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Pathoma > Chapter 6: White blood cell disorders > Flashcards

Chapter 6: White blood cell disorders Flashcards

1
Q

neoplastic proliferation of blasts; defined as the accumulation of greater than 20% of blasts in bone marrow

A

acute leukemia

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2
Q

blasts “crowd out” normal hematopoiesis resulting in anemia (fatigue), thrombocytopenia (bleeding), and neutropenia (infection).

A

acute leukemia

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3
Q

high or low WBC in acute leukemia?

A

high (blasts are large, immature cells, with punched out nucleoli

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4
Q

ALL

A

Acute lymphoblastic Leukemia (a sub-division of acute leukemia)

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5
Q

AML

A

acute myelogenous leukemia (a sub-division of acute leukemia)

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6
Q

neoplastic accumulation of LYMPHOblasts (>20%) in the bone marrow

A

ALL

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7
Q

Upon diagnostic testing, TdT positive nuclear staining is detected in an immature cell

A

ALL

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8
Q

TdT

A

a DNA polymerase found only in immature lymphobasts

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9
Q

very common in children. especially those with down syndrome (OLDER than 5)

A

ALL

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10
Q

Two types of ALL

A

B-ALL and T-ALL

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11
Q

Most common type of ALL

A

B-ALL

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12
Q

TdT+ lymphoblasts that express CD10, CD19, and CD20

A

B-ALL

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13
Q

requires prophalaxis to scrotum and CSF

A

chemotherapy for B-Acute lymphocytic leukemia

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14
Q

cytogenetic abnormalities associated with B-ALL

A

t(12;21) and t(9;22)

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15
Q

t(12;21)

A
  • good prognosis in B-ALL; usually seen in children

- ETV6 and RUNX1 genes

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16
Q

t(9;22)

A

Philadelphia ALL…you are fucked, what songs do you want at your funeral? seen mostly in adults. BCR-ABL genes

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17
Q

characterized by lymphoblasts (TdT+) that express markers ranging from CD2-CD8. CD10 is NOT seen.

A

T-ALL

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18
Q

Teenagers and thymic mass (mediastinal)

A

T-ALL—>Acute lymphoblastic lymphoma

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19
Q

Neoplastic accumulation of immature myeloid cell (>20%) bone marrow

A

AML (acute myeloid leukemia)

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20
Q

Positive MPO cytoplasmic staining

A

AML

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21
Q

Auer rods (MPO crystal aggregates)

A

AML

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22
Q

average age of AML?

A

50-60

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23
Q

APL

A

acute promyelocytic leukemia (which is an acute myeloid leukemia which is of course acute leukemia)

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24
Q

t(15;17)

A

APL

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25
Q

translocation of retinoic acid receptor (RAR) on chr. 17 to chr. 15; RAR disruption blocks the maturation and promyelocytes (blasts) accumulate

A

t(15;17) in APL

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26
Q

increased risk of DIC due to numerous primary granules (auer rods) in promyeloctyes

A

APL

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27
Q

Treatment of APL

A

ATRA (all-trans-retinoic-acid) which binds the altered RAR receptor and allows for maturation

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28
Q

Acute monocytic leukemia

A

proliferation of monoblasts; lack MPO

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29
Q

blasts characteristically infiltrate the GUMS

A

Acute monocytic leukemia

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30
Q

acute megakaryoblastic leukemia

A

lack MPO, proliferation of megakaryoblasts

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31
Q

Associated with down syndrome (except unlike ALL it arises before the age of 5 years old)

A

ACUTE MEGAKARYOBLASTIC LEUKEMIA

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32
Q

arises mainly from pre-existing dysplasia (myelodysplastic syndromes)

A

AML (acute myeloid leukemia)

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33
Q

Increased blast but still under 20%, hypercellular bone marrrow, and cytopenia

A

myelodisplastic syndrome

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34
Q

Neoplastic proliferation of MATURE circulating lymphocytes. characterized by a high white blood cell count

A

Chronic Leukemia

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35
Q

CLL

A

chronic lymphocytic leukemia

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36
Q

Neoplastic proliferation of naive B cells that co-express CD5 and CD20

A

CLL

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37
Q

Most common leukemia overall

A

CLL

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38
Q

Smudge cell

A

CLL

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39
Q

increased lymphocytes and smudge cells are seen in blood smear

A

CLL

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40
Q

Most common cause of death in CLL?

A

hypogammaglobulinemia (i.e. infection kills them)

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41
Q

3 Complications of CLL

A

1) hypogammaglobulinemia
2) Autoimmune hemolytic anemia
3) Richter transformation

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42
Q

Richter transformation

A

CLL transforms to diffuse large b-cell lymphoma (marked clinically by enlarged lymph node or spleen)

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43
Q

SLL

A

small lymphocytic lymphoma commonly arises from CLL (common lymphocytic leukemia) when involvement of lymph nodes leads to —> LAD (lymphadenopathy)

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44
Q

Hairy Cell Leukemia

A

neoplastic proliferation of mature B cells characterized by hairy cytoplasmic processes.

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45
Q

Cells are positive for tartrate-resistant acid phosphatase (TRAP+)

A

Hairy Cell Leukemia

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46
Q

TRAP+ stain, trapped in red pulp (splenomegaly), trapped in bone marrow (LAD absent). Also bone marrow fibrosis (dry tap)

A

Hairy cell leukemia

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47
Q

Treatment for Hairy Cell Leukemia

A

ADA inhibitor 2-CDA (cladribine)

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48
Q

ATLL

A

Adult T-Cell Leukemia/Lymphoma

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49
Q

Neoplastic proliferation of mature CD4+ T-cells

A

ATLL

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50
Q

associated with HTLV-1 (seen in japan and caribbean)

A

ATLL

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51
Q

rash (skin infiltration), LAD, hepatosplenomegaly, lytic bone lesions and hypercalcemia

A

ATLL

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52
Q

Mycosis fungoides

A

neoplastic proliferation of mature CD4+ t cells that infiltrate the skin, producing localized skin rash, plaques and nodules.

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53
Q

Pautrier microabscesses

A

aggregates of neoplastic cells in epidermis. seen in mycosis fungoides

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54
Q

characteristic lymphocytes with cerebriform nuclei (SEZARY CELLS) are seen in blood smear

A

mycosis fungoides

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55
Q

MPD

A

myeloproliferative disorders

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56
Q
  • disease of late adulthood (50-60)
  • high WBC with hypercellular bone marrow
  • neoplastic proliferation of mature cells in myeloid lineage
A

myeloproliferative disorders

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57
Q

MPDs include

A
  • CML (chronic myeloid leukemia)
  • PV (polycythemia vera)
  • ET (essential thrombocythemia)
  • myelofibrosis
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58
Q

BASOPHILIA is characteristically seen in

A

CML (chronic myeloid leukemia)

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59
Q

neoplastic proliferation of mature myeloid cells, especially granulocytes and their precursors (basophils)

A

CML

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60
Q

BCR -ABL fusion protein with increased tyrosine kinase activity

A

t(9;22) phili chromosome. seen in CML.

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61
Q

Patient with a 9;22 translocation presents to clinic with basophilia and splenomegaly. You are worried his CML is progressing to the accelerated phase of the disease. What are you most worried about ?

A

Disease progression to AML (2/3 of all cases) or ALL (1/3 of all cases)

62
Q

3 ways to distinguish CML from a leukemoid rxn.

A

1) Basophilia in CML
2) LAP negative (leukocyte alkaline phosphatase)
3) t(9;22) in CML

63
Q

PV (polycythemia vera)

A

proliferation of mature myeloid cells, especially red blood cells (granulocytes and platelets also increased)

64
Q

A myeloproliferative disorders associated with a JAK 2 kinase mutation

A

PV, ET, and myelofibrosis

65
Q

Clinical symptoms of PV are mostly due to

A

hyperviscosity of the blood

66
Q

Symptoms of PV:

A

1) blurry vision and head ache
2) increased risk of venous thrombosis
3) plethora (flushed face due to congestion)
4) ITCHING AFTER BATHING (increased histamine and mast cells)

67
Q

Ischemic infarction to liver is seen in Budd-Chiari syndrome. What MPDs most likely caused this syndrome?

A

PV

68
Q

PV treatment

A

phlebotomy or second-line hydroxyurea

69
Q

PV must be distinguished from reactive polycythemia. How?

A

In PV:

  • EPO is decreased (not increased like in renal cell carcinoma ectopic EPO reactive polycythemia)
  • SaO2 normal
70
Q

Neoplastic proliferation of platelets; RBCs and granulocytes are also increased

A

ET (essential thrombocythemia)

71
Q

True or false: ET rarely progresses to marrow fibrosis or acute leukemia and has no significant risk for hyperuricemia or gout

A

TRUE (platelets are simply blebs off of megakaryocytes aka have no nucleus aka no purine degradation)

72
Q

Myelofibrosis

A

neoplastic proliferation of mature myeloid cells; especially megakaryocytes

73
Q

megakaryocytes produces excess PDGF causing marrow fibrosis

A

Myelofibrosis

74
Q

Clinical triad of myelofribrosis

A

1) Splenomegaly (due to extramedullary hematopoiesis…stem cells have migrated back)
2) Leukoerythroblastic smear (tear drop)
3) Increased risk of infection, thrombosis and bleeding

75
Q

when is painful LAD usually occur?

A

lymph nodes that are draining a region of acute infection (acute lymphadenitis)

76
Q

when does painless LAD usually occur?

A
  • chronic inflammation
  • metastatic carcinoma
  • lymphoma
77
Q

Follicular hyperplasia (b-cell region) is seen in what?

A

HIV infection (dedritic cells) or RA

78
Q

paracortex hyperplasia (T-cell region) seen in what?

A

viral infection (IM)

79
Q

neoplastic proliferation of lymphoid cells that form a mass; may arise in a lymph node or in extranodal tissue

A

lymphoma

80
Q

NHL

A

non-hodgkin lymphoma (60% of lymphomas)

81
Q

HL

A

Hodgkin lymphoma (40% of lymphomas)

82
Q

NHL with small B cells

A

follicular lymphoma, mantle cell lymphoma, marginal zone lymphoma and SLL (remember from CLL)

83
Q

NHL with intermediate sized cells

A

Burkitt lymphoma

84
Q

NHL with Large B cells

A

Diffuse large B-cell lymphoma (DLBCL)

85
Q

malignant cells of hodgkin lymphoma

A

reed-sternberg cells

86
Q

malignant cells of NHL

A

lymphoid cells

87
Q

reed-sternberg cell composition

A

reactive cells (inflammatory cells and fibrosis)

88
Q

painless LAD usually arises late in life

A

NHL

89
Q

Painless LAD occasionally with “B” symptoms, usually arise in young adults (female)

A

HL

90
Q

Neoplastic proliferation of small B cells (CD20+) that form follicle like nodules

A

Follicular lymphoma

91
Q

driven by t(14;18)

A

follicular lymphoma

92
Q

t(14;18)

A
  • follicular lymphoma

- BCL2 on 18 translocates to the IgHeavy chain locus on chromosome 14. Anti-apoptotic upregulated.

93
Q

rituximab is an anti-CD20 antibody used to treat

A

follicular lymphoma

94
Q

Follicular lymphoma can progress to

A

diffuse large b-cell lymphoma (presents as an enlarging lymph node)

95
Q

lack tingle body macrophages in germinal centers

A

follicular lymphoma

96
Q

Bcl2 expression in follicule

A

follicular lymphoma

97
Q

mantle cell lymphoma

A

proliferation of small b-cells (CD20+) that expands the mantle zone

98
Q

t(11;14)

A

Mantle cell lymphoma

99
Q

Driven by t(11;14). Explain

A

Mantle cell lymphoma; Cyclin D1 (G1–>S) gene on 11 translocates to 14 IgHeavy. Upregulated. facilitates neoplastic proliferation.

100
Q

Marginal zone lymphoma (remember this is still a NHL)

A

proliferation of small b-cells (CD20+) that expands the marginal zone

101
Q

marginal zone is formed by ?

A

post-germinal b-cells (memory b-cells)

102
Q

An NHL associated with H.pylori gastritis, hashimoto thyroiditis, sjogren syndrome

A

Marginal zone lymphoma

103
Q

MALToma

A

marginal zone lymphoma in mucosal sights

104
Q

Gastric MALToma may regress with treatment of …

A

H. Pylori

105
Q

Neoplastic proliferation of inter-mediate sized b-cells (CD20+); associated with EBV and classically presents as an extranodal mass in a child or young adult

A

Burkitt Lymphoma

106
Q

African form of Burkitt lymphoma involves…

A

the jaw

107
Q

sporadic form of Burkitt lymphoma usually involves …

A

abdomen

108
Q

driven by t(8;14). Explain

A
  • Burkitt lymphoma
  • C-myc on 8 translocates to IgHeavy on 14
  • overexpression of c-myc oncogene promotes cell growth
109
Q

characterized by high mitotic index and starry-sky appearance on microscopy

A

Burkitt lymphoma

110
Q

most common form of NHL?

A

Diffuse Large B-Cell Lymphoma

111
Q

neoplastic proliferation of diffuse large b-cells (CD20+) that grow diffusely in sheets. clinically aggressive / high grade

A

Diffuse Large B-Cell Lymphoma

112
Q

arises sporadically or from transformation of low-grade lymphoma (follicular lymphoma)

A

Diffuse Large B-Cell Lymphoma

113
Q

Reed-Sternberg (RS) cells are what? and are usually positive for what?

A

Hodgkin Lymphoma: large B-cells with multi-lobed nuclei and prominent nucleoli (owl-eyed); classically CD15+ and CD30+

114
Q

RS cells secrete cytokines resulting in B symptoms which include

A

fever, chills, weight loss, and night sweats

115
Q

Reactive inflammatory cells make up a bulk of the tumor and form the basis for classification of HL. Subtypes include:

A

Nodular sclerosis
Lymphocyte-rich
Lymphocyte-depleted
Mixed Cellularity

116
Q

most common for of HL

A

Nodular sclerosis (70%)

117
Q

presents as enlarging cervical or mediastinal lymph node in young adult, usually female. Lymph node is divided by a band of sclerosis

A

Nodular sclerosis HL

118
Q

RS cells present in lake-like spaces (lacunar cells)

A

Nodular sclerosis HL

119
Q

Best prognosis of HL subtypes

A

lymphocyte rich HL

120
Q

Eosinophila seen in what?

A

Mixed Cellularity HL

121
Q

RS cells produce what resulting in eosinophila in mixed cellularity HL

A

IL-5

122
Q

What is the most aggressive subtype of HL (hint: it is usually seen in the elderly and HIV positive individuals)

A

Lymphocyte-depleted

123
Q

Dyscrasias

A

plasma cell disorders

124
Q

multiple myeloma, MGUS, and waldenstrom macroglobulinemia are examples of

A

plasma cell disorders

125
Q

malignant proliferation of plasma cells in bone marrow

A

multiple myeloma

126
Q

most common primary malignancy of bone

A

multiple myeloma

127
Q

High serum IL-6 may be present in what? what does IL-6 do?

A
  • multiple myeloma

- IL-6 stimulates plasma cell growth and immunoglobulin production

128
Q

Multiple Myeloma clinical features

A

see page 63 of pathoma

129
Q

MGUS

A

Monoclonal gammopathy of undetermined significance

130
Q

increased serum M spike on SPEP; other features of multiple myeloma are absent: no lytic bone leason, hypercalcemia, AL amyloid, or Bence Jones proteinuria)

A

MGUS

131
Q

B-cell lymphoma with monoclonal IgM production

A

Waldenstrom Macroglobulinemia

132
Q

Increased serum protein M-spike (comprised of IgM). Generalized LAD with no lytic bone lesions.

A

Waldenstrom Macroglobulinemia

133
Q

Visual and neurologic deficits (retinal hemorrhage or stroke)–IgM pentamer is large and causes serum hyperviscosity–> defective platelet aggregation–>bleeding

A

Waldenstrom Macroglobulinemia

134
Q

treatment for Waldenstrom Macroglobulinemia

A

plamaspheresis

135
Q

neoplastic proliferation of langerhan cells

A

langerhans cell histiocytosis

136
Q

What are langerhan cells?

A

–> specialized dendritic cells founds predominantly in the skin (derived from bone marrow monocytes). They present antigen to naive T-cells.

137
Q

Langerhans cell histiocytosis diseases (3)

A
  • Letter-Swiwe Disease
  • Eosinophilic granuloma
  • Hand-Schuler-Christain disease
138
Q

Characteristic birbeck (tennis racket) granules seen on electron microscopy.

A

Langerhans cell histiocytosis

139
Q

CD1a+ and S100+

A

Langerhans cell histiocytosis

140
Q
  • malignant proliferation of langerhans cells

- classic presentation is skin rash and cystic skeletal defects in an infant (

A

Letter-Swiwe Disease

141
Q

Benign proliferation of langerhans cells in bone. Classic presentation is pathologic fracture in adolescent.

A

Eosinophilic Granuloma

142
Q

Malignant proliferation of langerhans cells. Classic presentation of is scalp rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child.

A

Hand-Schuler-Christain Disease

143
Q

Name four chronic leukemias

A
  • CLL
  • Hairy Cell Leukemia
  • ATLL
  • Mycosis Fungoides
144
Q

In mycosis fungoides neoplastic mature CD4+ T-cells can aggregate in the epidermis to produces Pautrier microabscesses or can spread to involve the blood, producing _______ _________.

A

Sezary Syndrome

145
Q

what cancers have to capability of progressing to DLBCL?

A

CLL and follicular lymphoma

146
Q

Co-expression of CD10, CD19, and CD20 are seen in what two cancers? how do you tell them apart?

A
  • B-ALL and Follicular lymphoma and Burkitt lymphoma
  • Follicular lymphoma is Bcl2+ and B-ALL is TdT+
  • Burkitt lymphoma t(8;14) (increased c-myc)
147
Q

Co-expression of CD5, CD20 (CD19) is seen in what two cancers? How do you tell them apart?

A
  • Mantle Cell lymphoma and CLL
  • CLL is usually CD23+ (also look for smudge cells)
  • Mantle is CD23- and Cyclin D1+ with possible lymphomatoid polyposis (GI)
  • MANTLE= surface light chain restriction BRIGHT
  • CLL/SLL= surface light chain restriction DIM
148
Q

increased small mature lymphs with hyperclumped nuclear chromatin (soccer ball cells) and smudge cells

A

CLL/SLL

149
Q

The following are buzzwords for ________ _______? :

“Spreads in a contiguous fashion” ; “Reed-Sternberg Cell” ; “bimodal”

A

HL

150
Q

EBV can be associated with what three cancers?

A

Hodgkin Lymphoma, DLBCL, and Burkitt Lymphoma

Pathoma (1 decks)

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