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Flashcards in WBC Hematopathology Part 1 Deck (223):
1

What technologies were needed to establish flow cytometry?

1. Immunoflorescence
2. Computer processing
3. Monoclonal antibodies

2

What outbreak accelerated accpetance of lfow cytometry?

AIDS outbreak

3

Solution of cells that can be ran through flow cytometry include? 3

1. Peripheral blood
2. bone marrow
3. Solid tumor

4

How can WBC populations and subpopulations be characterized in flow cytometry?

Number and type of cell surface antigens

5

CD1-8 is antigen for what cells?

T Cells

6

CD 19-20 are antigens for what cells?

B cells

7

CD15 and 56 are antigens for what cells?

NK

8

CD45 is an antigen for what cells?

All lymphocytes

9

What is measured in flow cytometry?

Intensity of scattered light signals from each cell

10

In flow cytometry, what are the cells labeled with?

Fluorescent probe

11

How should flow cytometry be used clinically? 2

1. Supplementary to confirm diagnoses made by conventional morphologic studies
2. Provide prognostic information

12

Flow cytometry is best used how?

Technique to answer specific clinical questions

13

What should you do before flow cytometry?

Know what information is actually needed

14

What is immunophenotyping?

Identification and classificaiton of cells by determining the presence of specific cell surface antigens

15

Antibodies with a similar antigenic reactivity were assigned to what?

A cluster

16

HLA-DR antibody is seen where?

In immature cells

17

Two purposes of analyzing DNA in tumor cells?

1. Identify and quantitate the cell's DNA content
2. Determine the distribution of cells within different phases of cell cycle

18

How is abnormal DNA content defined in cancer cells

Aneuploidy

19

What does aneuploidy reflect?

Chromosomal aberrations or mutations

20

S-phase analysis examines what?

Proliferative capacity of neoplastic cells

21

What makes an image of the cell cycle?

DNA histogram

22

What do you call not enough white blood cells?

Leukopenia

23

What do you call too many WBC's?

Leukocytosis

24

Two types of leukocytosis?

Reactive
Neoplastic (leukemias/lymphomas)

25

WBC cancers cause what % of deaths in adults?
In kids?

9% in adults
40% in kids

26

Two types of leukopenia?

1. Neutropenia
2. Agranulocytosis

27

Main cause of reactive leukocytosis?

Infectious mononucleosis

28

Three causes of reactive lymphadenitis?

1. Acute nonspecific lymphadenitis
2. Chronic nonspecific lymphadenitis
3. Cat scratch disease

29

What is the most common WBC cytopenia

Neutropenia

30

Two ways to get lymphocytopenia?

1. Congenital immunodeficiency disease
2. Acquired (HIV, steroids, drugs, autoimmune, malnutritions, viruses)

31

Neutropenia has a total neutrophil count of what?

Less than a 1000/uL

32

What problem does having neutropenia cause?

Increased susceptibility to infection

33

3 categories of etiologies of neutropenia?
Which is most common

1. Inadequate/ineffective granulopoiesis
2. Accelerated removal of neutrophils
3. Drug toxicity (most common cause)

34

What would cause inadequate/ineffective granulopoiesis? (3)

1. Suppression of myeloid stem cells (aplastic anemia, leukemia)
2. Suppression of granulocyte precursors by drugs or chemo
3. Ineffective granulopoiesis due to megaloblastic anemia or myelodysplasia

35

Causes of accelerated removal/destruction of neturophils? 3

1. Immune mediated
2. Used up peripherally to fight infection
3. Splenic sequestration (hypersplenism)

36

Drug toxicity is related to what 3 drugs?

1. Chemo
2. Chloramphenicol
3. Sulfonamides

37

How does neutropenia present?

Flu-like

38

How serious can neutropenia get?

Fatal in hours if drops below 500/mm

39

Major complication of neutropenia?

Infection

40

Treatment for neutropenia? (3)

1. Recombinant hematopoietic growth factors (G-CSF, neupogen)
2. Antibiotics
3. Supportive care

41

Reactive leukocytosis is a common reaction to what?

Infectious/inflammatory stimuli

42

Reactive leukocytosis is classified how?

What particular white cell line is affected

43

What might reactive leukocytosis mimic?

Leukemia

44

Neutrophils are distributed in what five pools?

1. Precursor pool
2. Storage pool
3. Marginating pool
4. Circulating pool
5. Tissue pool

45

Bone marrow pool is comparable to peripheral blood pool how for neutrophils?

50X peripheral blood pool

46

Circulating pool of neutrophils is measured by what?

WBC count

47

Glucocorticoids have what effect on neutrophils?
How is this detected?

Decrease extravasation into tissues
Increased WBC count

48

Neutrophilic leukocytosis causes (7)

1. Acute bacterial infections
2. Sterile inflammation by tissue necrosis
3. Myeloproliferative disorders
4. Malignancy in marrow
5. Physiologic response to stress
6. Hemorrhage
7. Medications

49

Eosinophilic leukocytosis causes? (5)

1. Allergic disorders like asthma
2. Skin diseases (dermatitis)
3. Parasites
4. Drug reactions
5. Hodgkin/Non-hodgkin lymphomas

50

Basophilic leukocytosis cause? 1

Indicates myseloproliferative disease

51

Monocytosis cause? 3

1. Chronic infections (TB, endocarditis, rickettsia, malaria
2. Collagen vascular disease (SLE)
3. Inflamatory bowel disease

52

Lymphocytosis causes? 3

1. With monocytosis in chronic immune disease
2. Viral infections
3. Bordatella pertussis

53

Leukoerythroblastosis means what?

Presence of nucleated RBC's and left shift of the neutrophils (immature neutrophils in blood)

54

Toxic change in PMN's in neutrophilia? 3

Toxic granulation
Dohle bodies
Cytoplasmic vacuoles

55

Infectious mononucleosis is seen in what population?

Adolescents and young adults

56

Cause of infectious mono? 2

B-cell lymphocytotropic EBV
CMV

57

Symptoms of EBV? (7)

1. Fever
2. Sore throat
3. Generalized lymphadenitis
4. Atypical lymphocytes in peripheral blood
5. Lymph node changes
6. Splenomegaly
7. Hepatitis

58

Reactive lymphocytosis may resemble what other disease?

Leukemia

59

Lymph node changes in infectious mono may mimic what?

Lymphoma

60

Diagnosis of infectious mono? (3)

1. Lymphocytosis with atypical cells
2. Positive monospot test
3. Specific antibodies for EBV

61

How long does infectious mono last?

4-6 weeks

62

Reactive lymphadenitis is defined how?

Immune response against foreign antigens with lymph node enlargement

63

Acute nonspecific lymphadenitis may be localized where?
Or generalized why?

Local = Focal infection

Generalized = Systemic viral infection or bacteremia

64

Usual etiology of acute non-spec lymphadenitis?
Leads to what histologic change?

Bacterial

Follicular hyperplasia with large germinal centers

65

Main symptom of acute nonspecific lymphadenitis?

Nodes affected are enlarged and painful with abscess penetrating to the skin

66

Two special kinds of acute nonspecific lymphadenitis?

1. Bubos
2. Scrofula

67

What are bubos?

Enlarged lymph nodes most often associated with bubonic plague

68

What is scrofula?

Cervical lymphadenitis caused by mycobacteria

69

Adult cause of scrofula?
Children cause?

Adult = M. tuberculosis
Kids = Atypical tuberculosis

70

Chronic nonspecific lymphadenitis has what three forms?

1. Follicular hyperplasia
2. Paracortical hyperplasia
3. Sinus histiocytosis

71

Causes of paracortical hyperplasia? (3)

1. Virus like mono
2. Post vaccination
3. Dilantin

72

Are lymph nodes painful in chronic nonspecific lymphadenitis?
Where are the main sites?

No

Inguinal and axillary

73

Cat scratch fever is caused by what?

Bartonell ahenselae

74

Symptoms of cat scratch fever (2)?

1. Regional lymphadenopathy
2. Stellate necrotizing granulomas

75

Diagnosis of cat scratch fever? 3

1. Exposure to cats
2. Clinical findings
3. Positive skin test

76

Lymphoid neoplasms are derived from what?

Lynphocytes and their precursors

77

4 types of lymphoid neoplasms?

1. Acute leukemias (immature lymphocytes ALL)
2. Chronic leukemias (mature lymphocytes CLL)
3. Plasma cell dyscrasias (multiple myeloma)
4. Lymphomas (H and Non-H)

78

Myeloid neoplasms are derived from what?

Stem cell that gives rise to granulocytes, RBC's, platelets

79

2 types o fmyeloid neoplasms?

1. Acute leukemias (AML)
2. Chronic disorders

80

Histiocytic neoplasms are proliferations of what?

Histiocytes (macrophages) and dendritic cells

81

Main histiocytic neoplasm?

Langerhans cell histiocytosis

82

6 causes of neoplastic disorders?

1. Chromosomal tranlocations/Oncogenes
2. Inherited genetic factors
3. Viruses
4. Localized chronic immune stimulation
5. Iatrogenic factors
6. Smoking

83

How does the mutation of proto-oncogenes occur

Errors in lymphoid cells during antigen receptor gene rearrangement and diversification/antibody development

84

Oncoproteins have what 2 effects?

1. Block normal maturation
2. Give inappropriate proliferative signals

85

Development of neoplasia requires what?

Combination of multiple genetic lesions

86

Inherited genetic factors are seen in what two neoplastic disorders? 2

Down's syndrome (ALL)
Fanconi anemia

87

3 viruses and the WBC neoplasm they cause

1. HTLV-1 --> Adult T-cell leukemia/lymphoma
2. EBV: Burkitt, Hodgkin, B-cell lymphomas
3. KSHV/HHV8: B-cell primary effusion lymphoma

88

Environmental factors that cause WBC neoplasms? 2

1. H. pylori --> Gastric B cell lymphoma
2. Gluten sensitive enteropathy --> Intestinal T-cell lymphomas

89

Iatrogenic factors include what? (2)

Radiation
Chemotherapy

90

Smoking increases what neoplasm?

AML

91

What is common of cells in a lymphoid neoplasm? (2)

1. Arrested at particular stage
2. Reside in lymph nodes, peripheral blood, BM, or a combination

92

If a neoplasm is defined to tissue masses it is called? (Outside marrow)

Lymphoma

93

If a neoplasm is confined to peripheral blood and/or bone marrow is it called? (In Marrow)

Leukemia

94

What do most incurable lymphomas evolve into?

Leukemia

95

Tumors identical to leukemia sometimes arise as what?

Soft tissue masses with NO BM involvement

96

Hodgkin lymphoma is different for what main histologic difference?

Reed-Sternberg cell

97

Plasma cell neoplasms (dyscriasis) are commonly found in lymph nodes and peripheral blood?
What is their pathology related to?
Main thing they cause?

No they are not. Rarely

Ig secretion

Lytic bone lesions

98

What dictates clinical presentation of lymphoid neoplasms?

Anatomic distribution of disease

99

What proportion of lymph neoplasm is nodal?
What proportion is etranodal?
Presentation of each

2/3 nodal: No pain lymphadenopathy
1/2 extranodal: Tissue masses

100

What is required for diagnosis of lymphoid neoplasm?

Histologic examination of lymph nodes or tissues

101

What do all lymphoid neoplasms demostrate?
What does this mean?
How can this be determined?
Why does this work?

Clonality

Derived from single transformed cell = Monoclonal

Analysis of antigen receptor proteins

Gene rearrangement produces unique DNA sequence (monoclonal will have same)

102

80-85% of lymphoid neoplasms are of what origin?
What are the rest?

B-Cell origin

Rest are T cell, a few rare NK

103

What is a follicular lymphoma?

If the B-cell lymphoma recapitulate the follicular growth pattern of normal B cells

104

What is diffuse lymphoma?

B and T-cell neoplasms spread diffusely in the lymph node

105

If you have an effaced lymph node, how does that help your diagnosis?

Exclude lymphoma

106

Tumors of immune system will have what effect?
How does this present (2)

Disruption of normal immune regulatory mechanisms

1. Immunodeficiency
2. Autoimmunity

107

In terms of location, how are Non-Hodgkin lymphomas described?
Is staging important?

Present as particular tissue site, but actually widely disseminated and systemic

Staging not very important

108

How do you generally treat NHL's?

Systemic therapies

109

Hodgkin lymphomas are described how in terms of location?
Is staging important?

Present in single site and spread to contiguous lymph node groups.
Yes staging is important

110

What might cure hodgkin lymphoma?

Excision and local radiation

111

What is the progression of B cells with locations

Bone marrow: HSC --> CLP --> Pro B --> Pre B
Peripheral blood: Mature B --> Plasmacytoid B cell
Lymph nodes bone marrow: Plasma cell

112

What is progression of T cells with locations

Bone marrow: HSC --> CLP --> T/NK
Thymus: Pro-T --> Pre-T --> Intrathymic DP -->
1. Posthymic SP --> Peripheral Blood Th
2. Postthymic SP --> Peripheral blood Tc

113

Myeloid CD's? (3)

CD13-CD15

114

In acute leukemias what happens in differentiation?

There is a block so that the bad blasts have a prolonged generation time

115

What causes accumulation of blasts in marrow? 2
What is this associated with?

1. Clonal expansion
2. Failure of maturation

Suppression of normal hematopoiesis

116

How does acute leukemia generally present?

Symptoms? (4)

Abrupt stormy onset

1. Anemia --> Fatigue
2. Leukopenia --> Fever and infection
3. Thrombocytopenia --> Bruising/bleeding
4. Bone pain and tenderness

117

What are some acute leukemia symptoms seen mainly in ALL? 5

1. Lymphaenopathy
2. Hepatosplenomegaly
3. Testicular involvement
4. Compression of BV's and airways
5. CNS manifestations

118

CNS manifestations are seen mainly in what demographic?

Children

119

Lab findings of acute leukemias? 4

1. Anemia
2. Thrombocytopenia
3. WBC's greater than 100,000
4. Blasts in peripheral blood and bone marrow

120

What is the diagnostic feature of AL?

20-100% of cells in peripheral blood and bone marrow being blasts

121

Aleukemic leukemia presents how?

Pancytopenia with rare blasts in peripheral blood

122

Why is it important to differentiate ALL from AML?

Treatment

123

Precursor B- and T- cell leukemia/lymphoma is composed of what cells?
What age group?

Lymphoblasts
Children and young adults

124

Pre-B lymphoblastc tumors present in what form?
With what involvement?

Leukemias

Bone marrow and peripheral blood

125

Pre-T lymphoblastic tumors commonly present how?
Progress to what?

Mediastinal masses involving thymus

Leukemia

126

B and T cell tumors usually appear as what at some time in their course?

Acute Lymphoblastic Leukemia

127

ALL is the most common cancer of what?

Children

128

Pre-B ALL peaks at what age?

3

129

Pre-T cell tumors are more common with who?

Adolescent males

130

Pre-B cell tumors have what antigens? 2

CD19 and CD10

131

Pre-T cell tumors have what antigens? 4
Later which ones (3)

CD1, CD2, CD5, CD7

CD3, CD4, CD8

132

What enzyme is present in both Pre-B and Pre-T?

TdT

133

90% of precursor T and B tumors have what genetic change?
More common with which one?

Nonrandom karyotypic abnormalities

B cell (hyperdiploidy)

134

Which is more severe, nonrandom karyotypic abnormalities or other?

Other

135

Who has the best prognosis of all precursor T and B cell tumors?

Children 2-10 with pre-B cell tumor

136

What % of kids get complete remission of Pre-B cell tumor?

95%

137

11 Peripheral B-cell neoplasms

1. Small lymphocytic lymphoma/chronic lymphocytic leukemia
2. Follicular lymphoma
3. diffuse large B-cell lymphoma
4. Burkitt lymphoma/leukemia
5. mantle cell lymphoma
6. Marginal zone lymphoma
7. Hairy Cell leukemia
8. Plasma cell neoplasia
9. Lymphoplasmacytic lymphoma
10. Heav-chain disease
11. Primary/Immunocyte associated amyloidosis

138

Small lymphocytic lymphoma and chronic lymphocytic leukemia are related how?

CLL is circulating SLL

139

Special title of Small lymphocytic lymphoma/chronic lymphocytic leukemia?

Most common leukemia in Western world

140

Small lymphocytic lymphoma/chronic lymphocytic leukemia Age?
Sex?

60
Males more common

141

Small lymphocytic lymphoma/chronic lymphocytic leukemia appears how in peripheral blood?

Absolute lymphocytosis with smudge cells

142

Immunophenotype of Small lymphocytic lymphoma/chronic lymphocytic leukemia?

1. Pan-B markers (CD19 and 20)
2. Monoclonal kappa or delta light chains
3. CD23
4. CD5

143

CD23 appears in Small lymphocytic lymphoma or chronic lymphocytic leukemia

SLL

144

Status of B cells in Small lymphocytic lymphoma/chronic lymphocytic leukemia

Long lived, but nonfunctional B cells infiltrate BM, LN's, peripheral blood due to apoptosis defects

145

Clinical features of Small lymphocytic lymphoma/chronic lymphocytic leukemia? (2)

1. Asymptomatic at diagnosis
2. Autoimmune hemolytic anemia/thrombocytopenia

146

Survival length of Small lymphocytic lymphoma/chronic lymphocytic leukemia?

4-6 years

147

What does CLL/SLL transform into over time?

Aggressive tumors such as diffuse large B-cell lymphoma (Richter) or prolymphocytic leukemia

148

Key histopathic finding in SLL/CLL?

1. Smudge cells

149

Follicular lymphoma makes up what % of adult NHL's?

45%

150

FL arises from what?

Germinal center B cell

151

Markers for FL? (5)

1. Pan B markers (CD19,CD20)
2. CD10
3. BCL6
4. BCL2
5. No CD5

152

Folicular lymphoma age?
Sex?

Middle aged

Equal

153

How does FL present? (4)

1. Painless
2. Generalized lymphadenopathy
3. Bone marrow involved
4. t(14,18) --> Overexpress BCL2

154

Prognosis of FL?
Some progress to?

7-9 years

Diffuse large B-cell lymphoma

155

What is most common form of Large B-cell lymphoma?

Diffuse Large B-cell lymphoma

156

3 characteristics of Diffuse Large B-cell lymphoma?

1. Diffuse growth pattern
2. Aggressive
3. Heterogeneous in terms of pathogenesis

157

Markers for Diffuse Large B-cell lymphoma? 2

1. Pan B (CD19, CD20)
2. Variable after that due to different subtypes

158

Diffuse Large B-cell lymphoma age?

60 median age

159

How does Diffuse Large B-cell lymphoma present? (3)

1. Rapidly enlarging mass at single node or extranodal site
2. Spleen, liver involved
3. Bone marrow not involved initially
4. Aggressive

160

Result of Diffuse Large B-cell lymphoma if not treated?
If treated?
What % can be cured?

Rapidly fatal

Complete remission in 60-80%

50% can be cured

161

Burkitt lymphoma/leukemia (B-cell leukemia) has what special title?

Fastest growing human neoplasm

162

Burkitt lymphoma/leukemia (B-cell leukemia) histologic pattern?

Starry sky

163

Where are most Burkitt lymphoma/leukemia (B-cell leukemia) located?

Extranodal

164

Burkitt lymphoma/leukemia (B-cell leukemia) age?
Main involvement location in US?
In africa?

30% of childhood NHL's

Visceral (GI and sinus)

Jaw involvement

165

Burkitt lymphoma/leukemia (B-cell leukemia) markers? 5

1. CD19
2. CD20
3. CD10
4. BCL6
5. Translocations of c-MYC gene on Ch 8

166

Three translocations possible of c-MYC gene?
What is the other gene involved

1. t(8:14) and Ig Heavy chain (Most common)
2. t(2:8) and Ig light chain
3. t(8:22) and Ig light chain

167

All endemic Burkitt lymphoma/leukemia (B-cell leukemia) tumors are infected with what?

EBV

168

Majority of Burkitt lymphoma/leukemia (B-cell leukemia) can be cured via what?

Aggressive chemotherapy

169

Mantle Cell lymphoma is seen in what age group?

Middle aged males

170

Mantle Cell lymphomas are composed of what?

B cells resembling those in mantle zone surrounding normal lymphoid follicles

171

Majority of Mantle Cell lymphoma patients have what at diagnosis?
What else is typically involved

Generalized adenopathy

Extranodal sites: BM, spleen, liver, GI

172

Markers for Mantle Cell lymphoma?

1. CD19/CD20
2. CD5
3. CD23 negative
4. Overexpression of cyclin D1

173

How to differentiate between Mantle Cell lymphoma and SLL/CLL?

CD23 positive in SLL/CLL
CD23 negative in mantle cell lymphoma

174

Most Mantle Cell lymphoma have what translocation?
Effect?

t(11;14): IgH locus on 14 and cyclin D1 locus on 11

Increases cyclin D1 --> Promote G1 to S progression

175

Most Mantle Cell lymphoma present how?

Painless lymphadenopathy

176

Mantle cell lymphoma has what prognosis?

Aggressive and incurable with survival of 3-4 yeras

177

Marginal zone lymphoma arises where? 3

Lymph nodes
spleen
MALT (salivary glands, intestines, thyroid)

178

What bacteria commonly accompanies marginal zone lymphoma?

H. pylori

179

Extra-nodal marginal zone lymphoma is seen with what 3 associations?

1. Sjogren
2. Hashimoto thyroiditis
3. H. pylori

180

Extra-nodal marginal zone lymphoma occurs where?

MALT

181

Extra-nodal marginal zone lymphoma may regress if what happens?

Inciting agent removed

182

Extra-nodal marginal zone lymphoma may transition between what?

Reactive lymphoid hyperplasia and B-cell lymphoma

183

How to differentiate between acute leukemia and chronic?

Acute = 20% blasts
Chronic = <20% blasts

184

Hair cell leukemia is most commonly seen in who?

Middle-aged caucasian males

185

Histo sign?

Peripheral blood leukemic cells have fine hair-like cytoplasmic projections

186

Hairy cell leukemia markers? (5)

1. CD19
2. CD20
3. CD11c
4. CD25
5. CD103

187

Hairy cell leukemia triad of symptoms?

1. Splenomegaly
2. Pancytopenia
3. Dry tap on bone marrow aspiration

188

What treatment is Hairy cell leukemia really sensitive to?

Chemotherapy

189

Prognosis if treated?

Excellent with possible curable relapses

190

Hairy cell leukemia stains best with what stain?

TRAP (tartrate resistant acid phosphatase)

191

Plasma cell neoplasms are defined how?
Determined how?
Another name for this (2)

B-cell neoplasms of single clone of immunoglobin-secreting cells

Increase in serum levels of a single homogeneous Ig or its fragments (M component or monoclonal spike)

Monoclonal gammopathy or paraproteinemia

192

Neoplastic plasma cells have what main finding?

Bence-Jones proteinuria due to a poorly balanced synthesis of heavy and light chain. BJ is light chains in urine

193

When are the M components of plasma cell neoplasms normally seen in healthy people?

What is this called?

Elderly patients

Monoclonal gammopathy of undetermined significance (MGUS)

194

3 forms of plasma cell neoplasms to know?

1. Plasma cell myeloma/Multiple myeloma
2. Solitary plasmacytoma
3. MGUS

195

Plasma cell myeloma has what special title?

Most common plasma cell dyscrasias

196

Prognosis of plasma cell myeloma?

204 years

197

What proliferates in plasma cell myeloma?

Neoplastic plasma cells in BM

198

What physical finding is associated with plasma cell myeloma?

How does this present (2)?

Multifocal lytic lesions of axial skeleton

Pathologic fractures
Hypercalcemia

199

The M component of plasma cell myeloma is made up of what two Ig's mainly?

What % is Kappa or light chains?

What are these called in urine?

What % have both M component and Bence-Jones?

Half IgG and a quarter IgA

20% is light chains

Bence-Jones proteins

60-70%

200

Age of plasma cell myeloma?
Sex?

65-70
M>F

201

Clnical features of plasma cell myeloma occur for what reasons (3)

1. Effects of infiltration of organs and bones by the bad plasma cells
2. Production of excessive Ig's
3. Suppression of normal immunity

202

4 main findings of plasma cell myeloma?

1. Bone resoprtion
2. Renal insufficiency (BJ proteinuria is damaging)
3. Amyloidosis (Due to BJ)
4. Anemia/thrombocytopenia

203

What is the mnemonic to remember for organs involved in plasma cell myeloma?

CRAB

hyperCalcemia
Renal insufficiency
Anemia
Bone lesions

204

plasma cell myeloma peripheral blood finding? 2

Rouleaux formation
Mott Cells with Russell bodies

205

Prognosis of plasma cell myeloma?
If treated?

Poor

Remission but survival doesn't change

206

Localized plasmacytoma has what definition?

presence of a single lesion in skeleton or soft tissues

207

Most Localized plasmacytoma patients develop what?

Disseminated disease (myeloma)

208

What type of Localized plasmacytomas spread less commonly and can be cured locally?

Extraosseous plasmacytomas

209

MGUS is seen with what age group?

elderly

210

Diagnosis of MGUS should be made after what?

Excluding all other specific monoclonal gammopathies

211

How do you care for MGUS patient?

Keep assessing M component and Bence Jones levels

212

Lymphoplasmacytic lymphoma is defined how?

Mixed proliferation of B cells from small round lymphocytes to plasmacytoid lymphocytes to plasma cells

213

What is the M component of Lymphoplasmacytic lymphoma usually?
What happens if this gets high?

IgM

Hyperviscosity syndrome called Waldenstrom Macroglobulinemia (WM)

214

Lymphoplasmacytic lymphoma resembles other B-cell lymphomas in what presentations (4)

1. Diffuse infiltration of BM
2. Diffuse infiltration of LN's
3. Diffuse infiltration of liver and spleen
4. Autoimmune hemolysis via cold agglutinins

215

Prognosis for Lymphoplasmacytic lymphoma?

Incurable and progressive

216

Hyperviscosity syndrome is due to what?

Increased monoclonal IgM

217

4 manifestations of hyperviscosity syndrome?

1. Visual impairment
2. Neurologic problems
3. Bleeding
4. Cryoglobulinemia

218

Heavy-chain disease definition?

Only heavy chains produced of IgG, IgA, or IgM

219

How does IgG Heavy-chain disease present?

Diffuse lymphadenopathy with hepatosplenomegaly

220

How does IgA heavy-chain disease present?

Goes for the MALT

221

Primary or immunocyte-assicated amyloidosis is defined how?

Form of amyloidosis where monoclonal plasma cells produce excessive amounts of light chains

222

The amyloid deposits in Primary or immunocyte-assicated amyloidosis consist of what?

Partially degraded light chains --> Beta pleated sheets --> Amyloid

223

Effect of amyloid in Primary or immunocyte-assicated amyloidosis accumulating in organs?

Dysfunction