression NOT associated with structureal rearrangements of BCL1

``` Prolymphocytic Leukemia (PLL) IHC: -Negative for (2)* ```

- CD11c - CD25 *CD5, CD10, CD23

Heavy Chain Disease definition.

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

Marginal Zone Lymphoma - IHC: | -Plasma cells

Monoclonal cytoplasmic Light Chains

What is Franklin H chain disease?

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

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

- Hairy Cell Leukemia | - SLVL

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

-CLL

``` Prolymphocytic Leukemia (PLL) IHC: -Positive for (2)* ```

- CD79a - FMC-7 *CD19, CD20, sIg

Marginal Zone Lymphoma - Nodal Morphology: - Proliferation - Tumor cells: - -size - -nuclei - -cytoplasm - -additional cell types

-Nodular or diffuse proliferation Tumor cell morphology - Small lymphocytes - rounded to indented nuclei - abundant pale cytoplasm (monocytoid) - Clonal Plasma cells (rare Dutcher bodies)

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

- Hairy Cell Leukemia (+++) - MZL/SLVL (+) - CLL (+/-)

Lymphoplasmacytic lymphoma Lymph Node Morphology

Architecture may be preserved or effaced - LPL in interfollicular spaces and sinuses - PAS+ material in sinuses (+/-)

Waldenstrom macroglulinemia is LPL with what additional features?

- IgM monoclonal gammopathy | - Bone marrow involvement

MZL: Associated Antienic Stimulation: | -Splenic MALT

HCV

Hairy Cell Leukemia - Tissue Morphology: - Spleen - Liver

- Infiltrate RED pulp in spleen | - Infiltrate Sinusoids in liver

MZL,HCL,PLL,LPL,Heavy Chain Flashcards

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