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Pathology > WBC Hematopathology Part 1 > Flashcards

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
Q

WBC cancers cause what % of deaths in adults?

In kids?

A

9% in adults

40% in kids

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26
Q

Two types of leukopenia?

A
  1. Neutropenia

2. Agranulocytosis

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27
Q

Main cause of reactive leukocytosis?

A

Infectious mononucleosis

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28
Q

Three causes of reactive lymphadenitis?

A
  1. Acute nonspecific lymphadenitis
  2. Chronic nonspecific lymphadenitis
  3. Cat scratch disease
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29
Q

What is the most common WBC cytopenia

A

Neutropenia

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30
Q

Two ways to get lymphocytopenia?

A
  1. Congenital immunodeficiency disease

2. Acquired (HIV, steroids, drugs, autoimmune, malnutritions, viruses)

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31
Q

Neutropenia has a total neutrophil count of what?

A

Less than a 1000/uL

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32
Q

What problem does having neutropenia cause?

A

Increased susceptibility to infection

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33
Q

3 categories of etiologies of neutropenia?

Which is most common

A
  1. Inadequate/ineffective granulopoiesis
  2. Accelerated removal of neutrophils
  3. Drug toxicity (most common cause)
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34
Q

What would cause inadequate/ineffective granulopoiesis? (3)

A
  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
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35
Q

Causes of accelerated removal/destruction of neturophils? 3

A
  1. Immune mediated
  2. Used up peripherally to fight infection
  3. Splenic sequestration (hypersplenism)
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36
Q

Drug toxicity is related to what 3 drugs?

A
  1. Chemo
  2. Chloramphenicol
  3. Sulfonamides
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37
Q

How does neutropenia present?

A

Flu-like

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38
Q

How serious can neutropenia get?

A

Fatal in hours if drops below 500/mm

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39
Q

Major complication of neutropenia?

A

Infection

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40
Q

Treatment for neutropenia? (3)

A
  1. Recombinant hematopoietic growth factors (G-CSF, neupogen)
  2. Antibiotics
  3. Supportive care
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41
Q

Reactive leukocytosis is a common reaction to what?

A

Infectious/inflammatory stimuli

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42
Q

Reactive leukocytosis is classified how?

A

What particular white cell line is affected

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43
Q

What might reactive leukocytosis mimic?

A

Leukemia

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44
Q

Neutrophils are distributed in what five pools?

A
  1. Precursor pool
  2. Storage pool
  3. Marginating pool
  4. Circulating pool
  5. Tissue pool
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45
Q

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

A

50X peripheral blood pool

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46
Q

Circulating pool of neutrophils is measured by what?

A

WBC count

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47
Q

Glucocorticoids have what effect on neutrophils?

How is this detected?

A

Decrease extravasation into tissues

Increased WBC count

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48
Q

Neutrophilic leukocytosis causes (7)

A
  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
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49
Q

Eosinophilic leukocytosis causes? (5)

A
  1. Allergic disorders like asthma
  2. Skin diseases (dermatitis)
  3. Parasites
  4. Drug reactions
  5. Hodgkin/Non-hodgkin lymphomas
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50
Q

Basophilic leukocytosis cause? 1

A

Indicates myseloproliferative disease

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51
Q

Monocytosis cause? 3

A
  1. Chronic infections (TB, endocarditis, rickettsia, malaria
  2. Collagen vascular disease (SLE)
  3. Inflamatory bowel disease
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52
Q

Lymphocytosis causes? 3

A
  1. With monocytosis in chronic immune disease
  2. Viral infections
  3. Bordatella pertussis
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53
Q

Leukoerythroblastosis means what?

A

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

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54
Q

Toxic change in PMN’s in neutrophilia? 3

A

Toxic granulation
Dohle bodies
Cytoplasmic vacuoles

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55
Q

Infectious mononucleosis is seen in what population?

A

Adolescents and young adults

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56
Q

Cause of infectious mono? 2

A

B-cell lymphocytotropic EBV

CMV

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57
Q

Symptoms of EBV? (7)

A
  1. Fever
  2. Sore throat
  3. Generalized lymphadenitis
  4. Atypical lymphocytes in peripheral blood
  5. Lymph node changes
  6. Splenomegaly
  7. Hepatitis
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58
Q

Reactive lymphocytosis may resemble what other disease?

A

Leukemia

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59
Q

Lymph node changes in infectious mono may mimic what?

A

Lymphoma

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60
Q

Diagnosis of infectious mono? (3)

A
  1. Lymphocytosis with atypical cells
  2. Positive monospot test
  3. Specific antibodies for EBV
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61
Q

How long does infectious mono last?

A

4-6 weeks

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62
Q

Reactive lymphadenitis is defined how?

A

Immune response against foreign antigens with lymph node enlargement

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63
Q

Acute nonspecific lymphadenitis may be localized where?

Or generalized why?

A

Local = Focal infection

Generalized = Systemic viral infection or bacteremia

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64
Q

Usual etiology of acute non-spec lymphadenitis?

Leads to what histologic change?

A

Bacterial

Follicular hyperplasia with large germinal centers

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65
Q

Main symptom of acute nonspecific lymphadenitis?

A

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

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66
Q

Two special kinds of acute nonspecific lymphadenitis?

A
  1. Bubos

2. Scrofula

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67
Q

What are bubos?

A

Enlarged lymph nodes most often associated with bubonic plague

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68
Q

What is scrofula?

A

Cervical lymphadenitis caused by mycobacteria

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69
Q

Adult cause of scrofula?

Children cause?

A 
Adult = M. tuberculosis
Kids = Atypical tuberculosis
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70
Q

Chronic nonspecific lymphadenitis has what three forms?

A
  1. Follicular hyperplasia
  2. Paracortical hyperplasia
  3. Sinus histiocytosis
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71
Q

Causes of paracortical hyperplasia? (3)

A
  1. Virus like mono
  2. Post vaccination
  3. Dilantin
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72
Q

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

A

No

Inguinal and axillary

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73
Q

Cat scratch fever is caused by what?

A

Bartonell ahenselae

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74
Q

Symptoms of cat scratch fever (2)?

A
  1. Regional lymphadenopathy

2. Stellate necrotizing granulomas

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75
Q

Diagnosis of cat scratch fever? 3

A
  1. Exposure to cats
  2. Clinical findings
  3. Positive skin test
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76
Q

Lymphoid neoplasms are derived from what?

A

Lynphocytes and their precursors

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77
Q

4 types of lymphoid neoplasms?

A
  1. Acute leukemias (immature lymphocytes ALL)
  2. Chronic leukemias (mature lymphocytes CLL)
  3. Plasma cell dyscrasias (multiple myeloma)
  4. Lymphomas (H and Non-H)
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78
Q

Myeloid neoplasms are derived from what?

A

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

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79
Q

2 types o fmyeloid neoplasms?

A
  1. Acute leukemias (AML)

2. Chronic disorders

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80
Q

Histiocytic neoplasms are proliferations of what?

A

Histiocytes (macrophages) and dendritic cells

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81
Q

Main histiocytic neoplasm?

A

Langerhans cell histiocytosis

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82
Q

6 causes of neoplastic disorders?

A
  1. Chromosomal tranlocations/Oncogenes
  2. Inherited genetic factors
  3. Viruses
  4. Localized chronic immune stimulation
  5. Iatrogenic factors
  6. Smoking
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83
Q

How does the mutation of proto-oncogenes occur

A

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

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84
Q

Oncoproteins have what 2 effects?

A
  1. Block normal maturation

2. Give inappropriate proliferative signals

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85
Q

Development of neoplasia requires what?

A

Combination of multiple genetic lesions

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86
Q

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

A

Down’s syndrome (ALL)

Fanconi anemia

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87
Q

3 viruses and the WBC neoplasm they cause

A
  1. HTLV-1 –> Adult T-cell leukemia/lymphoma
  2. EBV: Burkitt, Hodgkin, B-cell lymphomas
  3. KSHV/HHV8: B-cell primary effusion lymphoma
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88
Q

Environmental factors that cause WBC neoplasms? 2

A
  1. H. pylori –> Gastric B cell lymphoma

2. Gluten sensitive enteropathy –> Intestinal T-cell lymphomas

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89
Q

Iatrogenic factors include what? (2)

A

Radiation

Chemotherapy

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90
Q

Smoking increases what neoplasm?

A

AML

91
Q

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

A
  1. Arrested at particular stage

2. Reside in lymph nodes, peripheral blood, BM, or a combination

92
Q

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

A

Lymphoma

93
Q

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

A

Leukemia

94
Q

What do most incurable lymphomas evolve into?

A

Leukemia

95
Q

Tumors identical to leukemia sometimes arise as what?

A

Soft tissue masses with NO BM involvement

96
Q

Hodgkin lymphoma is different for what main histologic difference?

A

Reed-Sternberg cell

97
Q

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

A

No they are not. Rarely

Ig secretion

Lytic bone lesions

98
Q

What dictates clinical presentation of lymphoid neoplasms?

A

Anatomic distribution of disease

99
Q

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

A

2/3 nodal: No pain lymphadenopathy

1/2 extranodal: Tissue masses

100
Q

What is required for diagnosis of lymphoid neoplasm?

A

Histologic examination of lymph nodes or tissues

101
Q

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

A

Clonality

Derived from single transformed cell = Monoclonal

Analysis of antigen receptor proteins

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

102
Q

80-85% of lymphoid neoplasms are of what origin?

What are the rest?

A

B-Cell origin

Rest are T cell, a few rare NK

103
Q

What is a follicular lymphoma?

A

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

104
Q

What is diffuse lymphoma?

A

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

105
Q

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

A

Exclude lymphoma

106
Q

Tumors of immune system will have what effect?

How does this present (2)

A

Disruption of normal immune regulatory mechanisms

  1. Immunodeficiency
  2. Autoimmunity
107
Q

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

A

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

Staging not very important

108
Q

How do you generally treat NHL’s?

A

Systemic therapies

109
Q

Hodgkin lymphomas are described how in terms of location?

Is staging important?

A

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

110
Q

What might cure hodgkin lymphoma?

A

Excision and local radiation

111
Q

What is the progression of B cells with locations

A

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

112
Q

What is progression of T cells with locations

A

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
Q

Myeloid CD’s? (3)

A

CD13-CD15

114
Q

In acute leukemias what happens in differentiation?

A

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

115
Q

What causes accumulation of blasts in marrow? 2

What is this associated with?

A
  1. Clonal expansion
  2. Failure of maturation

Suppression of normal hematopoiesis

116
Q

How does acute leukemia generally present?

Symptoms? (4)

A

Abrupt stormy onset

  1. Anemia –> Fatigue
  2. Leukopenia –> Fever and infection
  3. Thrombocytopenia –> Bruising/bleeding
  4. Bone pain and tenderness
117
Q

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

A
  1. Lymphaenopathy
  2. Hepatosplenomegaly
  3. Testicular involvement
  4. Compression of BV’s and airways
  5. CNS manifestations
118
Q

CNS manifestations are seen mainly in what demographic?

A

Children

119
Q

Lab findings of acute leukemias? 4

A
  1. Anemia
  2. Thrombocytopenia
  3. WBC’s greater than 100,000
  4. Blasts in peripheral blood and bone marrow
120
Q

What is the diagnostic feature of AL?

A

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

121
Q

Aleukemic leukemia presents how?

A

Pancytopenia with rare blasts in peripheral blood

122
Q

Why is it important to differentiate ALL from AML?

A

Treatment

123
Q

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

A

Lymphoblasts

Children and young adults

124
Q

Pre-B lymphoblastc tumors present in what form?

With what involvement?

A

Leukemias

Bone marrow and peripheral blood

125
Q

Pre-T lymphoblastic tumors commonly present how?

Progress to what?

A

Mediastinal masses involving thymus

Leukemia

126
Q

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

A

Acute Lymphoblastic Leukemia

127
Q

ALL is the most common cancer of what?

A

Children

128
Q

Pre-B ALL peaks at what age?

A

3

129
Q

Pre-T cell tumors are more common with who?

A

Adolescent males

130
Q

Pre-B cell tumors have what antigens? 2

A

CD19 and CD10

131
Q

Pre-T cell tumors have what antigens? 4

Later which ones (3)

A

CD1, CD2, CD5, CD7

CD3, CD4, CD8

132
Q

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

A

TdT

133
Q

90% of precursor T and B tumors have what genetic change?

More common with which one?

A

Nonrandom karyotypic abnormalities

B cell (hyperdiploidy)

134
Q

Which is more severe, nonrandom karyotypic abnormalities or other?

A

Other

135
Q

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

A

Children 2-10 with pre-B cell tumor

136
Q

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

A

95%

137
Q

11 Peripheral B-cell neoplasms

A
  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
Q

Small lymphocytic lymphoma and chronic lymphocytic leukemia are related how?

A

CLL is circulating SLL

139
Q

Special title of Small lymphocytic lymphoma/chronic lymphocytic leukemia?

A

Most common leukemia in Western world

140
Q

Small lymphocytic lymphoma/chronic lymphocytic leukemia Age?
Sex?

A

60

Males more common

141
Q

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

A

Absolute lymphocytosis with smudge cells

142
Q

Immunophenotype of Small lymphocytic lymphoma/chronic lymphocytic leukemia?

A
  1. Pan-B markers (CD19 and 20)
  2. Monoclonal kappa or delta light chains
  3. CD23
  4. CD5
143
Q

CD23 appears in Small lymphocytic lymphoma or chronic lymphocytic leukemia

A

SLL

144
Q

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

A

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

145
Q

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

A
  1. Asymptomatic at diagnosis

2. Autoimmune hemolytic anemia/thrombocytopenia

146
Q

Survival length of Small lymphocytic lymphoma/chronic lymphocytic leukemia?

A

4-6 years

147
Q

What does CLL/SLL transform into over time?

A

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

148
Q

Key histopathic finding in SLL/CLL?

A
  1. Smudge cells
149
Q

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

A

45%

150
Q

FL arises from what?

A

Germinal center B cell

151
Q

Markers for FL? (5)

A
  1. Pan B markers (CD19,CD20)
  2. CD10
  3. BCL6
  4. BCL2
  5. No CD5
152
Q

Folicular lymphoma age?

Sex?

A

Middle aged

Equal

153
Q

How does FL present? (4)

A
  1. Painless
  2. Generalized lymphadenopathy
  3. Bone marrow involved
  4. t(14,18) –> Overexpress BCL2
154
Q

Prognosis of FL?

Some progress to?

A

7-9 years

Diffuse large B-cell lymphoma

155
Q

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

A

Diffuse Large B-cell lymphoma

156
Q

3 characteristics of Diffuse Large B-cell lymphoma?

A
  1. Diffuse growth pattern
  2. Aggressive
  3. Heterogeneous in terms of pathogenesis
157
Q

Markers for Diffuse Large B-cell lymphoma? 2

A
  1. Pan B (CD19, CD20)

2. Variable after that due to different subtypes

158
Q

Diffuse Large B-cell lymphoma age?

A

60 median age

159
Q

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

A
  1. Rapidly enlarging mass at single node or extranodal site
  2. Spleen, liver involved
  3. Bone marrow not involved initially
  4. Aggressive
160
Q

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

A

Rapidly fatal

Complete remission in 60-80%

50% can be cured

161
Q

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

A

Fastest growing human neoplasm

162
Q

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

A

Starry sky

163
Q

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

A

Extranodal

164
Q

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

A

30% of childhood NHL’s

Visceral (GI and sinus)

Jaw involvement

165
Q

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

A
  1. CD19
  2. CD20
  3. CD10
  4. BCL6
  5. Translocations of c-MYC gene on Ch 8
166
Q

Three translocations possible of c-MYC gene?

What is the other gene involved

A
  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
Q

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

A

EBV

168
Q

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

A

Aggressive chemotherapy

169
Q

Mantle Cell lymphoma is seen in what age group?

A

Middle aged males

170
Q

Mantle Cell lymphomas are composed of what?

A

B cells resembling those in mantle zone surrounding normal lymphoid follicles

171
Q

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

A

Generalized adenopathy

Extranodal sites: BM, spleen, liver, GI

172
Q

Markers for Mantle Cell lymphoma?

A
  1. CD19/CD20
  2. CD5
  3. CD23 negative
  4. Overexpression of cyclin D1
173
Q

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

A

CD23 positive in SLL/CLL

CD23 negative in mantle cell lymphoma

174
Q

Most Mantle Cell lymphoma have what translocation?

Effect?

A

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

Increases cyclin D1 –> Promote G1 to S progression

175
Q

Most Mantle Cell lymphoma present how?

A

Painless lymphadenopathy

176
Q

Mantle cell lymphoma has what prognosis?

A

Aggressive and incurable with survival of 3-4 yeras

177
Q

Marginal zone lymphoma arises where? 3

A

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

178
Q

What bacteria commonly accompanies marginal zone lymphoma?

A

H. pylori

179
Q

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

A
  1. Sjogren
  2. Hashimoto thyroiditis
  3. H. pylori
180
Q

Extra-nodal marginal zone lymphoma occurs where?

A

MALT

181
Q

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

A

Inciting agent removed

182
Q

Extra-nodal marginal zone lymphoma may transition between what?

A

Reactive lymphoid hyperplasia and B-cell lymphoma

183
Q

How to differentiate between acute leukemia and chronic?

A 
Acute = 20% blasts
Chronic = <20% blasts
184
Q

Hair cell leukemia is most commonly seen in who?

A

Middle-aged caucasian males

185
Q

Histo sign?

A

Peripheral blood leukemic cells have fine hair-like cytoplasmic projections

186
Q

Hairy cell leukemia markers? (5)

A
  1. CD19
  2. CD20
  3. CD11c
  4. CD25
  5. CD103
187
Q

Hairy cell leukemia triad of symptoms?

A
  1. Splenomegaly
  2. Pancytopenia
  3. Dry tap on bone marrow aspiration
188
Q

What treatment is Hairy cell leukemia really sensitive to?

A

Chemotherapy

189
Q

Prognosis if treated?

A

Excellent with possible curable relapses

190
Q

Hairy cell leukemia stains best with what stain?

A

TRAP (tartrate resistant acid phosphatase)

191
Q

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

A

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
Q

Neoplastic plasma cells have what main finding?

A

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

193
Q

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

What is this called?

A

Elderly patients

Monoclonal gammopathy of undetermined significance (MGUS)

194
Q

3 forms of plasma cell neoplasms to know?

A
  1. Plasma cell myeloma/Multiple myeloma
  2. Solitary plasmacytoma
  3. MGUS
195
Q

Plasma cell myeloma has what special title?

A

Most common plasma cell dyscrasias

196
Q

Prognosis of plasma cell myeloma?

A

204 years

197
Q

What proliferates in plasma cell myeloma?

A

Neoplastic plasma cells in BM

198
Q

What physical finding is associated with plasma cell myeloma?

How does this present (2)?

A

Multifocal lytic lesions of axial skeleton

Pathologic fractures
Hypercalcemia

199
Q

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?

A

Half IgG and a quarter IgA

20% is light chains

Bence-Jones proteins

60-70%

200
Q

Age of plasma cell myeloma?

Sex?

A

65-70

M>F

201
Q

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

A
  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
Q

4 main findings of plasma cell myeloma?

A
  1. Bone resoprtion
  2. Renal insufficiency (BJ proteinuria is damaging)
  3. Amyloidosis (Due to BJ)
  4. Anemia/thrombocytopenia
203
Q

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

A

CRAB

hyperCalcemia
Renal insufficiency
Anemia
Bone lesions

204
Q

plasma cell myeloma peripheral blood finding? 2

A

Rouleaux formation

Mott Cells with Russell bodies

205
Q

Prognosis of plasma cell myeloma?

If treated?

A

Poor

Remission but survival doesn’t change

206
Q

Localized plasmacytoma has what definition?

A

presence of a single lesion in skeleton or soft tissues

207
Q

Most Localized plasmacytoma patients develop what?

A

Disseminated disease (myeloma)

208
Q

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

A

Extraosseous plasmacytomas

209
Q

MGUS is seen with what age group?

A

elderly

210
Q

Diagnosis of MGUS should be made after what?

A

Excluding all other specific monoclonal gammopathies

211
Q

How do you care for MGUS patient?

A

Keep assessing M component and Bence Jones levels

212
Q

Lymphoplasmacytic lymphoma is defined how?

A

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

213
Q

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

A

IgM

Hyperviscosity syndrome called Waldenstrom Macroglobulinemia (WM)

214
Q

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

A
  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
Q

Prognosis for Lymphoplasmacytic lymphoma?

A

Incurable and progressive

216
Q

Hyperviscosity syndrome is due to what?

A

Increased monoclonal IgM

217
Q

4 manifestations of hyperviscosity syndrome?

A
  1. Visual impairment
  2. Neurologic problems
  3. Bleeding
  4. Cryoglobulinemia
218
Q

Heavy-chain disease definition?

A

Only heavy chains produced of IgG, IgA, or IgM

219
Q

How does IgG Heavy-chain disease present?

A

Diffuse lymphadenopathy with hepatosplenomegaly

220
Q

How does IgA heavy-chain disease present?

A

Goes for the MALT

221
Q

Primary or immunocyte-assicated amyloidosis is defined how?

A

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

222
Q

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

A

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

223
Q

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

A

Dysfunction

Pathology (36 decks)

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