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MTAP 2 > Hema > Flashcards

Hema Flashcards

1
Q

Following a myelodysplastic syndrome or myeloproliferative disorder
* Without antecedent myelodysplastic syndrome

A

Acute myeloid leukemia with multilineage dysplasia

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2
Q

Abnormalities in granulopoiesis include hypogranular cytoplasm and hypolobulated or
bizarrely segmented nuclei

A

Acute myeloid leukemia with multilineage dysplasia

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3
Q

Acute myeloid leukemia with multilineage dysplasia usually occurs in these age grp

A

ADULTS

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4
Q

characterized by ringed sideroblasts, vacuolated cytoplasm, and
nuclei that are multiple, fragmented, or megaloblastic

A

Abnormal erythropoiesis

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5
Q

small or have single-lobed or multiple, discrete nuclei in AML with multilineage dysplasia

A

Abnormal megakaryocytes

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6
Q

In MDS, from alkylating agent result, it may progress to this type of AML

A

AML with maturation (M2)

Less common: M4, M5, M6, M7

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7
Q

AML (FAB) result from topoisomerase type II inhibitors

A

M4
M5

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8
Q

average interval between topoisomerase II inhibitors treatment and AML

A

33 months
(without passing MDS phase)

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9
Q

examples of Topoisomerase type II inhibitor

A

etoposide
doxorubicin

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10
Q

Classifies CML by chronic, accelerated, or blast phase

A

WHO

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11
Q

typically seen in blood smear with CML

A

small megakaryocytes
granulocytic hyperplasia

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12
Q

Describe chronic phase of CML

A

▪ mild anemia; leukocytosis (>25x109
/L)
▪ increased myelocytes
and mature neutrophils
▪ Basophils increased
▪ Eosinophilia
▪ Serum LDH and uric acid – increased (due to excessive cell proliferation)

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13
Q

Describe ACCELERATED PHASE of CML

A

Fulfilling at least one of the following:

o Increasing WBC ct
o Increased spleen size
o Persistent thrombocytopenia (<100 x109
/L) or thrombocytosis (>1000 x 109
/L)
o Blast: 10-19%
o Basophilia: > 20%
o Clonal evolution

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14
Q

Describe BLAST PHASE of CML

A

one or more of these features:

o Blast: >20% of peripheral WBCs or nucleated BM cells

o Blast proliferating in extramedullary site (skin, lymph node, spleen)

o Large aggregates of blasts in BM

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15
Q

WHO DIAGNOSTIC CRITERIA FOR CML:

A
  1. Persistent monocytosis (>1x109
    /L)
  2. No Philadelphia chromosome on BCR/ABR fusion gene
  3. <20% blasts (myeloblasts, monoblast, or promonocyte)
  4. Dysplasia in >1 myeloid lines
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16
Q

Characterized by hypercellular marrow, erythrocytosis, granulocytosis, and thrombocytosis, myelofibrosis

A

CHRONIC MYELOPROLIFERATIVE DISORDERS

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17
Q

All may terminate in ACUTE LEUKEMIA

A

CHRONIC MYELOPROLIFERATIVE DISORDERS

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17
Q

Defect of the myeloid stem cell (named for the cell line most greatly affected)

A

CHRONIC MYELOPROLIFERATIVE DISORDERS

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18
Q

– gene that instructs protein production that
promotes cell growth and development

A

Janus kinase 2 (JAK2) oncogene

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18
Q

Molecular diagnostic studies (helpful) for this disorders

A

CHRONIC MYELOPROLIFERATIVE DISORDERS

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19
Q

Fusion gene

A

BCR/ABL oncogen

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20
Q

Conditions where JAK 2 is present

A

✓ Polycythemia vera
✓ Chronic idiopathic myelofibrosis
✓ Essential thrombocythemia

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21
Q

Conditions where BCR/ABL gene is present

A

✓ Chronic Myelogenous Leukemia
✓ Acute Lymphocytic Leukemia

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22
Q

Malignant hyperplasia of the multipotential myeloid stem cell causes increase in all cell lines; High blood viscosity

A

Hypercellular Marrow

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23
Q
  • Malignant hyperplasia of the multipotential myeloid stem cell causes increase in all cell lines “pancytosis” (particularly RBCs → WBCs + Platelets)
  • High blood viscosity
A

Polycythemia Vera

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24
Q

LAP in PV

A

Increased

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25
Q

Distinct gene for PV

A

JAK2 oncogene

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26
Q

ESR in PV

A

Probability of low ESR due to viscous blood

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27
Q

proliferation of granulocytes (granulocytosis)

A

Chronic Myelogenous Leukemia (CML)

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28
Q

Bone marrow has an increased M:E ratio (25:1)

A

CML

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29
Q

Blast crisis

A

CML

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29
Q

LAP in CML

A

Low

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30
Q

CML has this chromosome

A

Philadelphia chromosome t(9;22)

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31
Q

Proliferation of megakaryocytes

A

Essential Thrombocythemia (ET)

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32
Q

Platelets >1000 x 109/L (Giant forms, platelet function abnormalities)

A

Essential Thrombocythemia (ET)

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33
Q

Myeloid stem cell disorder characterized by proliferation of erythroid, granulocytic, and
megakaryocytic precursors in marrow with dyspoiesis

A

Chronic Idiopathic Myelofibrosis

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34
Q

Progressive marrow fibrosis (fibrin clots may accumulate causing blood vessel blockage → stroke,
heart attack

A

Chronic Idiopathic Myelofibrosis

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35
Q

Distinct cell found in Chronic Idiopathic Myelofibrosis

A

Teardrop cells (RBC)

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36
Q

Group of acquired clonal disorders affecting pluripotential stem cells

A

MYELODYSPLASTIC SYNDROMES (MSDs)

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37
Q

Progressive blood cytopenias, despite bone marrow hyperplasia

A

MYELODYSPLASTIC SYNDROMES (MSDs)

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38
Q

High incidence of terminating in acute myelogenous leukemia

A

MYELODYSPLASTIC SYNDROMES (MSDs)

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39
Q

MDS development is triggered by

A

chemotherapy, radiation, chemicals

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40
Q

WHO classification of MDS has an additional groups

A

Refractory cytopenia with multilineage dysplasia,

5q deletion syndrome

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41
Q

5 subgroups of MDS using the FAB classification scheme (up to 30% blasts in bone marrow)

A

RA
RARS
CMML
RAEB
RAEB-t

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42
Q

Anemia that is refractory (not responsive) to therapy

A

Refractory anemia (RA)

43
Q

Refractory anemia (RA)
BM blasts:
PB blasts:

A

BM blasts: <5%
PB blasts: <1%

44
Q

Ringed sideroblasts comprise >15% of bone marrow nucleated cells

Dimorphic erythrocytes

A

RARS

45
Q

Leukocytosis
BM blasts: 5-20%
PB blast: <5%
Absolute monocytosis >1.0 x 109/L

A

CMML

46
Q

BM blasts: 5-20%
PB blast: <5%
NO absolute monocytosis

A

RAEB

47
Q

Trilineage (RBC, WBC, platelet) cytopenias

A

RAEB

48
Q

WHO classification reassigns this MDS as an ACUTE LEUKEMIA

A

RAEB-t

49
Q

RAEB-t
BM blasts:
PB blasts:

A

BM blasts: >20-<30%
PB blasts: >5%

50
Q

ALL is differentiated based on morphology including:

A
  • Cell size
  • Prominence of nucleoli
  • Amount and appearance of cytoplasm
50
Q

CMML, RAEB
BM blasts:
PB blasts:

A

BM blasts: 5-20%
PB blasts: <5%

51
Q

most common cancer in children, 23% of cancer diagnoses among children <15 years of age

A

ACUTE LYMPHOBLASTIC LEUKEMIA

52
Q

results from the malignant transformation of normal developing T cells in the thymus, the so-called THYMOCYTES

A

T-cell acute Lymphoblastic leukemia (T-ALL)

53
Q

Small homogenous lymphoblasts
Scanty cytoplasm, inconspicuous nucleoli
Round irregular/indistinct nucleus

A

ALL L1

54
Q

Most common CHILDHOOD ALL with best prognosis

A

ALL L1

55
Q

Large heterogenous lymphoblasts
Abundant basophilic cytoplasm
Clefted nuclei
Nucleoli present

A

ALL L2

55
Q

WHO CLASSIFICATION OF ALL is based on

A

Phenotyping

56
Q

Adult type ALL

A

ALL L2

56
Q

Burkitt type ALL

A

L3

57
Q

Large homogenous lymphoblast
VACUOLATED
Rarest

A

ALL L3

57
Q

WHO classification of ALL

A

PRECURSOR B-CELL ALL
PRECURSOR T-CELL ALL
MATURE B-CELL ALL

58
Q

ALL with poor prognosis

A

ALL L3

59
Q
  • 75% of cases occur in children <6 y/o
  • 85% of ALL are this type
A

PRECURSOR B-CELL ALL

60
Q

(+): enlarged lymph nodes, liver, spleen
* Leukocyte count: variable
* Lymphoblasts are pleomorphic and vary from small to large with nuclei having prominent or
inconspicuous nucleoli, compact or dispersed chromatin
* Blue or blue-gray cytoplasm is usually scant (but may be abundant)
* Coarse azurophilic granules may be present

A

PRECURSOR B-CELL ALL

61
Q

Neoplasm of lymphoblasts committed to the B-cell lineage

A

PRECURSOR B-CELL ALL

62
Q

under PRECURSOR B-CELL ALL

A

o Early Pre-B cell ALL
o Common ALL
o Pre-B cell ALL

63
Q
  • 15% of childhood ALL
  • 25% of adult ALL
A

PRECURSOR T-CELL ALL

64
Q
  • Leukocyte count: often markedly elevated
  • Lymphoblasts are similar to those in precursor B-cell ALL with a wide variation in morphology
A

PRECURSOR T-CELL ALL

65
Q

Under PRECURSOR T-CELL ALL

A

o Early T cell ALL
o Intermediate T-ALL
o Mature T-ALL

66
Q

Neoplasm of lymphoblasts committed to the T-cell lineage

A

PRECURSOR T-CELL ALL

67
Q

expressed by specific cell lines at different maturation stages

A

Cluster differentiation (CD) markers

68
Q
  • B cell malignancy (CD19, CD20 positive)
  • Smudge cells
  • Small lymphocyte lymphoma – CLL lymphoma phase
A

Chronic Lymphocytic Leukemia (CLL)

69
Q

CHRONIC LYMPHOPROLIFERATIVE DISORDERS

A

CLL
HCL
Prolymphocytic leukemia

70
Q
  • B cell malignancy (CD19, CD20 positive)
  • Dry tap on bone marrow aspiration
  • TRAP stain positive
A

Hairy Cell Leukemia (HCL)

71
Q
  • B cell (most common) or T cell malignancy
A

Prolymphocytic leukemia

72
Q

Plasma cell neoplasms

A

MULTIPLE MYELOMA

WALDENSTROM MACROGLOBULINEMIA

73
Q

Monoclonal gammopathy causes B cell production of excess IgG or IgA

A

MM

74
Q

Identified on serum protein
electrophoresis by an “M” spike in the gamma globulin region

A

MM

75
Q
  • Bone tumors
  • Increased blood viscosity
  • Prolonged bleeding
  • Bence Jones proteins (free light
    chains-kappa or lambda)
A

MM

76
Q

Monoclonal gammopathy causes B cell production of excess IgM (macroglobulin)
and decreased production of other
immunoglobulins

A

WALDENSTROM MACROGLOBULINEMIA

77
Q
  • Lymphadenopathy and
    hepatosplenomegaly
  • No bone tumors
  • Increased blood viscosity
A

WALDENSTROM MACROGLOBULINEMIA

78
Q

T/F

WHO classification considers CLL and SLL (small lymphocytic leukemia) as one entity with different clinical presentations

A

T

79
Q

CL L vs SLL

A

Diagnosis is based on the predominant site of involvement

CLL - peripheral blood (lymphocytosis, >10x109/L) and bone marrow involvement

SLL - lymph nodes and other lymphoid organs

80
Q

Proliferation of malignant cells in solid lymphatic tissues

A

LYMPHOMAS

81
Q

→ group of closely related disorders that are characterized by over proliferation of
one or more types of cells of the lymphoid system (lymphoreticular stem cells, lymphocytes,
reticular cells and histiocytes)

A

Lymphomas

81
Q

→ includes various forms of leukemias and malignant lymphomas
that are of lymphoreticular origin.

A

Lymphoproliferative disorder

81
Q

METHODS for lymphomas

A

Tissue biopsy,
CD4 marker identification,
Cytogenetics,
PCR

82
Q

aka Classical Hodgkin Lymphoma, Nodular Lymphocyte Predominant Hodgkin Lymphoma

A

Hodgkin Lymphoma

83
Q

Aneuploidy, or a deviation from the diploid no. of chromosomes

Gain of chromosomes 1, 2, 5, 12, and 21 is a recurring numerical abnormality

A

Hodgkin Lymphoma

84
Q

Structural rearrangements involving chromosome 1 are frequently observed

A

Hodgkin Lymphoma

85
Q

– Follicular lymphoma
– Multiple myeloma
– Chronic lymphocytic lymphoma

A

Low-grade/Indolent type NHL

85
Q

– diffuse large B-cell lymphoma
– most frequent type of NHL
– Burkitt’s lymphoma

A

High-grade/Aggressive type NHL

86
Q

40% of lymphomas

A

HODGKIN

86
Q

3 different types of diffuse large B-cell lymphomas

A
  1. Germinal center, B-cell–like lymphoma
  2. Activated B-cell–like lymphoma
  3. Type 3 diffuse large B-cell lymphoma (NEW)
87
Q

Affected lineage in HOGKIN

A

B-cell lineage

87
Q

WHO classification of HODGKIN

A

Nodular sclerosis (70%)
Mixed cellularity (20%)
Lymphocyte rich
Lymphocyte depleted

87
Q

Associated with EBV

A

HODGKIN

88
Q

60% of lymphomas

A

HODGKIN

89
Q

B cell neoplasms in NON HODGKIN

A

a) Mantle cell
b) Follicular
c) Burkitt

90
Q

Skin itching, leading to ulcerative tumors

Sezary syndrome

CD2, CD3, CD4 (+)

A

MYCOSIS FUNGOIDES
(CUTANEOUS T CELL
LYMPHOMA)

91
Q

leukemic phase of cutaneous T-cell lymphoma

A

Sezary Syndrome

92
Q

typically the size of a small lymphocyte and has a dark-staining, clumped, nuclear chromatin pattern

A

Sézary cell

93
Q

derived from mature or post thymic T cells

A

Mature T-Cell & NK-Cell Neoplasms

94
Q

Used for cytochemistry studies

A

Fixatives containing alcohol (methanol or ethanol), acetone, formaldehyde

95
Q

Leukocyte count reference value

A

3.4 – 9.7 X 109/L

96
Q

Found in the central part of smear

A

Lymphocytes

97
Q

Found in the edge part of the smear

A

Monocytes
Granulocytes

MTAP 2 (23 decks)

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