WBC Hematopathology Part 1 Flashcards

1
Q

What technologies were needed to establish flow cytometry?

A
  1. Immunoflorescence
  2. Computer processing
  3. Monoclonal antibodies
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2
Q

What outbreak accelerated accpetance of lfow cytometry?

A

AIDS outbreak

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3
Q

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

A
  1. Peripheral blood
  2. bone marrow
  3. Solid tumor
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4
Q

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

A

Number and type of cell surface antigens

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5
Q

CD1-8 is antigen for what cells?

A

T Cells

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6
Q

CD 19-20 are antigens for what cells?

A

B cells

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7
Q

CD15 and 56 are antigens for what cells?

A

NK

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8
Q

CD45 is an antigen for what cells?

A

All lymphocytes

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9
Q

What is measured in flow cytometry?

A

Intensity of scattered light signals from each cell

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10
Q

In flow cytometry, what are the cells labeled with?

A

Fluorescent probe

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11
Q

How should flow cytometry be used clinically? 2

A
  1. Supplementary to confirm diagnoses made by conventional morphologic studies
  2. Provide prognostic information
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12
Q

Flow cytometry is best used how?

A

Technique to answer specific clinical questions

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13
Q

What should you do before flow cytometry?

A

Know what information is actually needed

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14
Q

What is immunophenotyping?

A

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

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15
Q

Antibodies with a similar antigenic reactivity were assigned to what?

A

A cluster

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16
Q

HLA-DR antibody is seen where?

A

In immature cells

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17
Q

Two purposes of analyzing DNA in tumor cells?

A
  1. Identify and quantitate the cell’s DNA content

2. Determine the distribution of cells within different phases of cell cycle

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18
Q

How is abnormal DNA content defined in cancer cells

A

Aneuploidy

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19
Q

What does aneuploidy reflect?

A

Chromosomal aberrations or mutations

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20
Q

S-phase analysis examines what?

A

Proliferative capacity of neoplastic cells

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21
Q

What makes an image of the cell cycle?

A

DNA histogram

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22
Q

What do you call not enough white blood cells?

A

Leukopenia

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23
Q

What do you call too many WBC’s?

A

Leukocytosis

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24
Q

Two types of leukocytosis?

A

Reactive

Neoplastic (leukemias/lymphomas)

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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