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Heme/Lymph - Exam 1 > 10-28 Clone Wars - Attack of the T and B Lymphoid Drones > Flashcards

10-28 Clone Wars - Attack of the T and B Lymphoid Drones Flashcards

1
Q

What is this? What does Gomez have to say about this?

A

neoplastic lymphoid cells surround a small, atrophic germinal center, producing a mantle zone pattern of growth.

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2
Q

You’re visualizing a Mantle Cell Lymphoma under high power. Dsecribe what you see?

A

a homogeneous population of small lymphoid cells with somewhat irregular nuclear outlines, condensed chromatin, and scant cytoplasm

(Large cells resembling prolymphocytes (seen in chronic lymphocytic leukemia) and centroblasts (seen in follicular lymphoma) are absent)

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3
Q

What does MALT Lymphoma stand for? (softball card)

A

Marginal Zone Lymphoma

or

Mucosal-Associated Lympoid Tissue Lymphoma

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4
Q

Why are marginal zone lymphomas named the way they are?

A

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

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5
Q

What are the body location(s) for marginal zone lymphomas?

A

Body Location and Morphology:

Extranodal (GI & Spleen) and/or lymph nodes

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6
Q

Why do marginal zone lymphomas arise where they do?

A

Arise in tissues involved by chronic inflammatory disorders of autoimmune or infectious etiology

(Helicobacter pylori, Campylobacter jejuni, Sjogren syndrome, Hashimoto thyroiditis)

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7
Q

What is the pathology behind the spread and possible regression of marginal zone lymphomas?

A

Remain localized for long periods, spreading systemically only late in course

If gastric, may regress if inciting agent (H. pylori) is removed

T-helper cell driven

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8
Q

What is the immunophenotype of marginal zone lymphomas?

A

Positive CD19, CD79a, BCL2

Negative CD5, CD10, CD23, cyclin D1

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9
Q

What’s this?

A

A type of marginal zone lymphoma, a gastric MALT lymphoma

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10
Q

What sorts of translocations are associated with marginal zone lymphomas?

A

Genetics

No translocations initially but later can develop:

t(1;14) BCL10; IgH

t(11;18) MALT1;IAP2

t(14;18) IgH;IAP2

BCL10 and MALT1 activate NF-κB

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11
Q

What is the prognosis associated with marginal zone lymphomas? What can these tumors progress to?

A

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

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12
Q

What is the epidemiology of Hairy Cell Leukemia?

A

Rare, 2% of all leukemias

Predominately a disease of middle-aged Caucasian males (M:F 5:1)

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13
Q

What is the immunophenotype associated with hairy cell leukemia?

A

CD19, CD20, CD79a, sIg, CD22, CD25, PAX5, CD11c , CD103, DBA.44, TRAP (tartrate resistant acid phosphatase)

also CD123, HC2, FMC7 and annexin A1

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14
Q

What are the cytogenics behind hairy cell leukemia?

A

Cytogenetics/Molecular Genetics:

High incidence somatic hypermutation (post germinal center)

BRAF mutations

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15
Q

What are the clinical features behind hairy cell leukemia?

A

Clinical Features:

Infiltration bone marrow, liver & spleen by neoplastic cells

Massive splenomegaly common

Pancytopenia with increased susceptibility to infection

Increased atypical mycobacterial infections

Leukocytosis only in 15-20%

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16
Q

What is the prognosis associated with hairy cell leukemia?

A

Indolent course

Tumor cells “exceptionally sensitive” to chemotherapy

Long-lasting remission in majority of patients

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17
Q

What’s this?

A

Hairy cell leukemia:

Phase-contrast microscopy shows tumor cells with fine hairlike cytoplasmic projections

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18
Q

What’s this?

A

Hairy cell leukemia:

In stained smears, these cells have round or folded nuclei and modest amounts of pale blue, agranular cytoplasm

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19
Q

What is often referred to as a plasma cell dyscrasia?

A

B-cell clone that usually synthesizes and usually secrets a single homogeneous immunoglobulin or its fragments

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20
Q

What percentage of deaths do plasma cell neoplasms cause?

A

Cause 15% of deaths from white cell neoplasms

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21
Q

We know that plasma cell dyscrasias usually synthesize and usually secrete a single homogeneous immunoglobulin or its fragments. What name do you use to refer to these components?

A

Monoclonal Ig in the blood is referred to as “M component”, monoclonal protein, dysproteinemia or paraproteinemia

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22
Q

What is an important cytological finding in plasma cell dyscrasias?

A

Rouleaux in peripheral blood smear

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23
Q

What’s this?

A

Plasma cell dyscrasia:

Rouleaux in peripheral blood smear

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24
Q

What are Bence-Jones proteins? Where do they come from?

A

Neoplastic plasma cells often synthesize excess light or heavy chains along with complete immunoglobulins

•Free L (light) chains are known as Bence Jones protein that are primarily detected in the urine since blood levels are quickly eliminated in urine

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25
Q

Name the clinicopathological entities associated with monoclonal gammopathy.

A

Monoclonal gammopathy

Multiple Myeloma

Plasmacytoma

Smoldering myeloma

Heavy (Light) Chain Disease

Waldenstrom Macroglobulinemia

Primary or Immunocyte-Associated Amyloidosis

Monoclonal Gammopathy of Undetermined Significance (MGUS)

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26
Q

What is another name for multiple myeloma?

A

Multiple myeloma (plasma cell myeloma)

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27
Q

Where are plasmacytomas located? What is the prognosis?

A

Plasmacytoma (single mass)

Intraosseous – most eventually progress to multiple myeloma

Soft tissue – may be cured by local resection

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28
Q

What is the primary sign of a Smoldering Myeloma?

A

Smoldering Myeloma (asymptomatic with high plasma M component > 3gm/dL)

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29
Q

What is the ‘typical’ progression of a smoldering myeloma?

A

Smoldering Myeloma (asymptomatic with high plasma M component > 3gm/dL)

~75% per progress to multiple myeloma within 15 years

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30
Q

What is the typical finding associated with Heavy (light) chain disease?

A

Heavy (Light) Chain Disease: Secretes free H (L) chain fragments

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31
Q

What is the primary finding in Waldenstrom Macroglobulinemia?

A

Waldenstrom Macroglobulinemia: High levels of IgM

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32
Q

What is the primary finding in primary or immunocyte-associated amyloidosis?

A

Primary or immunocyte-associated amyloidosis:

free L chains leading to AL type amylodosis

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33
Q

What is the primary finding in MGUS? What is a progression sometimes associated with this cancer?

A

Monoclonal gammopathy of undetermined significance (MGUS):

M components <3 gm/dL identified in blood, but no signs or symptoms

~1% per year progress to multiple myeloma

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34
Q

What would you expect to see on electophoresis with a case of MGUS? What about immunofixation?

A

Monoclonal Gammopathy of Undetermined Significance:

Serum protein electrophoresis (Left) showed a sharp peak in the gamma globulin region.

Immunofixation electrophoresis (IFE, Right) showed a monoclonal IgA, κ band.

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35
Q

What is the epidemiology associated with multiple myeloma?

A
  • Incidence begins to increase substantially between ages 50 and 60 and peaks in 8th and 9th decade
  • 1% US deaths caused by malignancy
  • ~ 15% WBC malignancy deaths
  • 10,000-12,000 cases of multiple myeloma/ year in U.S.
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36
Q

How does the incidence of mult myeloma vary by race, gender?

A

•Incidence varies by race; slightly higher incidence in males

•9/100,000 Blacks

•4/100,000 Caucasians

•2.5/100,000 Chinese

•1.5/100,000 Japanese

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37
Q

What age group is mult myeloma most frequently dx’ed in? What is median age at Dx?

A

Most frequently Dx’ed in age group 65-74

Median age at Dx: 69

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38
Q

What is a progression sometimes associated with mult myeloma?

A

Rarely develop plasma cell leukemia (MYC mutations)

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39
Q

What drives proliferation of mult myeloma?

A

IL-6 drives proliferation

Tumor M1P1α upregulates RANKL and inhibits osteoblasts (Wnt pathway)

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40
Q

What cell markers do mult myelomas often express?

A

Express CD38, CD138, CD79a and cytoplasmic immunoglobulins

(+/- CD56)

Do not usually express CD19

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41
Q

What signs can mult myelomas show on radiographs?

A

Radiographic punched-out lytic defects, usually 1-4 cm in diameter

Tumor M1P1α upregulates RANKL and inhibits of osteoblasts (Wnt pathway)

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42
Q

What’s this?

A

Multiple myeloma of the skull (radiograph, lateral view).

The sharply punched-out bone lesions are most obvious in the calvarium

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43
Q

What are the common locations for mult myelomas?

A

Vertebrae 66%

Ribs 44%

Skull 41%

Pelvis 28%

Clavicle 10%

Scapula 10%

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44
Q

Of the plasma cell dyscrasias, which are the most prevalent by percent?

A

Mult myeloma –> 80-90% PCD

Solitary Plasmacytoma –> 5-10% PCD

Extramedullary Plasmacytoma –> <5% PCD

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45
Q

What is the average age of onset for mult myeloma, versus solitary plasmacytoma and extramedullary plasmacytoma?

A

MM - 59

SP - 56

EP - 59

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46
Q

What are the age ranges consistent for Dx of mult myeloma, versus solitary plasmacytoma versus extramedullary plasmacytoma?

A

MM –> 30-80

SP –> <20 or >80

EP –> <20 or >80

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47
Q

What are the primary locations for mult myeloma versus solitary plasmacytoma versus extramedullary plasmacytoma?

A

MM –> Multiple bone marrow lesions

SP –> Any bone;>50% in vertebrae

EP –> 80-90% upper airway

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48
Q

How often (as a percentage) are monoclonal proteins associated with mult myeloma versus solitary plasmacytoma versus extramedullary plasmacytoma?

A

mm –> 99% (98-100%)

SP –> 43% (22-77%)

EP –> 27% (0-86%)

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49
Q

Describe plasma cells seen in multiple myeloma?

A

Multiple myeloma (bone marrow aspirate).

Normal marrow cells are largely replaced by plasma cells, including forms with multiple nuclei, prominent nucleoli, and cytoplasmic droplets containing Ig.

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50
Q

What’s this?

A

Plasma Cell Variants in Plasma Cell Dyscrasias “Flame Cell”

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51
Q

What’s this?

A

Plasma Cell Variants in Plasma Cell Dyscrasias

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52
Q

What’s this?

A

Plasma Cell Variants in Plasma Cell Dyscrasias “Russel Body” - Gihugnormous eosinophilic granule

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53
Q

What’s this?

A

Neoplastic dysmorphic multiple myeloma plasma cells fill the marrow.

Sheets of atypical plasma cells (plasmablasts)

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54
Q

What would you expect to see on a serum protein electrophoresis for M-protein detection in mult myeloma versus a sample of normal serum?

A

Polyclonal IgG in normal serum (arrow) appears as a broad band; in contrast, serum from a patient with multiple myeloma contains a single sharp protein band (arrowhead) in this region of the electropherogram

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55
Q

What lab test is used to screen for suspected mult myeloma? What confirms it?

A

M protein detection in multiple myeloma. Serum protein electrophoresis (SP) is used to screen for a monoclonal immunoglobulin (M protein).

The suspected monoclonal Ig is confirmed and characterized by immunofixation.

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56
Q

You order an electrophoresis for a suspected mult myeloma case, which comes back positive. What do you order to confirm it, and what do you expect to see?

A

Polyclonal IgG in normal serum (arrow) appears as a broad band; in contrast, serum from a patient with multiple myeloma contains a single sharp protein band (arrowhead) in this region of the electropherogram.

The suspected monoclonal Ig is confirmed and characterized by immunofixation. In this procedure, proteins separated by electrophoresis within a gel are reacted with specific antisera. After extensive washing, proteins that are cross-linked by antisera are retained and detected with a protein stain. Note the sharp band in the patient serum is cross-linked by antisera specific for IgG heavy chain (G) and kappa light chain (κ), indicating the presence of an IgGκ M protein. Levels of polyclonal IgG, IgA (A), and lambda light chain (λ) are also decreased in the patient serum relative to normal, a finding typical of multiple myeloma.

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57
Q

What are the clinical features of multiple myeloma? What symptoms can they cause?

A

Lytic bone lesions → pathologic fractures and substantial bone pain

Hypercalcemia (metastatic calcification)

Suppression of humoral immunity recurrent infections

Renal insufficiency

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58
Q

What is the second most common cause of death in patients with multiple myeloma? What causes this morbidity?

A

Renal Failure

Second only to infection as cause of death

Due to toxicity of hypercalcemia and Bence-Jones proteins

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59
Q

In addition to lytic bone lesions, hypercalcemia, suppression of humoral immunity, and renal insufficiency; what are some other clinical features associated with multiple myeloma?

A

Increased immunoglobulins (AL amyloidosis) and Bence-Jones proteins

Monoclonal Gammopathy:

IgG (60%)>IgA>IgM/IgD/IgE

(15-20% produce only light chains)

Hyperviscosity with IgA, IgG3 and IgM subtypes:

Visual impairment, neurologic symptoms, bleeding, cryoglobulinemia

1% produce no monoclonal protein

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60
Q

What is the untreated prognosis for mult myeloma?

A

Untreated survival is 6 – 12 months from diagnosis

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61
Q

What is the prognosis for mult myeloma if treated with chemo?

A

With chemotherapy, median survival is 4-6 years

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62
Q

What means a good prognosis with mult myeloma?

A

Seems like a bit of an oxymoron, but:

Cyclin D1 translocations - good prognosis

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63
Q

What is the relative mortality of mult myeloma, by age group?

A

Mirrors incidence with only a shift to the right of 5-6 years

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64
Q

What is the lymphoid neoplasm that has a much higher incidence and death rate in african-americans than caucasians?

A

Mult myeloma

65
Q

What are the 2 staging systems for mult myeloma? How many stages for each?

A

Salmon-Durie staging system

Stage I - Stage III, subclassification A and B too

International Staging System

Stages I - Stage III

66
Q

When does lymphoplasmacytic lymphoma typically present?

A

B-cell neoplasm of older adults; presents in 6th & 7th decade

67
Q

What is a common cause of Waldenstrom Macroglobinemia? What other Ig types are associated with this?

A

Lymphoplasmacytic Lymphoma:

Common cause of Waldenstrom Macroglobulinemia (IgM) but can also produce IgG or IgA

68
Q

What are some common morphological findings associated with lymphoplasmacytic lymphoma?

A

Morphology: lymphocytes, plasma cells, and intermediate forms; Immunoglobulin inclusions

(Russell bodies –cytoplasm;

Dutcher bodies-nucleus)

69
Q

What are the genetic and biochemical aberrations associated with lymphoplasmacytic lymphoma?

A

Genetics: MYD88 mutations leading to NF-κB activation

70
Q

What cell markers/immunophenotype does lymphoplasmacytic lymphoma typically express?

A

Immunophenotype: Positive for CD19, CD79a, surface and cytoplasmic immunoglobulins (IgM, IgD, IgG or IgA); also variable CD20, and CD38

71
Q

What are the clinical features of lymphoplasmacytic lymphoma?

A

Lymphadenopathy, hepatomegaly and splenomegaly

10% have RBC hemolysis

~50% have Hyperviscosity Syndrome from high levels IgM

72
Q

What are the signs and symptoms of Hyperviscosity Syndrome?

A

Hyperviscosity Syndrome from high levels IgM

Visual Impairment

Neurologic problems: dizziness, deafness & stupor

Bleeding

Cryoglobulinemia

No lytic bone lesions

Usually no Bence-Jones proteinuria or amyloidosis

73
Q

What is the prognosis for lymphoplasmacytic lymphoma?

A

Incurable with median survival 4-6 years from time of diagnosis

74
Q

What’s this?

A

Lymphoplasmacytoid Lymphoma

Bone marrow biopsy shows a characteristic mixture of small lymphoid cells exhibiting various degrees of plasma cell differentiation. In addition, a mast cell with purplish red cytoplasmic granules is present at the left-hand side of the field.

75
Q

What are the peripheral T-cell neoplasms?

A
  • Peripheral T-cell Lymphoma, unspecified
  • Anaplastic Large Cell Lymphoma
  • Adult T-cell Leukemia/Lymphoma
  • Mycosis Fungoides/Sezary Syndrome
  • Large Granular Lymphocytic Leukemia
  • Extranodal NK/T-cell Lymphoma
76
Q

What makes up 5-10% of non-Hodgkin lymphoma in US?

A

Peripheral T-cell and NK cell neoplasms

77
Q

Compared to B-cell neoplasms, how do peripheral T-cell and NK cell neoplasms compare in terms of aggressiveness and prognosis?

A

Much more aggressive neoplasms than B-cell neoplasms

Much worse prognosis than B-cell neoplasms

​

78
Q

Where do peripheral T cell and NK cell neoplasms occur, usually?

A

Occur Predominately in Extranodal Sites

79
Q

How are peripheral T cell and Nk cell neoplasms Dx’ed?

A

Diagnosis completely on flow cytometry marker studies

Exhibit T-cell surface CD markers

Lack TDT

May or may not express CD4 or CD8

80
Q

Characterize adult T cell leukemia/lymphoma according to:

cell of origin

genotype

salient clinical features

A

cell of origin - Helper T cell

genotype - HTLV-1 provirus present in tumor cells

salient clinical features - Adults with cutaneous lesions, marrow involvement, and hypercalcemia; occurs mainly in Japan, West Africa, and the Caribbean; aggressive

81
Q

Characterize peripheral T cell lymphoma, unspecified according to:

cell of origin

genotype

salient clinical features

A

cell of origin - Helper or cytotoxic T cell

genotype - No specific chromosomal abnormality

salient clinical features - Mainly older adults; usually presents with lymphadenopathy; aggressive

82
Q

Characterize anaplastic T cell lymphoma, unspecified according to:

cell of origin

genotype

salient clinical features

A

cell of origin - Cytotoxic T cell

genotype - Rearrangements of ALK in a subset

salient clinical features - Children and young adults, usually with lymph node and soft-tissue disease; aggressive

83
Q

Characterize extranodal NK/ T cell lymphoma according to:

cell of origin

genotype

salient clinical features

A

cell of origin - NK-cell (common) or cytotoxic T cell (rare)

genotype - EBV-associated; no specific chromosomal abnormality

salient clinical features - Adults with destructive extranodal masses, most commonly sinonasal; aggressive

84
Q

Characterize mycosis fungoides/Sezary Syndrome according to:

cell of origin

genotype

salient clinical features

A

cell of origin - Helper T cell

genotype - No specific chromosomal abnormality

salient clinical features - Adult patients with cutaneous patches, plaques, nodules, or generalized erythema; indolent

85
Q

Characterize large granular lymphocytic leukemia according to:

cell of origin

genotype

salient clinical features

A

cell of origin - Two types: cytotoxic T cell and NK cell

genotype - Point mutations in STAT3

salient clinical features - Adult patients with splenomegaly, neutropenia, and anemia, sometimes, accompanied by autoimmune disease

86
Q

What is a way to break down all the different types of T cell lymphomas?

A
87
Q

What does peripheral T cell lymphoma, unspecified usually present as?

A

•Present with lymphadenopathy and sometimes systemic signs (pruritis, fever, and weight loss)

88
Q

What is peripheral t cell lymphoma, unspecified usually associated with?

A

•T-cell lymphomas associated with lymph nodes

89
Q

How does the prognosis for peripheral T cell lymphoma, unspecified usually compare with B cell tumors? What is the typical survival?

A

•Significantly worse prognosis than comparable B-cell

•Survival < 1 year with treatment

90
Q

What cell surface proteins are typically expressed with peripheral T cell lymphoma, unspecified?

A

•CD2, CD3, CD5, αβ or γδ T-cell receptors, +/- CD4 or CD8

91
Q

What’s this?

A

Peripheral T-cell lymphoma, unspecified (lymph node).

A spectrum of small, intermediate, and large lymphoid cells, many with irregular nuclear contours, is visible

92
Q

How much is lymphoma due to anaplastic large cell lymphoma, in kids and adults?

A

•Approximately 10-20% of childhood lymphoma and <5% of adult lymphomas

93
Q

What are the genetics associated with anaplastic large cell lymphoma?

A

ALK+ or ALK-

94
Q

What is the pathogenesis behind ALK+ anaplastic large cell lymphoma?

A
  • ALK+ [anaplastic lymphoma kinase (ALK) gene (2p23)]
  • Rearrangement present more in children & young adults
  • results in fusion protein which activates tyrosine kinase (JAK/STAT)
  • Most common t(2;5) ALK and nucleophosmin (NPM) on chromosome 5
  • Excellent response to treatment when ALK+ (75% cured)
95
Q

What is the prognosis associated with ALK- anaplastic large cell lymphoma?

A
  • ALK-
  • Usually in older adults
  • poor prognosis ~other peripheral T-cell lymphomas
96
Q

What are the cell surface markers typically associated with anaplastic large cell lymphoma?

A

•Markers – CD30, CD2, CD4, CD45, CD25, +/- CD25, +/- ALK

97
Q

What’s this?

A

Anaplastic large cell lymphoma

Several “hallmark” cells with horseshoe-like or “embryoid” nuclei and abundant cytoplasm lie near the center of the field.

98
Q

What’s this?

A

Anaplastic large cell lymphoma

Immunohistochemical stain demonstrating the presence of ALK fusion protein

99
Q

When does adult T cell leukemia/lymphoma occur?

A

Only in adults infected with human T-cell leukemia virus Type 1 (HTLV-1)

100
Q

What is the pathogenesis behind adult T cell leukemia?

A

•Only in adults infected with human T-cell leukemia virus Type 1

(HTLV-1)

•Virus encodes Tax protein activating NF-κβ

101
Q

Where is adult t cell lymphoma endemic?

A

•Endemic in Southern Japan, West Africa & Caribbean

102
Q

What are the signs associated with adult t cell lymphoma?

A

•Hepatosplenomegaly, lymphadenopathy, skin lesions, lymphocytosis, hypercalcemia

103
Q

What is the progression and prognosis associated with adult t cell leukemia?

A
  • Rapidly progressive; fatal within months
  • Median survival 8 months
104
Q

What is a characteristic finding in adult t cell leukemia?

A

•Characteristic cloverleaf nucleii

105
Q

What’s this?

A

cloverleaf nuclei, associated with adult t cell leukemia/lymphoma

106
Q

What is a CD4+ T cell lymphoma of the skin? How does this happen?

A

mycosis fungoides

CLA, CCR4 & CCR10 expression leads to skin infiltration

107
Q

What are the characteristic histological findings associated with Mycosis Fungoides?

A

Lymphoma cells with ceribriform nuclei

108
Q

What are the clinical signs associated with mycosis fungoides? What is the typical prognosis?

A

Inflammatory (erythrodermic) pre-mycotic patch, plaque, & tumor phases

Aggressive neoplasm with median survival 8-9 years (M>F)

109
Q

What is Sezary Syndrome?

A

Mycosis Fungoides

Sezary syndrome is variant in which skin involvement is manifest as a generalized exfoliative erythroderma

110
Q

What is a phase of Sezary syndrome? What is the survival?

A

Leukemic phase with Sezary cell (cerebriform nuclei) seen in Sezary syndrome and 25% of plaque

→Survival <3 years

111
Q

Describe the histological findings associated with large granular lymphocytic leukemia.

A

•Lymphocytes with abundant blue cytoplasm containing scattered azurophilic granules

112
Q

What are the variants associated with large granular lymphocytic leukemia?

A
  • Variants
  • CD3+ T-cell (indolent)
  • CD56+ NK cell (aggressive)
113
Q

What are some clinical findings associated with large granular lymphocytic leukemia?

A
  • Neutropenia & anemia despite scant marrow involvement
  • Involves splenic red pulp and hepatic sinusoids (hepatomegaly)
  • Associated with rheumatologic disorders
114
Q

What is Felty syndrome?

A

Large Granular Lymphocytic Leukemia

•Associated with rheumatologic disorders - Felty syndrome in some (rheumatoid arthritis, splenomegaly and neutropenia)

115
Q

What’s this?

A

large granular lymphocytic leukemia

116
Q

What are some other names for extranodal NK/T cell lymphoma?

A

•P.K.A. lethal midline granuloma, midline malignant reticulocytosis and angiocentric lymphoma

117
Q

What happens, pathologically, in extranodal NK/T cell lymphoma?

A
  • Most CD56+ NK cell
  • Tumor cells typically surround and invade small vessels
118
Q

What does extranodal NK/T cell lymphoma typically present as?

A
  • Clinically presents as sinonasal lymphoma…..aggressive neoplasm poorly responsive to chemotherapy
  • Sometimes midline skin or testes involved
119
Q

What is an important association you should make with extranodal NK/T cell lymphoma?

A

•EBV related

120
Q

How many different diseases make up Hodgkin Lymphoma? How do they differ?

A

Classical Hodgkin Lymphoma

Nodular Lymphocyte predominance

Differ in clinical features and behavior, morphology, immunophenotype, and composition of cellular background

121
Q

How many subtypes make up classical hodgkin lymphoma? How are they different, and how are they the same?

A
  • Classical Hodgkin lymphoma (95%):
  • Nodular sclerosis (65-70%)
  • Mixed cellularity (20-25%)
  • Lymphocyte-rich (<5%)
  • Lymphocyte depleted (<5%)

differ in their sites of involvement, clinical features, growth pattern, presence of fibrosis, composition of cellular background, number or degree of atypia of tumor cells, and frequency of EBV infection

same immunophenotype though

122
Q

What’s this?

A

5 cm lymph node (obviously from a patient with lymphadenopathy). The node should normally be soft, pink-tan & < less 1 cm in size. This lymph node is involved with Hodgkin Disease.

123
Q

What is the molecular pathogenesis of hodgkins lymphoma?

A
  • Germinal center or post germinal center B-cell with V(D)J recombination and somatic hypermutation
  • Aneuploid
  • Activated transcription factor NF-κβ - NF-κB (nuclear factor kappa-light-chain-enhancer of activated B cells)
124
Q

What are some cross-talk signals between Reed-Sternberg cells and surrounding normal cells in classical Hodgkin Lymphoma?

A
125
Q

What is the common morphology and immunophenotype associated with nodular sclerosis subtype of Hodgkin Lymphoma?

A

Frequent lacunar cells and occasional diagnostic RS cells; background infiltrate composed of T lymphocytes, eosinophils, macrophages, and plasma cells; fibrous bands dividing cellular areas into nodules.

RS cells PAX5+, CD15+, CD30+; 33% EBV+*

126
Q

What are the typical clinical features associated with the nodular sclerosis subtype of Hodgkin Lymphoma?

Where does it typically manifest?

What is the most common grade?

A

Most common subtype; usually stage I or II disease; frequent mediastinal involvement; equal occurrence in males and females (F = M), most patients young adults

127
Q

What is the common morphology and immunophenotype associated with mixed cellularity subtype of Hodgkin Lymphoma?

A

Mixed cellularity (20-25%)

Frequent mononuclear and diagnostic RS cells; background infiltrate rich in T lymphocytes, eosinophils, macrophages, plasma cells;

RS cells PAX5+, CD15+, CD30+; 75% EBV+

128
Q

What are the typical clinical features associated with the mixed cellularity subtype of Hodgkin Lymphoma?

In what population is this the most common Hodgkins lymphoma?

What grades are most common?

A

More than 50% present as stage III or IV disease; M > F; biphasic incidence, peaking in young adults and again in adults older than 55 MOST COMMON FORM OF HODGKIN LYMPHOMA IN PATIENTS>50

129
Q

What is the common morphology and immunophenotype associated with Lymphocyte rich subtype of Hodgkin Lymphoma?

A

Lymphocyte rich (<5%)

Frequent mononuclear and diagnostic RS cells; background infiltrate rich in T lymphocytes;

RS cells PAX5+, CD15+, CD30+; EBV- (1 case)

130
Q

What are the typical clinical features associated with the lymphocyte rish subtype of Hodgkin Lymphoma?

A

Uncommon; M greater than F; tends to be seen in older adults

131
Q

What is the common morphology and immunophenotype associated with Lymphocyte depletion subtype of Hodgkin Lymphoma?

A

Reticular variant: Frequent diagnostic RS cells and variants and a paucity of background reactive cells;

RS cells PAX5+, CD15+, CD30+; 50% EBV+

132
Q

What are the typical clinical features of the lymphocyte depletion subtype of Hodgkin Lymphoma?

A

Uncommon; more common in older males, HIV-infected individuals, and in developing countries; often presents with advanced disease

133
Q

What is the morphology and immunophenotype associated with the lymphocyte predominance subtype of Hodgkin lymphoma?

A

Lymphocyte predominance (5%)

Frequent L&H (popcorn cell) variants in a background of follicular dendritic cells and reactive B cells;

RS cells CD20+, CD15-, C30-; EBV-, BCL6+

134
Q

What are the typical clinical features associated with lymphocyte predominance Hodgkin lymphoma?

In what body regions does this typically present?

A

Lymphocyte predominance (5%)

Uncommon; young males with cervical or axillary lymphadenopathy; mediastinal

135
Q

What’s this?

A

Diagnostic Reed-Sternberg cell, with two nuclear lobes, large inclusion-like nucleoli, and abundant cytoplasm, surrounded by lymphocytes, macrophages, and an eosinophil

CD30+, CD15+, CD20-, CD45-, PAX5+, +/- EBV

136
Q

What’s this?

A

Reed-Sternberg cell, mononuclear variant.

137
Q

What’s this?

A

Hodgkin lymphoma, nodular sclerosis type.

A low-power view shows well-defined bands of pink, acellular collagen that subdivide the tumor into nodules.

138
Q

What’s this?

A

Reed-Sternberg cell, lacunar variant (Nodular Sclerosis).

This variant has a folded or multilobated nucleus and lies within a open space, which is an artifact created by disruption of the cytoplasm during tissue sectioning

139
Q

What’s this?

A

Hodgkin lymphoma, mixed-cellularity type.

A diagnostic, binucleate Reed-Sternberg cell is surrounded by reactive cells, including eosinophils (bright red cytoplasm), lymphocytes, and histiocytes.

140
Q

What’s this?

A

Classical HL: Negative CD45 staining of RS cells and Variants

141
Q

What’s this?

A

Classical HL: CD30 membrane and paranuclear staining of RS cells and Variants

142
Q

What’s this?

A

Classical HL: Positive CD15 staining of RS cells and Variants

143
Q

What’s this?

A

Hodgkin disease, nodular lymphocyte-predominance

144
Q

What’s this?

A

Hodgkin lymphoma, lymphocyte predominance type.

Numerous mature-looking lymphocytes surround scattered, large, pale-staining lymphohistiocytic variants (“popcorn” cells).

145
Q

What’s this?

A

Reed-Sternberg cell, lymphohistiocytic variant.

“Popcorn cell” of nodular lymphocyte predominance subtype !

Several such variants with multiply infolded nuclear membranes, small nucleoli, fine chromatin, and abundant pale cytoplasm are present.

CD20+, CD 45+, BCL6+, CD15-, CD30-, EBV-

146
Q

What is the progression of clinical features of Hodgkin Lymphoma?

A

Lymphadenopathy at first

Later develop involvement of spleen, liver, marrow, etc.

B symptoms

LN, itching, abdominal pain, or vomiting with EtOH consumption

Cutaneous anergy (suppressed TH1 immune responses)

147
Q

What lymphodenopathies should you associate with each type of Hodgkin Lymphoma?

A
  • Lymphadenopathy at first:
  • Classical Hodgkin lymphoma
  • Cervical, mediastinal, axillary, para-aortic
  • Bimodal age distribution
  • Nodular lymphocyte predominance Hodgkin lymphoma
  • Cervical, axillary, inguinal
  • Young males < 35 years
148
Q

What do “B symptoms” include?

A

•Fever, drenching night sweats, weight loss

149
Q

What post treatment clinical features are associated with Hodgkin Lymphoma?

A

•Post-treatment develop myelodysplasia, leukemias, non-Hodgkin lymphoma, lung cancer, gastric cancer, sarcoma and melanoma

150
Q

What are the stages of Hodgkin and non-Hodgkin lymphomas?

A

Stages I-IV

All stages are further divided on the basis of the absence (A) or presence (B) of the following symptoms: unexplained fever, drenching night sweats, and/or unexplained weight loss of greater than 10% of normal body weight.

151
Q

What are the findings in stage I lymphoma?

A

Clinical Staging of Hodgkin and Non-Hodgkin Lymphomas (Ann Arbor Classification)

I

Involvement of a single lymph node region (I) or a single extra-lymphatic organ or site (IE).

152
Q

What are the findings in stage II lymphoma?

A

Clinical Staging of Hodgkin and Non-Hodgkin Lymphomas (Ann Arbor Classification)

II

Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or localized involvement of an extra-lymphatic organ or site (IIE).

153
Q

What are the findings associated with stage III lymphoma?

A

Clinical Staging of Hodgkin and Non-Hodgkin Lymphomas (Ann Arbor Classification)

III

Involvement of lymph node regions on both sides of the diaphragm without (III) or with (IIIE) localized involvement of an extra-lymphatic organ or site.

154
Q

What are the findings associated with stage IV lymphoma?

A

Clinical Staging of Hodgkin and Non-Hodgkin Lymphomas (Ann Arbor Classification)

IV

Diffuse involvement of one or more extra-lymphatic organs or sites with or without lymphatic involvement.

155
Q

What are the cure rates associated with stage I/IIA in Hodgkin Disease? Stage IV?

A

Hodgkin Disease Cure Rate Stage I/IIA almost 90%;

Stage IV 5 year survival 60-70%

156
Q

What are some major differences between Hodgkin and non-Hodgkin Lymphoma? Distinguish between:

age of onset

locality

spread

LNs involved

extranodal involvement

response to therapy

A
157
Q

What should you think of when asked about the age of initial diagnosis with Hodgkin Lymphoma?

A

“Bi-Modal Peaks” 20-29 years, > 70 years

158
Q

What should you think of when asked about age of initial diagnosis for non-Hodgkin Lymphoma?

A

Large majority after age 50

159
Q
A

Heme/Lymph - Exam 1 (5 decks)

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