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Pathology > WBC Hematopathology Part 2 > Flashcards

WBC Hematopathology Part 2 Flashcards

1
Q

Peripheral T-cell tumors are what % of NHL’s in US and Asia?

A

5-10%

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2
Q

Peripheral T cell and NK cell tumors are most common where?

A

In Far East

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3
Q

How do nodes appear in peripheral T cell neoplasms?

A

Diffuse effacement of nodal architecture

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4
Q

Markers for T-cell neoplasms? 2

A
  1. Pan-T Markers (CD2/3/5)

2. CD4 or CD8 possibly

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5
Q

What defines a T-cell malignancy?

A

Clonal T-cell receptor gene rearrangements

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6
Q

How do T cell neoplasms generally present? (5)

A 
Disseminated adenopathy
Eosinophilia
pruritis
Fever
Weight loss
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7
Q

How do T cell neoplasms respond to therapy?

A

Respond poorly due to being so aggressive

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8
Q

5 Peripheral T cell neoplasms?

A
  1. Anaplastic large cell lymphoma
  2. Adult T-cell leukemia/Lymphoma
  3. Mycosis Fungoides and Sezary Syndrome
  4. Large Granular Lymphocytic Leukemia
  5. Extranodal NK/T-Cell lymphoma
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9
Q

Anaplastic Large Cell lymphoma has what rearrangement?

This produces what?

A

Rearrangement of ALK gene on chromosome 2p23

Active tyrosine kinases

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10
Q

What is the hallmark cell in Anaplastic Large Cell lymphoma

A

Large, anaplastic cells

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11
Q

What does Anaplastic Large Cell lymphoma’s lymph node pattern mimic? (2)

A
  1. Metastatic carcinoma

2. Hodgkin lymphoma variants

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12
Q

Anaplastic Large Cell lymphoma with ALK rearrangement are seen in what population?
What is prognosis?

A

Kids

Good prognosis

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13
Q

Anaplastic Large Cell lymphoma without ALK rearrangement is seen in what population?
What is prognosis?

A

Older adults

Worse prognosis

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14
Q

Adult T-cell leukemia/lymphoma is seen in adults infected with what?

A

Retrovirus HTLV-1

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15
Q

Where is HTLV-1 seen in the world? 3

A

Japan
West Africa
Caribbean

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16
Q

Clinical findings in Adult T-cell leukemia/lymphoma? 4

A
  1. Skin lesions
  2. Hypercalcemia
  3. Hepatosplenomegaly
  4. Elevated WBC count with multilobulated CD4’s
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17
Q

Prognosis of Adult T-cell leukemia/lymphoma?

A

Less than a year, extremely aggressive

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18
Q

Mycosis Fungoides and Sezary syndrome are two manifestations of what?

What are the hallmark cells involved?

A

Cutaneous T-cell lymphoma

Peripheral CD4 T cells wth a crebriform appearance

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19
Q

How does Mycosis FUnoides present as?

Where do the T cells go?

A

Inflammatory pre-mycotic phase and progresses through a plaque phase to a tumor phase.

infiltrate skin

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20
Q

Sezary syndrome has what main presentation?

What is prognosis?

A

Generalized exfoliative erythroderma

Incurable but long survival

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21
Q

Large granular lymphocytic leukemia is seen how?
What population mainly has it?
What organs does it avoid?

A

With granules which is strange for lymphocytes

Adults

Lymph nodes and liver

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22
Q

Hallmark of Large granular lymphocytic leukemia?

A

Lymphocytes in peripheral blood and BM with blue cytoplasm and scattered azurophilic granules

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23
Q

Two symptoms of Large granular lymphocytic leukemia?

A

Neutropenia

Anemia

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24
Q

Two variants of Large granular lymphocytic leukemia
Which is more severe?
Which has splenomegaly and lymphocytosis?

A

T cell: Mild/Lymphocytosis + Splenomegaly

NK Cell: Aggressive

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25
Q

What are the five subtypes of hodgkin lymphomas?

A 
– Nodular sclerosis
– Mixed cellularity
– Lymphocyte-rich
– Lymphocyte depletion (rare)
– Lymphocyte predominance (LP)
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26
Q

Which of the hodgkin subtypes are classical hodgkin lymphoma?

A

First four (not LP) since the R-S cells have similar immunophenotype

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27
Q

What makes LP different from other hodgkin subtypes?

A

Malignant cells express a different B-cell phenotype (CD20, CD15 and CD30 -)

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28
Q

Hodgkin lymphoma neoplasm is located where in body?
What virus causes some of these?
Prognosis
Best prognosis indicator?

A

Germinal center or post-germinal center B lymphocytes

EBV

Very good

Clinical stage is great indicator

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29
Q

Side effects of chemo/radiotherapy against Hodgkin lymphoma?

A
  1. Myelodysplasia
  2. Acute leukemia
  3. Lung cancer
  4. Melanoma
  5. breast cancer
  6. Gastric cancer
  7. NHL
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30
Q

How does one avoid the cancers caused by chemo and radiotherapy?

A

Use new drug combinations and less of the drugs

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31
Q

What is Stage I Hodgkin?

A

Involvement of single lymph node region or single extra-nodal site

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32
Q

Stage II hodgkin?

A

Two or more lymph node regions (or extranodal sites) on same side of diagphragm

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33
Q

Stage III Hodgkin’s

A

Involvement of lymph node regions on both sides of the diaphragm.
May include spleen or localized extranodal disease

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34
Q

Stage IV Hodgkin’s

A

Diffuse extralymphatic disease in liver BM, lung, skin

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35
Q

Most common Hodgkin subtype?

A

Nodular sclerosis

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36
Q

Nodular sclersosis is what % of HL?

Difference in sexes?

A

65-70%

Sexes are equal

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37
Q

What are the two histo signs of nodular sclerosis?

A

Lacunar cells

Bands of collagen fibrosis

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38
Q

R-S cells in nodular sclerosis have what markers?

A
  1. Positive for CD15 and CD30

2. Negative for CD45 and other B and T markers

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39
Q

Age of nodular sclerosis patients?

A
  1. young adults
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40
Q

Mixed cellularity hodgkin lymphoma is the most common form of HL in what group of patients?
Sex difference?

A

Males older than 50

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41
Q

Immunophenotype of mixed cellularity hodgkin?

A

Same as Nodular

  1. Positive for CD15 and CD30
  2. Negative for CD45 and other B and T markers
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42
Q

Patients with mixed cellularity hodgkin are more likely to present how?
Prognosis

A

Advanced disseminated disease

Prognosis remains really good

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43
Q

RS cells in mixed cellularity hodgkin contain what at 70%?

A

EBV

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44
Q

Lymphocyte-Rich hodgkin lymphoma has what cell predominate?

A

Reactive lymphocytes

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45
Q

How to distinguish between Lymphocyte Rich and Lymphocyte Predominance?

A

Lymphocyte Rich has frequent mononuclear R-S cells of characteristic HL immunophenotype

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46
Q

Lymphocyte-Rich hodgkin lymphoma is associated with what virus at 40%

A

EBV

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47
Q

Prognosis of Lymphocyte-Rich HL?

A

Very good to excellent prognosis

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48
Q

What is the least common form of hodgkin lymphoma?

A

Lymphocyte Depletion

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49
Q

What cells are low in Lymphocyte-Depletion?
Which are high?
What is immunotype for these cells?

A

Few lymphocytes

Abudant R-S cell variants

HL immunotype

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50
Q

Age and patient group of Lymphocyte depletion HL?

A

Elderly

HIV-positive

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51
Q

EBV positive in what % of Lymphocyte depletion HL?

A

90%

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52
Q

What is prognosis in Lymphocyte depletion HL?

A

Less favorable

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53
Q

Lymphocyte predominance HL is what patient group?

A

Males younger than 35

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54
Q 
Lymphocyte predominance HL is characterized by what cells? 
Other cells (2)
A

Lymphohistiocytic variant cells/Popcorn cells

Reactive lymphocytes and histiocytes

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55
Q

Are RS cells present in Lymphocyte predominance HL?

A

Yes, but very rare

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56
Q

L and H cells have what markers? (4)

What does this mean?

A
  1. Positive for CD20 and BCL6 (b cell lymphoma characteristic)
  2. Negative for CD15 and CD30 (RS cells)

This is really a B-cell lymphoma

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57
Q

Is LP associated with EBV?

A

No

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58
Q

three major categories of myeloid neoplasms?

A

Acute myeloid leukemias
Myelodysplastic Syndromes
Myeloproliferative disorders

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59
Q

What is AML?

A

blockage in differentiation of early myeloid cells which causes blasts to accumulate in BM and then circulate in peripheral blood

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60
Q

What is Myelodysplastic syndrome?

A

Terminal differentiation of early myeloid cells occurs but is disordered and ineffective

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61
Q

How is MDS marked?

A

Presence of dysplastic marrow precursors and peripheral blood cytopenias

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62
Q

What is a myeloproliferative disorder?

A

Neoplastic clone retains capacity to undergo terminal differentiation with dysregulated goal.

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63
Q

Myeloproliferative disorders result in what peripheral blood?

A

Increased RBC’s, WBC’s and platelets

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64
Q

Acute myeloid leukemia is due to what?

A

Acquired oncogenic mutation blocking normal cell differentiation –> Accumulation of myeloblasts in marrow

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65
Q

AML leads to what?

Which appears how (3)

A

Marrow failure

Anemia, thrombocytopenia, neutropenia

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66
Q

Acute Myeloid Leukemia occurs when?

What age most?

A

All ages

Peaks after 60

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67
Q

Acute myeloid leukemia signs and symptoms resemble what?

Related to what?

A

Acute lymphoblastic leukemia

Splenomegaly and lymphadenopathy

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68
Q

How might AML rarely prsent?

Infiltration of what suggests monocytic types?

A

Discrete tissue mass like a sarcoma or myeloid tumor

Infiltration of skin and gingiva

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69
Q

Diagnosis of AML
What is done first?
What is the key diagnostic finding?

A

Karyotypic findings

Greater than 20% myeloblasts in BM

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70
Q

Cytogenetic abberations in AML? (2)

A
  1. Disrupt genes encoding TF’s for normal differentiation

2. Activate mutated tyrosine kinases –> Constant proliferation signals

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71
Q

The M3 AML affects what population most?
What does it progresss to?
Chromosomal abnormality?
Genes involved?

A

Younger people

DIC

t(15;17)(q22:q21)

PMU/RARA

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72
Q

What stains do you do for AML? 3

A
  1. Myeloperoxidase (myelocytic)
  2. Specific esterase (myelocytic)
  3. Nonspecific esterase (monocytic)
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73
Q

Markers of AML? 4

A

CD13
CD14
CD15
CD33

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74
Q

What are the WHO classifications of AML? 4

A
  1. AML with particular genetic abnormalities
  2. AML with MDS-like features
  3. AML, therapy related
  4. AML not otherwise specified
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75
Q

AML has what particular genetic abnormalities?

A

t(8;21)
inv(16)
t(15;17)

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76
Q

Using FAB classification, which AML’s are precursor of neutrophils?

A

M0 through M3

77
Q

Using FAB classification, which AML has both myeloid and monocytic precursors?

A

M4 and M4 eos

78
Q

Under FAB classification, which AML’s are precursors of different things?

A

M5: Monocytic
M6: Erythroid
M7: Acute megakaryocytic

79
Q

AML prognosis?

A

Chemo can get you into complete remission but 5 year survival is low.

80
Q

High risk forms of AML and relapsing AML are treated with what?

A

BM transplant

81
Q

Main histo findings of AML? 2

A

Myeloblasts

Auer rods

82
Q

Acute promyelocytic leukemia (APL/AML-M3) exhibits what genetic mutation?

83
Q

What protein forms in APL/M3?

A

Fusion of retinoic acid receptor alpha (RARA) and PML gene to make a product that blocks myeloid maturation at promyelocyte stage

84
Q

How to overcome RARA-PML block in APL?

A

Vitamin A derivatives and chemo

85
Q

APL is associated with what two things

A

DIC

Bleeding diatheses

86
Q

What is the key cell finding in AML?

A

Auer rods in a Faggot cell

87
Q

What is cause of most myelodysplastic syndromes?

A

Idiopathic/primary

88
Q

What is identified in 70% of myelodysplastic syndromes?

A

Clonal chromosome abnormality in chromosomes 5, 7, 8, 20

89
Q

What is the mean age of onset of myelodysplastic syndromes

90
Q

Symptoms of myelodysplastic syndromes appear similar to what?

A

Pancytopenia: Infeciton, anemia, hemorrhage

91
Q

What are the subtypes of myelodysplastic syndromes classified based on? 3

A
  1. Number of blasts
  2. cell lines
  3. Presence of ring sideroblasts
92
Q

What must be excluded before a diagnosis of myelodysplastic syndromes can be made?

A

All non-clonal causes for dysplasia must be disproved such as drugs, growth factor therapy, virus, immune disorder, vitamin deficient, too much zinc

93
Q

How do myelodysplastic syndromes respond to therapy?

A

Poorly, but some may live more than 5 years.

94
Q

What do younger patients with myelodysplastic syndromes get for treatment?

A

BM transplant

95
Q

what is main cause of death in myelodysplastic syndromes?

A

Marrow failure: Thrombocytopenia and neutropenia

96
Q

Myeloproliferative disorders have what mutation?

2 examples

A

Mutated tyrosine kinases causing GF-independent proliferation

  1. BCR-ABL
  2. JAK2
97
Q

What are four features of myeloproliferative disorders?

A
  1. Increased marrow proliferation (RBC, WBC, PLT)
  2. Neopalstic stem cells seed secondary hematopoietic organs (hepatosplenomegaly)
  3. Variable transofrmation to a spent phase with marrow fibrosis and peripheral cytopenis
  4. Variable transformation to acute leukemias
98
Q

Four main forms of myeloproliferative disorders?

A
  1. Chronic Myeloid leukemia
  2. Polycythemia Vera
  3. Essential thrombocytosis
  4. Primary myelofibrosis
99
Q

Chronic myeloid leukemia affects what age mainly?

A

40-60 adults

100
Q

What is main physical symptom of CML?

A

Splenomegaly

101
Q

WBC count in CML?

Other cells present (2)

A

Over a 100,000

Eosinophils and basophils

102
Q

CML has what % blasts?
What does bone marrow show?
What is the Leukocyte Alkaline Phosphatase (LAP) score?
Prognosis?

A

less than 10%

Panhyperplasia

Decreased leukocyte alkaline phosphatase score

Untreated survival of 3 years

103
Q

50% of CML patients enter what phase?

what does this entail (4)

A

Accelerated phase

  1. increasing anemia
  2. Thrombocytopenia
  3. More mutations
  4. Blast crisis eventually
104
Q

50% of CML patients enter wha tother phase?

There is a 30:70 ratio of what?

A

Blast crisis

30% are B cell ALL and 70% are AML

105
Q

Treatment for CML?

A

Gleevec inhibits BCR-ABL fusion product of the mutated tyrosine kinase gene t(9;22)

106
Q

What % get remission with Gleevec in CML?

107
Q

What is treatment of choice in younger CML patients?

A

BM transplant

108
Q

Define polycythemia

A

Increase in concentration of erythrocytes in the blood

109
Q

2 forms of polycythemia

A
  1. Relative polycythemia

2. Absolute polycythemia

110
Q

What changes in relative polycythemia?

A

Increase in hematocrit due to a decrease in plasma volume.

NO CHANGE IN TOTAL RBC mass

111
Q

Causes of relative polycythemia (2)

A
  1. Diminished plasma volume due to shock

2. Stress polycythemia

112
Q

Absolute polycythemia is due to what?

A

Increase in total red cell mass

113
Q

Two types of absolute polycythemia

A

Primary

Secondary

114
Q

Primary polycythemia is characterized by what

115
Q

Secondary polycythemia is characterized by what?

A

High or normal EPO

116
Q

Appropriate EPO production occurs when?

A

Hypoxia and arterial O2 desaturation (high altitude, smoker, methemoglobinemia,)

117
Q

Inappropriate EPO production occurs when (2)

A
  1. Neoplasms

2. Renal pathology

118
Q

Polycythemia Vera has signs and symptoms due to what?

A

EPO-independent increase in RBC mass

119
Q

Polycythemia has strong association with what mutation?

120
Q

Increased blood volume and viscosity are due to what in polycythemia vera?

A

Increase in RBC cell mass

121
Q 
Polycythemia
HCT:
WBC:
PLT:
Basophils:
A 
HCT = Over 60%
WBC: = High
PLT = High
Basophils = High
122
Q

6 main signs of polycythemia vera?

A
  1. Mild hepatosplenomegaly
  2. Panhyperplasia and Decreased Iron
  3. Plethora (ruddy red complexion)
  4. Cyanosis
  5. Intense pruritis
  6. Major bleeding and thrombotic episodes
123
Q

Polycythemia vera has an age of what?

A

60 years old

124
Q

Death of polycythemia?

A

Bleeding or thrombosis

125
Q

RBC mass in polycythemia vera can be kept maintained by what?

A

Phlebotomies

126
Q

After 10 years what happens to Polycythemia vera patients?

A

Transition into spent phase with extramedullary hematopoiesis and splenomegaly

127
Q 
Major criteria for polycythemia vera? (2)
Minor criteria (2)
A
  1. Elevated Hb
  2. Presence of JAK2 mutation
  3. BM biopsy showing hypercellularity with trilineage growth
  4. Serum EPO decreased
  5. Endogenous erythroid colony formation in vitro
128
Q

Essential thrombocytosis is an increase in proliferation confined to what elements?

A

Megakaryocytic elements (platelets)

129
Q

Diagnosis of essential thrombocytosis is described how?

A

Diagnosis of exclusion since all myelo-proliferative disorders can be associated with thrombocytosis

130
Q

PLT count in essential thrombocytosis?

A

Greater than 600,000

131
Q

Bone marrow of essential thrombocytosis appears how?

A

Hypercellular with increased abnormal megakaryocytes

132
Q

Platelets appear how in essential thrombocytosis?

A

Enlarged and functionally abnormal

133
Q

50% of essential thrombocytosis is associated with what mutation?

134
Q

Course of essential thrombocytosis?

A

Long asymptomatic periods with occaisional thrombotic or hemorrhagic crises

135
Q

Two progressions of essential thrombocytosis?

A
  1. Spent phase of myelofibrosis

2. Transform to AML

136
Q

Survival of essential thrombocytosis?

A

12-15 years

137
Q

Primary myelofibrosis has its fibrosis occur where early?

138
Q

Marrow fibrosis has what effect on body?

A

Suppress hematopoiesis –> pancytopenias –> Spleen and liver respond –> Extreme hepatosplenomegaly

139
Q

What cell type increases and appears strange in primary myelofibrosis?
What does this stimulate?

A

Megakaryocytes

Stimualtes benign fibroblasts to lay down collagen and form marrow fibrosis

140
Q

What mutation is in 50-60% of primary myelofibrosis?

141
Q

Peripheral blood in primary myelofibrosis demonstrates what? 2

A

Leukoerythroblastosis and Teardrop cells

142
Q

Primary myelofibrosis appears in what age?

4 symptoms to present with?

A

older than 60

  1. Anemia
  2. Splenomegaly
  3. Fibrosis
  4. Gout
143
Q

Cell changes in Primary myelofibrosis? 4

A
  1. Increase basophils
  2. Abnormal platelet morphology
  3. Increased abnormal megakaryocytes
  4. Marked marrow fibrosis
144
Q

Survival length in Primary myelofibrosis?

145
Q

Langerhans Cell histiocytosis is what?

A

Clonal proliferation of dendritic antigen-presenting cells normally found in many organs

146
Q

What do cells contain in Langerhans Cell histiocytosis?

Markers (2)

A

Birbeck granules

S-100 and CD1a antigens

147
Q

Old name for Langerhans Cell histiocytosis?

A

Histiocytosis X

148
Q

Three entities of Langerhans Cell histiocytosis?

A

Multifocal multisystem LCH
Unifcoal and multifocal unisystem LCH
Pulmonary LCH

149
Q

Multifocal multisystem LCH occurs when in life?
Main cell and its appearance?
Prognosis?

A

Before age 2

Monocytic cell with convoluted/folded nucleus

Agressive and fatal if untreated. Chemo can keep you alive

150
Q

Unifocal and multifocal unisystem LCH involves what action?

A

Erosive accumulations of Langerhans cells in medullary cavities of bones

151
Q

What bones are usually involved in Unifocal and multifocal unisystem LCH? 3

A

Skull
Ribs
Femur

152
Q

Unifocal and multifocal unisystem LCH age?
Primary complication

Prognosis?

A

Young children

Diabetes insipidus

Spontaneous regression or chemo

153
Q

Unifocal and multifocal unisystem LCH has unifocal lesions in what ages?

A

Older children or adults

154
Q

Pulmonary LCH involves what patient group mainly?

A

Adult smokers

155
Q

Pulmonary LCH can be defined as what better?

A

Reactive hyperplasia

156
Q

Four splenic functions that impact disease states?

A

Phagocytosis of blood cells and particulate matter
Antibody production
Hematopoiesis
Sequestration of formed blood elements

157
Q

Splenic insufficiency/Asplenia is due to what? 2

A

Splenectomy

Autoinfarction

158
Q

Splenectomy is due to what? (2)

A
  1. Traumatic injury that ruptures spleen

2. Hemolytic anemias

159
Q

Autoinfarction can be due to what?

A

Sickle cell disease

160
Q

Splenic insufficiency/Asplenia results in what?

What is this due to?

Doctor’s reaction?

A

Increased susceptibility to sepsis by encapsulated bacteria

Loss of filtering and Ab production

Vaccinate these people appropriately

161
Q

What is hypersplenism characterized by?

A
  1. Splenomegaly
  2. Anemia, leukopenia, thrombocytopenia
  3. Hyperplasia of marrow precursors of deficient type
162
Q

Correction of hypersplenism blood cytopenias is done by what?

A

Splenectomy

163
Q

Massive splenomegaly is a weight of what?

Main cause?

A

Weight > 1000 grams

Myeloproliferative disorders

164
Q

Moderate splenomegaly is what weight?

A

500-1000 grams

165
Q

Mild splenomegaly is what weight?

A

< 500 grams

166
Q

Nonspecific acute splenitis is what?

A

Nonspecific reaction to any blood-borne infection

167
Q

What happens within the spleen in Nonspecific acute splenitis? 2

A

Congestion of red pulp with neutrophils

Plasma cells throughout spleen

168
Q

Congestive splenomegaly definition?

What type of disorders cause this?

3 examples?

A

Enlargement due to chronic venous congestion

Disorders leading to splenic or portal vein hypertension

  1. Central venous congestion
  2. Cirrhosis of liver
  3. Portal vein thrombosis
169
Q

What is Congestive splenomegaly usually associated with?

A

Hypersplenism

170
Q

Primary splenic neoplasms are how common?
Exception?
Most common type?

A

Very rare

Tumors in lymphohematopoietic system

Benign tumors

171
Q

What is more common in congenital anomalies: hypoplasia or complete absence?

A

Hypoplasia

172
Q

What is a common finding in congenital anomalies of spleen?

A

Accessory spleen (spleniculi)

173
Q

When are accessory spleens really important? 2

A

Setting of splenectomy in treatment of

  1. Hemolytic anemias
  2. ITP
174
Q

Splenic rupture is precipitated by what?

A
  1. Crushing injury

2. Severe blow

175
Q

When does spontaneous splenic rupture occur?

A

Minor physical insult to a spleen that is fragile by underlying condition like mono, malaria, lymph cancers

176
Q

Are chronically enlarged spleens likely to rupture?

A

No. Fibrosis makes them tough

177
Q

Thymus plays a big role in what? (2)

A
  1. T-cell differentiation

2. CMI

178
Q

3 frequent disorders of thymus?

A
  1. Developmental disorder
  2. Thymic hyperplasia
  3. Thymoma
179
Q

Di-Geroge syndrome definition:

Mutation:

A

Thymic hypoplasia or apalasia with parathyroid developmental failures
22q11.2 deletion

180
Q

Symptoms of DiGeorge Syndrome (CATCH-22) 5

A 
Cardiac abnormality
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia/Hypoparathyroidism
181
Q

Why are thymic cysts important?

A

Thymic neoplasms are often associated with cysts

182
Q

Does a normal thymus have lympoid follicles?

183
Q

B-cell germinal centers/follicles in medulla of thymus suggest what?

A

Thymic Follicular Hyperplasia

184
Q

What disease has Thymic Follicular Hyperplasia the most?

What is the treatment?

A

Myasthenia gravis

Thymectomy

185
Q

Thymoma is what?

A

Thymic tumor in which neoplastic element is epithelial

186
Q

3 types of thymoma?
Most common
Most malignant

A
  1. Noninvasive (most common and benign)
  2. Invasive
  3. Thymic carcinoma (most malignant/severe)
187
Q

Thymic carcinomas are usually what type of carcinoma?

A

Squamous cell

188
Q

Thymomas are seen with what disease especially?

A

Myasthenia gravis

Pathology (36 decks)

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