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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
* Malignant hyperplasia of the multipotential myeloid stem cell causes increase in all cell lines “pancytosis” (particularly RBCs → WBCs + Platelets) * High blood viscosity
Polycythemia Vera
24
LAP in PV
Increased
25
Distinct gene for PV
JAK2 oncogene
26
ESR in PV
Probability of low ESR due to viscous blood
27
proliferation of granulocytes (granulocytosis)
Chronic Myelogenous Leukemia (CML)
28
Bone marrow has an increased M:E ratio (25:1)
CML
29
Blast crisis
CML
29
LAP in CML
Low
30
CML has this chromosome
Philadelphia chromosome t(9;22)
31
Proliferation of megakaryocytes
Essential Thrombocythemia (ET)
32
Platelets >1000 x 109/L (Giant forms, platelet function abnormalities)
Essential Thrombocythemia (ET)
33
Myeloid stem cell disorder characterized by proliferation of erythroid, granulocytic, and megakaryocytic precursors in marrow with dyspoiesis
Chronic Idiopathic Myelofibrosis
34
Progressive marrow fibrosis (fibrin clots may accumulate causing blood vessel blockage → stroke, heart attack
Chronic Idiopathic Myelofibrosis
35
Distinct cell found in Chronic Idiopathic Myelofibrosis
Teardrop cells (RBC)
36
Group of acquired clonal disorders affecting pluripotential stem cells
MYELODYSPLASTIC SYNDROMES (MSDs)
37
Progressive blood cytopenias, despite bone marrow hyperplasia
MYELODYSPLASTIC SYNDROMES (MSDs)
38
High incidence of terminating in acute myelogenous leukemia
MYELODYSPLASTIC SYNDROMES (MSDs)
39
MDS development is triggered by
chemotherapy, radiation, chemicals
40
WHO classification of MDS has an additional groups
Refractory cytopenia with multilineage dysplasia, 5q deletion syndrome
41
5 subgroups of MDS using the FAB classification scheme (up to 30% blasts in bone marrow)
RA RARS CMML RAEB RAEB-t
42
Anemia that is refractory (not responsive) to therapy
Refractory anemia (RA)
43
Refractory anemia (RA) BM blasts: PB blasts:
BM blasts: <5% PB blasts: <1%
44
Ringed sideroblasts comprise >15% of bone marrow nucleated cells Dimorphic erythrocytes
RARS
45
Leukocytosis BM blasts: 5-20% PB blast: <5% Absolute monocytosis >1.0 x 109/L
CMML
46
BM blasts: 5-20% PB blast: <5% NO absolute monocytosis
RAEB
47
Trilineage (RBC, WBC, platelet) cytopenias
RAEB
48
WHO classification reassigns this MDS as an ACUTE LEUKEMIA
RAEB-t
49
RAEB-t BM blasts: PB blasts:
BM blasts: >20-<30% PB blasts: >5%
50
ALL is differentiated based on morphology including:
* Cell size * Prominence of nucleoli * Amount and appearance of cytoplasm
50
CMML, RAEB BM blasts: PB blasts:
BM blasts: 5-20% PB blasts: <5%
51
most common cancer in children, 23% of cancer diagnoses among children <15 years of age
ACUTE LYMPHOBLASTIC LEUKEMIA
52
results from the malignant transformation of normal developing T cells in the thymus, the so-called THYMOCYTES
T-cell acute Lymphoblastic leukemia (T-ALL)
53
Small homogenous lymphoblasts Scanty cytoplasm, inconspicuous nucleoli Round irregular/indistinct nucleus
ALL L1
54
Most common CHILDHOOD ALL with best prognosis
ALL L1
55
Large heterogenous lymphoblasts Abundant basophilic cytoplasm Clefted nuclei Nucleoli present
ALL L2
55
WHO CLASSIFICATION OF ALL is based on
Phenotyping
56
Adult type ALL
ALL L2
56
Burkitt type ALL
L3
57
Large homogenous lymphoblast VACUOLATED Rarest
ALL L3
57
WHO classification of ALL
PRECURSOR B-CELL ALL PRECURSOR T-CELL ALL MATURE B-CELL ALL
58
ALL with poor prognosis
ALL L3
59
* 75% of cases occur in children <6 y/o * 85% of ALL are this type
PRECURSOR B-CELL ALL
60
(+): 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
PRECURSOR B-CELL ALL
61
Neoplasm of lymphoblasts committed to the B-cell lineage
PRECURSOR B-CELL ALL
62
under PRECURSOR B-CELL ALL
o Early Pre-B cell ALL o Common ALL o Pre-B cell ALL
63
* 15% of childhood ALL * 25% of adult ALL
PRECURSOR T-CELL ALL
64
* Leukocyte count: often markedly elevated * Lymphoblasts are similar to those in precursor B-cell ALL with a wide variation in morphology
PRECURSOR T-CELL ALL
65
Under PRECURSOR T-CELL ALL
o Early T cell ALL o Intermediate T-ALL o Mature T-ALL
66
Neoplasm of lymphoblasts committed to the T-cell lineage
PRECURSOR T-CELL ALL
67
expressed by specific cell lines at different maturation stages
Cluster differentiation (CD) markers
68
* B cell malignancy (CD19, CD20 positive) * Smudge cells * Small lymphocyte lymphoma – CLL lymphoma phase
Chronic Lymphocytic Leukemia (CLL)
69
CHRONIC LYMPHOPROLIFERATIVE DISORDERS
CLL HCL Prolymphocytic leukemia
70
* B cell malignancy (CD19, CD20 positive) * Dry tap on bone marrow aspiration * TRAP stain positive
Hairy Cell Leukemia (HCL)
71
* B cell (most common) or T cell malignancy
Prolymphocytic leukemia
72
Plasma cell neoplasms
MULTIPLE MYELOMA WALDENSTROM MACROGLOBULINEMIA
73
Monoclonal gammopathy causes B cell production of excess IgG or IgA
MM
74
Identified on serum protein electrophoresis by an “M” spike in the gamma globulin region
MM
75
* Bone tumors * Increased blood viscosity * Prolonged bleeding * Bence Jones proteins (free light chains-kappa or lambda)
MM
76
Monoclonal gammopathy causes B cell production of excess IgM (macroglobulin) and decreased production of other immunoglobulins
WALDENSTROM MACROGLOBULINEMIA
77
* Lymphadenopathy and hepatosplenomegaly * No bone tumors * Increased blood viscosity
WALDENSTROM MACROGLOBULINEMIA
78
T/F WHO classification considers CLL and SLL (small lymphocytic leukemia) as one entity with different clinical presentations
T
79
CL L vs SLL
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
Proliferation of malignant cells in solid lymphatic tissues
LYMPHOMAS
81
→ 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)
Lymphomas
81
→ includes various forms of leukemias and malignant lymphomas that are of lymphoreticular origin.
Lymphoproliferative disorder
81
METHODS for lymphomas
Tissue biopsy, CD4 marker identification, Cytogenetics, PCR
82
aka Classical Hodgkin Lymphoma, Nodular Lymphocyte Predominant Hodgkin Lymphoma
Hodgkin Lymphoma
83
Aneuploidy, or a deviation from the diploid no. of chromosomes Gain of chromosomes 1, 2, 5, 12, and 21 is a recurring numerical abnormality
Hodgkin Lymphoma
84
Structural rearrangements involving chromosome 1 are frequently observed
Hodgkin Lymphoma
85
– Follicular lymphoma – Multiple myeloma – Chronic lymphocytic lymphoma
Low-grade/Indolent type NHL
85
– diffuse large B-cell lymphoma – most frequent type of NHL – Burkitt’s lymphoma
High-grade/Aggressive type NHL
86
40% of lymphomas
HODGKIN
86
3 different types of diffuse large B-cell lymphomas
1. Germinal center, B-cell–like lymphoma 2. Activated B-cell–like lymphoma 3. Type 3 diffuse large B-cell lymphoma (NEW)
87
Affected lineage in HOGKIN
B-cell lineage
87
WHO classification of HODGKIN
Nodular sclerosis (70%) Mixed cellularity (20%) Lymphocyte rich Lymphocyte depleted
87
Associated with EBV
HODGKIN
88
60% of lymphomas
HODGKIN
89
B cell neoplasms in NON HODGKIN
a) Mantle cell b) Follicular c) Burkitt
90
Skin itching, leading to ulcerative tumors Sezary syndrome CD2, CD3, CD4 (+)
MYCOSIS FUNGOIDES (CUTANEOUS T CELL LYMPHOMA)
91
leukemic phase of cutaneous T-cell lymphoma
Sezary Syndrome
92
typically the size of a small lymphocyte and has a dark-staining, clumped, nuclear chromatin pattern
Sézary cell
93
derived from mature or post thymic T cells
Mature T-Cell & NK-Cell Neoplasms
94
Used for cytochemistry studies
Fixatives containing alcohol (methanol or ethanol), acetone, formaldehyde
95
Leukocyte count reference value
3.4 – 9.7 X 109/L
96
Found in the central part of smear
Lymphocytes
97
Found in the edge part of the smear
Monocytes Granulocytes

MTAP 2 (23 decks)

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