Hematologic Malignancies Flashcards
(256 cards)
1
Q
neoplasm of lymphoblasts committed to the B-cell lineage.
A
precursor B-cell ALL
2
Q
neoplasm of lymphoblasts committed to the T-cell lineage.
A
Precursor T lymphoblastic leukemia/lymphoblastic
lymphoma (precursor T-cell ALL)
3
Q
ACUTE LEUKEMIA (FAB CLASSIFICATION)
small cells
predominant; nuclear shape is
regular with an occasional
cleft; chromatin pattern is
homogeneous and nucleoli
are rarely visible; cytoplasm is
moderately basophilic
A
L1 (homogeneous)
4
Q
ACUTE LEUKEMIA (FAB CLASSIFICATION)
Large cells with an irregular
nuclear shape; clefts in the
nucleus are common; one or
more large nucleoli are visible;
cytoplasm varies in color
A
L2 (heterogeneous)
5
Q
ACUTE LEUKEMIA (FAB CLASSIFICATION)
Cells are large and homogeneous in size; nuclear
shape is round or oval; one to three prominent nucleoli;
cytoplasm is deeply basophilic with numerous
vacuoles
A
L3 (Burkitt lymphoma type)
6
Q
Lymphadenopathy and hepatomegaly are present in %
A
75%
7
Q
ALL: LABORATORY DATA
Total leukocyte count
A
elevated in 60% to 70% of
patients; ranging from 50 to 100 × 10
9/L.
8
Q
ALL: LABORATORY DATA
Approx. 25% of patients exhibits
A
Leukocytopenia
9
Q
ALL: LABORATORY DATA
predominance of _____
A
Blast Cells
10
Q
ALL: LABORATORY DATA
close to lymphoblasts, lymphocytes & smudge cells
A
100%
11
Q
Arise from malignant transformation at various stages of
development from acquired genetic abnormalities that lead to abnormal changes in cell growth and differentiation patterns.
A
Mature Lymphoid Neoplasms
12
Q
Symptoms are common in these conditions mature lymphoid neoplasms
A
unexplained weight loss( > 10% body weight) in 8 months prior to staging
unexplained persistent or recurrent fever( > 38C) in prior
month
recurrent drenching night sweats during prior month
13
Q
Characterized by the presence of leukocytosis
with an increased number of mature lymphocytes,
lymphocytosis, on a peripheral blood film
A
CHRONIC LYMPHOCYTIC LEUKEMIA / SMALL LYMPHOCYTIC LYMPHOMA
14
Q
Most common form of leukemia in adults in Western
countries but it is very rare in far Eastern countries
A
CHRONIC LYMPHOCYTIC
LEUKEMIA / SMALL
LYMPHOCYTIC LYMPHOMA
15
Q
CHRONIC LYMPHOCYTIC LEUKEMIA / SMALL
LYMPHOCYTIC LYMPHOMA
Neoplasms composed of mature small B
lymphocytes in the peripheral blood, bone marrow,
spleen, and lymph nodes, appearing functionally
incompetent that express ——
A
CD5
16
Q
median age of onset of CHRONIC LYMPHOCYTIC LEUKEMIA / SMALL LYMPHOCYTIC LYMPHOMA
A
65 years
17
Q
a biologically and clinically
heterogeneous hematologic malignancy
characterized by a gradually progressive
accumulation of morphologically mature B
lymphocytes in the blood, bone marrow, and
lymphatic tissues.
A
B-CLL
18
Q
Characterizarion of B-CLL
A
CD5+
CD19+
CD23+ monoclonal B cells
19
Q
most consistent finding which is present in approximately 50% of patients in chromosomal alteration
A
trisomy of chromosome 12
20
Q
associated with B-CLL
chromosomal alteration
A
translocation of chromosomes 8 and 14
21
Q
Chromosomal alterations:
translocation of chromosomes 9 and 22
A
Ph observed in non-T and non-B types
22
Q
B-cells display the classic surface
immunoglobulin (SIg) markers.
A
CD19
CD20
CD24
CD5
23
Q
CHRONIC LYMPHOCYTIC LEUKEMIA /
SMALL LYMPHOCYTIC LYMPHOMA
also frequently present.
A
Hepatosplenomegaly
24
Q
Uncommon chronic lymphoproliferative disorder of the B-lymphocyte type.
More common in males than in females
A
HAIRY CELL LEUKEMIA (HCL)
(Leukemic reticuloendotheliosis)
25
appearance of fine, hair-like, irregular cytoplasmic projections that are characteristic of lymphocytes in this disease
Hairy cell leukemia
26
Isoenzyme 5 is a ---- present in large amounts in the hairy cells of HCL
pyrophosphatase
27
----- may show less than complete
resistance to L(+) tartaric acid and stain faintly positive
atypical lymphocytes of infectious mononucleosis, CLL and lymphosarcoma
28
HAIRY CELL LEUKEMIA
The cytochemical features of HCL include a
strong acid phosphatase reaction that is not
inhibited by
tartaric acid or tartrate-resistant acid
phosphatase (TRAP) stain.
29
Isoenzyme ---- is present
in HCL
5
30
Immunological Markers of HCL (typical type)
CD19+
CD20+
CD22+
CD24+
CD25+
31
display strong surface immunoglobulin (SIg).
HAIRY CELL LEUKEMIA
32
Chromosomal alterations in CHRONIC LYMPHOCYTIC LEUKEMIA / SMALL LYMPHOCYTIC LYMPHOMA
13q deletion
11q deletion
trisomy of chromosome 12
17p deletion
translocation of chromosomes 8 and 14
translocation of chromosomes 9 and 22( Ph)
33
Clinical Signs & Symptoms for Chronic Lymphocytic Leukemia
Abnormal findings discovered on a complete blood count (CBC)
Common symptoms include body malaise, low-grade fever, and night sweats.
Hepatosplenomegaly is also frequently present.
34
Serum electrophoresis of Chronic Lymphocytic Leukemia shows:
hypogammaglobulinemia
35
more aggresive type of HCL
HAIRY CELL LEUKEMIA VARIANT
36
Result in differential diagnosis of vHCL from typical HCL is cytochemical staining (TRAP).
vHCL: negative
HCL : positive
37
Poor prognosis in immunophenotyping using flow cytometry
vHCL
38
presently considered as a type of chronic leukemia. The cells are also B lymphocytes, just like the classic HCL.
Hairy Cell Leukemia Variant
39
very rare form of HCL-V that shows a slightly more prominent nucleoli than the typical HCL.
Japanese Variant
40
used to differentiate similar morphology in japanese variant of HCL
polyclonal B cell lymphocytosis
41
PROLYMPHOCYTIC LEUKEMIA is a malignancy of B prolymphocytes affecting ____________
blood
bone marrow
spleen
42
Characterized by a large number of small lymphocytes with scant cytoplasm and the immature features of prolymphocytes in the peripheral blood.
PROLYMPHOCYTIC LEUKEMIA
43
Prolymphocytes must exceed------ of lymphoid cells in the peripheral blood.
55%
44
Immunological Markers of prolymphocytic leukemia
CD19+
CD20+
CD24+
CD22+
Cells display strong SIg.
45
a malignant bone marrow–based, plasma cell neoplasm associated with abnormal protein production.
MULTIPLE MYELOMA (PLASMA CELL MYELOMA)
46
a malignant bone marrow–based, plasma cell neoplasm associated with abnormal protein production.
MULTIPLE MYELOMA (PLASMA CELL MYELOMA)
47
an increased number of plasma cells in the peripheral blood and should be considered a form of multiple myeloma and not a separate entity.
Plasma cell leukemia
48
Multiple myeloma sually evolves from an asymptomatic premalignant stage of clonal plasma cell proliferation called
monoclonal gammopathy of undetermined significance (MGUS)
49
Age onset of multiple myeloma
between 40 and 70 years
peak incidence in the seventh decade of life.
50
Clinical significance of __________
Bone pain, weakness, fatigue
Abnormal bleeding - may be a prominent feature
In some patients: major symptoms result from acute infection, renal insufficiency, hypercalcemia, or amyloidosis.
Approximately 90% of patients suffer from broadly disseminated destruction of the skeleton.
Multiple myeloma
51
compensatory decrease in synthesis and increase in catabolism of normal immunoglobulins.
Multiple myeloma
52
may develop as a result of bone marrow failure in multiple myeloma
granulocytopenia
53
Electrophoresis of serum in multiple myeloma usually demonstrates the overproduction of
IgM 19Sveedbeg units
54
Protein seen in patients with multiple myeloma
Bence- Jones protein.
55
a B-cell neoplasm characterized by lymphoplasmo-proliferative disorder with infiltration of the bone marrow and a monoclonal immunoglobulin M (IgM) protein
WALDENSTRÖM PRIMARY MACROGLOBULINEMIA
56
increased viscosity which requires plasmapheresis to alleviate symptoms
Waldenstrom macroglobulinemia
57
somatic mutation in the myeloid differentiation _________ a member of the Toll-like receptor pathway and found in over 90% of patients with waldenstrom macroglobulinemia
a molecular marker for the disease and can differentiate it from other lymphomas that morphologically exhibit plasmacytic differentiation.
Factor 88 or MYD88 gene
58
Heavy Chain Diseases is a rare syndromes characterized by the production of the
gamma, alpha, mu heavy chains of immunoglobulin and soft tissue tumors
59
Lymphoproliferative disorders associated with ____ chain
gamma
60
Lymphoproliferative disorders often resembles
PLL or plasmacytoma
61
may resemble CLL
mu heavy chain disease
62
associated with MALT lymphoma.
alpha chain disease
63
a LPN characterized by secretion of a truncated gamma chain without light chain binding sites
Gamma heavy chain disease
64
most common, and involves younger age group
Manifested by malabsorption and diarrhea accompanying a massive lymphoplasmatic infiltration of intestinal mucosa, sometimes evolving to large B cell lymphoma.
Alpha heavy chain disease
65
usually with CLL, with vacuolated plasma cells in the BM
u heavy chain disease
66
group of closely related disorders that are characterized by the overproliferation of one or more types of cells of the lymphoid system such as lymphoreticular stem cells, lymphocytes, reticulum cells, and histiocytes
LYMPHOMAS
67
post-thymic neoplastic disorder of T cells associated with retroviral infection by the human T lymphotropic virus type 1( HTLV-1).
Adult T Cell Leukemia/ Lymphoma ( ATLL)
68
Human T lymphotropic virus type 1 (HTLV-1) Is transmitted via
placental circulation, breastfeeding, blood transfusion, or sexual contact.
69
medium to large sized lymphocytes, have accentuated, convoluted nuclei, coarsely clumped chromatin, and deeply basophilic cytoplasm. “ Flower cell” is coined for this morphology.
ATLL cells
70
ATLL immunophenotype is generally consistent with
T helper cells:
CD3 and CD4 are expressed
CD25 and CCR4 are highly expressed
CD7 and CD8 are absen
71
Indication of prognostic significance in ATLL can be used as a tumor marker for assessing disease status.
soluble form of IL-2
72
Treatment for HTLV-1 virus involves the combination of
interferon alpha and azidothymidine
73
Anti- CCR4 monoclonal antibody
mogamulizumab
74
reported to effect long-term survival.
Allogeneic stem cell transplantation
75
Aggressive form of cancer of mature B cells
Burkitt Lymphoma/ Leukemia (BL)
76
3 types of Burkitt Lymphoma
endemic
sporadic
HIV associated
Responsive to chemotherapy
77
mostly patients are asymptomatic. A separate scoring system, Fabry International Prognostic Index (FIBI) has been developed to aid in decision –making.
Follicular Lymphoma
78
Treatment for follicular lymphoma
combination chemotherapy like that used in other forms of NHL
79
3%-6% of non-Hodgkin lymphoma (NHL) cases, with extensive lymphadenopathy
Extranodal disease is common with GI tract as the primary area of involvement. In the indolent form, the disease is restricted to the blood, BM, and spleen.
Mantle cell lymphoma (MCL)
80
the most common form of NHL, 25%-30% belong to this type.
Diffuse Large B cell Lymphoma (DLBCL)
81
large with a diffuse pattern in the lymph node.
DLBCL cells
82
Pleomorphic forms can be seen in multiple myeloma and anaplastic large cell lymphoma and may be confused with DLBCL subtypes. Such cases need ----------------------- to rule out other lymphoid neoplasms and confirm the diagnosis.
immunochemistry and genetic testing
83
Translocation involving the BCL6 gene ----- of patients
30%
84
gene, a marker associated with t(14;18) and FL occurs in 20%to 30% of DLBCL and may complicate dx.
Translocation of the BCL2
85
typically associated with Burkitt leukemia/ lymphoma is rearranged in 10% of DLBCL patients.
MYC gene
86
standard chemotherapy has improved patient outcomes.
anti-CD20 monoclonal antibody and rituximab
87
indolent B cell lymphoma associated with chronic antigen stimulation either in the setting of infection or autoimmunity
Marginal Zone Lymphoma
88
3 subtypes of Marginal Zone Lymphoma
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue( MALT)- the most common
Splenic marginal zone lymphoma
Nodal marginal zone lymphoma- localized in the lymph node
89
T cell disorder associated with large pleomorphic cells, some small and others are medium sized.
Anaplastic Large Cell Lymphoma (ALCL)
90
ALCL expresses
CD30
91
ALCL: common in younger population, better prognosis
ALK Positive
92
ALCL: common in older patients; associated with breast implants
ALK negative
93
ack defining features that would place them in another category
Peripheral T Cell Lymphoma- Not Otherwise Specified (PTCL-NOS)
94
rearrangement of TCR disorder
post-thymic T cells
95
Peripheral T Cell Lymphoma- Not Otherwise Specified (PTCL-NOS): Immunophenotypically abnormal cells
T cell antigen mismatch with the absence of CD7 or CD5 as the most common finding
96
Treatmentfor PTCL-NOS
combination chemotherapy
97
cells reacting to the neoplasm predominates rather than the neoplastic cells;classification: Rye, Ann Arbor( mostly used- depends on histologic type and extent of tissue involvement, affects young and the elderly. 85% curable.
Hodgkin Lymphoma
98
Hallmark cell for Hodgkin Lymphoma
Reed -Sternberg cell
99
malignant lymphocytes arrested at certain stages of maturation, classified by Rappaport system: usually neoplasm of B cell, fatal.
Non-Hodgkin Lymphoma
100
Hodkin Disease persistent defect in the cellular immunity with abnormalities in
T lymphocytes
IL-2 production
increased sensitivity to suppressor monocytes
normal T suppressor cells.
101
a deviation from the diploid number of chromosomes, resulting from the gain or loss of chromosomes or from polyploid cell is a characteristic feature of Hodgkin disease.
Aneuploidy
102
a recurring numerical abnormality in aneuploidy
chromosomes 1, 2, 5, 12, and 21
103
Most frequent type of NHL is
diffuse large B-cell lymphoma.
104
that expresses high levels of genes characteristic of germinal center, B-cell–like lymal germinal center B cells
Germinal center, B-cell–like lymphoma
105
which expresses genes characteristic of mitogenically activated blood B cells
Activated B-cell–like lymphoma
106
has a heterogeneous gene expression that suggests it includes more than one subtype of lymphoma
type 3 diffuse large B-cell lymphoma
107
Leukemic phase of cutaneous T-cell lymphoma, mycosis fungoides. Affects older males, usually.
Sezary Syndrome
108
Sezary Syndrome affects the skin and involves primarily
T lymphoctes
109
Sezary Syndrome is positive for
CD2, CD3, CD4 antigens
110
tumor cell, is typically the size of a small/ medium/large lymphocyte and has a dark-staining, clumped, nuclear chromatin pattern. The convoluted( cerebriform) nucleus, resembling a monocyte nucleus
Sézary cell
111
Treatment for sezary syndrome
steroid, nitrogen mustard, or phototherapy. Advanced stage is incurable
112
derived from mature or post-thymic T cells.
Mature T-Cell & NK-Cell Neoplasms
113
found often in children in Africa & New Guinea, affects jaw and facial bones. EBV play a role in transforming the B lymphocyte by binding to surface receptors
Burkitt lymphoma
114
found often in children in Africa & New Guinea, affects jaw and facial bones. EBV play a role in transforming the B lymphocyte by binding to surface receptors
Burkitt lymphoma
115
Receptor for EBV
CD21
116
dark blue cytoplasm, with multiple vacuoles creating a starry sky pattern
Burkitt cell
117
an MPN arising from a single genetic translocation in a pluripotential HSC( hemocytoblast) producing a clonal overproduction of the myeloid cell line.
CHRONIC MYELOID LEUKEMIA
118
The clonal origin of HSC in CML was verified in studies of female heterozygous for
G-6PD
119
20% of all cases of leukemia is represented by
slightly common in men than in women
CHRONIC MYELOID LEUKEMIA
120
a tyrosine kinase inhibitor that has changed prognosis and treatment for CML
Imatinib
121
mediators of the signaling cascade,determining key roles in diverse biological processes like growth, differentiation, metabolism and apoptosis in response to external and internal stimuli
Tyrosine kinase
122
present in proliferating HSCs and their progeny in CML and must be identified to confirm the diagnosis.
Ph chromosome
123
discovered that Ph is a reciprocal translocation between the long arms of chromosomes 9 and 22.
1973 Rowley
124
ABL1 proto-oncogene
band q34 of chromosome 9 to the breakpoint cluster region(BCR) of band q11 of chromosome 22
125
Unique chimeric gene
BCR-ABL1
126
Found in the Ph chromosome, and also identified with Ph chromosome-positive ALL.
BCR-ABL1 fusion gene
127
transcribes and translates to a p185/p190 protein is present in 50% of Ph chromosome positive
minor chimeric BCR-ABL1 gene
128
Lab findings in CML
Hyperuricemia
Uricosuria
TL of greater than 300,000/uL( in 15%)
Ph chromosome (cytogenetic analysis)
BCR-ABL1 fusion gene using fluorescence in situ hybridization, and /or detecting BCR-ABL1 fusion transcript by qualitative reverse transcriptase PCR
Initial test: LAP/NAP, where result is lower than normal or reference range.
129
Several diseases appear clinically similar to CML but do not exhibit the Ph chromosome and express only
pseudo-Gaucher cells.
130
a MPN that manifests with blood, BM, extramedullary infiltrative patterns similar to those of CML, except that only neutrophilic granulocytes are present and fewer than 10% of blood neutrophils are immature.
Chronic Neutrophilic Leukemia ( CNL) i
131
involves a comparable expansion of monocytes, including functional monocytes
Chronic Monocytic leukemia
132
classified by WHO as myelodysplastic/ myeloproliferative diseases because of the overlap in clinical, laboratory, or morphologic findings.
Juvenile Myelomonocytic Leukemia and Adult Chronic Myelomonocytic leukemia-
133
These work by inhibiting transcription of DNA to RNA by alkylation
Alkylating agents
134
combination with 6-thioguanine was used to reduce tumor burden.
busulfan
135
improve patient survival treatment for CML
Hydroxyurea and 6-mercaptopurine
136
suppression of Ph chromosome thus reducing the rate of cellular progression to blast cells, and increase the long-term patient survival especially when combined cytarabine
Interferon-alpha
137
autologous/ allogeneic HSCs- curative, esp. in patients below 55 y/o.
BM and stem cell transplantation
138
followed by transplantation of mobilized normal progenitor cells that exhibit CD34+ surface markers.
Ablative chemotherapy
139
bind the abnormal BCR-ABL1 protein, blocking the constitutive tyrosine kinase activity and reducing signal transduction activation
Use of synthetic proteins
140
MDS was referred to as
refractory anemia, smoldering anemia,oligoblastic leukemia, or preleukemias.
141
Cooperative Leukemia Study Group proposed terminology and a specific set of morphologic criteria to describe what are known as myelodysplastic syndromes(MDS).
1982- FAB
142
a new classification that included molecular, cytogenetic, and immunologic criteria in addition to morphologic features.
1997- WHO
143
group of acquired clonal hematologic disorders characterized by progressive cytopenias in the peripheral blood, reflecting defects in erythroid, myeloid, and /or megakaryocytic maturation.
MDS
144
Median age of diagnosing MDS
76 y/o
145
precursor state for many hematologic disorders, including MDS. 10% of patients older than 65, and about 20% older than age 90 have CHIP.
Clonal hematopoiesis of indeterminate potential (CHIP)
146
interact with MDS
Somatic mutations
Epigenetic modifications
BM microenvironment
Environmental stimuli whether CHIP develops into MDS/ any hematologic disease
Severity of the subsequent disorder
Risk for transformation into AML
147
MDS types of mutation
de novo( primary MDS no known cause
therapy-related MDS(secondary to exposure to chemicals or radiation( not associated with prior disease treatment)
inherited- familial tendency / familial predispotion
148
2 morphologic findings common to all types of MDS
presence of progressive cytopenias despite cellular bone marrow( normal rate of production of cells)-NV 25-75%.
dyspoiesis in one or more cell lines
149
may be responsible of ineffective hematopoiesis in MDS. Increased in early stage of the disease, when blood cytopenias are evident
Disruption of apoptosis
150
most common is the presence of oval macrocytes,macro-ovalocyes especially when these cells are seen in normal Vit B12, and folate values.
DISERYTHROPOIESIS
151
most common is the presence of oval macrocytes,macro-ovalocyes especially when these cells are seen in normal Vit B12, and folate values.
DISERYTHROPOIESIS
152
When progression to leukemia is apparent, apoptosis is
decreased
increased neoplastic cell survival and expansion of the abnormal clone
153
DISERYTHROPOIESIS Blood: most common is the presence of
oval macrocytes,macro-ovalocyes especially
154
Hypochromic microcytes in the presence of adequate iron stores
central pallor more than 1/3
155
Dimorphic red blood cell (RBC) population
hypochromic cells and normochromic cells.
156
DNA particles, sign of impaired erythropoiesis
Howell –jolly bodies
157
DNA particles, sign of impaired erythropoiesis
Howell –jolly bodies
158
-red cell with siderotic granules- iron granules stained by Perl’s or Prussian Blue
siderocytes
159
RBC precursors with more than 1 nucleus or abnormal nuclear shapes
BM
160
hallmark cell of MDS
Ring sideroblast
161
Megaloblastoid cellular development in the presence of n ----- values
ormal Vit. B12 and folate/ folic acid
162
BM may exhibit
hypoplasia or hyperplasia
163
is a common finding in dysplastic BM.
Monocytic hyperplasia
164
Morphologic Evidences of Dysmegakaryopoiesis:
Giant platelets-macrothrombocytes
Platelets with abnormal granulation-hypogranular or agranular Circulating micromegakaryocytes
Large mononuclear megakaryocytes
Micromegakaryocytes or micromegakaryoblasts or both
Abnormal nuclear shapes in the megakaryocytes/ megakaryoblasts
165
not sufficient evidence for MDS, because several other conditions can cause similar morphologic features
Dysplasia
166
cause pancytopenia and dysplasia
vitamin B12 or folate(folic acid) deficiency- can cause pancytopenia and dysplasia
Exposure to heavy metals- copper deficiency may cause reversible myelodysplasia
Hematologic disorders like Fanconi anemia, Congenital dyserythropoietic anemia
Parvovirus B19
Some chemotherapeutic agents
PNH- paroxysmal nocturnal hemoglobinuria-decrease in CD55 and CD56= DAF, acquired condition
HIV/AIDS
The need for thorough history and physical examination is imperative.
167
Refractory cytopenia with unilineage dysplasia to MDS with single lineage dysplasia (MDS-SLD)
MDS WITH SINGLE LINEAGE DYSPLASIA
168
disease myoblasts do not have auer rods. If auer rods are noted, the disorder is classified as MDS with excess blasts-2. Median survival is about 31-38 months, with a 10%-12% risk of transformation to AML within 5 years.
MDS WITH MULTILINEAGE DYSPLASIA (MDS-MLD)
169
an erythroid precursor with at least 5 iron granules per cell,
DISERYTHROPOIESIS
170
MDS WITH RING SIDEROBLASTS
mutation in the spliceosome gene SF3B1.
171
accounts for 3%-10% of all MDS cases and has a median age of presentation of 71.
MDS-RS with single lineage dysplasia-
172
accounts for 3%-10% of all MDS cases and has a median age of presentation of 71.
MDS-RS with single lineage dysplasia-
173
1 or more cytopenias and dysplasia in 2 or more myeloid cell lines. This has a worse prognosis than MDS-RS with single lineage dysplasia.
MDS-RS with multilineage dysplasia-
174
1 or more cytopenias and dysplasia in 2 or more myeloid cell lines. This has a worse prognosis than MDS-RS with single lineage dysplasia.
MDS-RS with multilineage dysplasia-
175
Ttrilineage cytopenias
Significant dysmyelopoiesis, dysmegakaryopoiesis, or both, are common in MDS-EB.
MDS WITH EXCESS BLASTS
176
Trilineage cytopenias
Significant dysmyelopoiesis, dysmegakaryopoiesis, or both, are common in MDS-EB.
MDS WITH EXCESS BLASTS charactarestcs
177
the only WHO recognized MDS with a defining cytogenetic abnormality.
MDS WITH ISOLATED DEL deletion of 5q (5q-)
178
specific changes necessary for classification into other MDS subtypes.
MDS, UNCLASSIFIABLE (MDS-U)
179
Childhood MDS increased frequency of specific inherited gene mutations such as
RUNX1, SOS1, GATA2. ANKRD26 and others.
180
In Chronic Myelomonocytic Leukemia(CMML) there is absence of:
absence of BCR/ABL1 fusion genes
absence of rearrangements involving PDGFRA, PDGFB, FGFR1 OR PCM1-JAK2
181
characterized by leukocytosis with morphologically dysplastic neutrophils and their precursors. Basophilia may be present but is not a prominent feature. Multilineage dysplasia is common.
Atypical Chronic Myeloid Leukemia, BCR/ABL1 Negative
182
clonal disorder of granulocytes and monocyte cell lines. It affects 1 month to 14 years of age.
Juvenile Myelomonocytic Leukemia
183
The mutation is in SF3B1 and JAK2 V617F
MDS/MPN with Ring Sideroblasts and Thrombocytosis (MDS/MPN-RS-T)-
184
cases which meet the criteria for MDS/MPN but do not meet the aforementioned subcategories.
MDS/MPN, Unclassifiable
185
Cytogenetics
Except for del(5q) = (5q-) no cytogenetic abnormality is specific to a subtype of MDS
The most common abnormalities involve chromosome 5, 7, 8, 18, 20, and 13.
The most common single abnormalities besides del(5q) are trisomy 8 and monosomy 7, 12p-, iso 17, and loss of the Y chromosome.
186
Molecular Alterations
SF3B1, ASXL1, SRSF2,DNMT3A, and RUNX1.
187
confers facorable prognosis
SF3B1
188
negative prognosis and predicts higher risk of AML
TP53
189
3 different ways in which epigenetics may facilitate oncogenesis
methylation of CpG islands
histone modification
alteration of micro RNA expression
190
3 different ways in which epigenetics may facilitate oncogenesis
methylation of CpG islands
histone modification
alteration of micro RNA expression
191
Treatment for the low risk groups( to overcome deficient BM production)
Supportive therapy
192
Examples of support therapy
blood transfusion
erythroid stimulating agents- EPO
thrombopoietin- TPO
G-CSF
prophylactic antibiotics
iron chelation
193
thymocyte globulin
cyclosporine
anti- thymocyte globulin
They decrease risk of leukemic transformation to AML
Immunosuppressive therapy
194
50% of cases has remission.
Lenalidomide
195
patients with IPSS score of intermediate 2 or higher and patient with 10% blasts. Successful in patients below 70 y/o and without any comorbidity
HSC transplantation-
196
patients with IPSS score of intermediate 2 or higher and patient with 10% blasts. Successful in patients below 70 y/o and without any comorbidity
HSC transplantation-
197
During the second trimester of fetal development, the primary site of blood cell production is the
Liver
198
organs is responsible for the maturation of T lymphocytes and regulation of their expression of CD4 and CD8?
Thymus
199
The best source of active bone marrow from a 20 year old would be:
Iliac Crest
200
Physiologic programmed cell death is termed:
Apoptosis
201
Which organ is the site of sequestration of platelets?
sheep
202
Which organ is the site of sequestration of platelet
thymus
203
Which one of the following morphologic changes occurs during normal blood cell maturation?
Condensation of nuclear chromatin
204
cells is a product of the common lymphoid progenitor
T Lymphocyte
205
growth factor is produced in the kidneys and is used to treat anemia associated with kidney disease?
EPO
206
Which one of the following cytokines is required very early in the differentiation of a hematopoietic stem cell
FLT3 ligand
207
When a patient has severe anemia and the bone marrow is unable to effectively produce red blood cells to meet the increased demand, one of the body’s responses i
Extramedullary hematopoiesis in the liver and spleen
208
MDS are most common in which age group?
Older than 50 years
209
What is a major indication of MDS in the peripheral blood and bone marrow?
Dyspoiesis
210
An alert hematologist should recognize all of the following peripheral blood abnormalities as diagnostic clues in MDS EXCEPT:
Target cells
211
For an erythroid precursor to be considered a ring sideroblast, the iron-laden mitochondria must encircle how much of the nucleus?
1/3
212
According to the WHO classification of MDS, what percentage of blasts would constitute transformation to an acute leukemia?
20%
213
A patient has anemia, oval macrocytes, and hypersegmented neutrophils. Which of the following tests would be most efficient in differential diagnosis of this disorder?
Vitamin B12 and folate levels
214
A 60-year-old woman comes to the physician with fatigue and malaise. Her hemoglobin is 8 g/dL, hematocrit is 25%, RBC count is 2.00 3 1012/L, platelet count is 550 3 109 /L, and WBC count is 3.8 3 109 /L. Her WBC differential is unremarkable. Bone marrow shows erythroid hypoplasia and hypolobulated megakaryocytes; granulopoiesis appears normal. Ring sideroblasts are rare. Chromosome analysis reveals the deletion of 5q only. Based on the classification of this disorder, what therapy would be most appropriate?
Supportive therapy; lenalidomide if the disease progresses
215
Which of the following is LEAST likely to contribute to the death of patients with MDS?
Neuropathy
216
Into what other hematologic disease does MDS often convert?
SAML
217
Chronic myelomonocytic leukemia is classified in the WHO system as
MDS/MPN
218
Non-Hodgkin lymphoma can be best differentiated from reactive disorders by:
Blood film review
219
Which laboratory test is most suggestive of autoimmune hemolytic anemia in a patient with CLL?
Lymphocyte count
220
What is the best test or method for determining if a clonal population of T cells is present in a specimen?
Karyotyping
221
A rise in the lymphocyte count from 4.1 3 109 /L to 5.5 3 109 /L in a patient with monoclonal B lymphocytosis suggests:
A reactive condition
Lymph node biopsy
222
If not treated, which of the following would generally be associated with the best outcome?
Burkitt lymphoma
223
What do CLL and myeloma have in common?
Light chain restriction
224
In Hodgkin lymphoma the Reed-Sternberg cell and _________ are malignant.
popcorn cells
225
In most cases the diagnosis of lymphoma relies on all of the following except:
Molecular analysis
226
Which of the following is present in monoclonal gammopathy of underdetermined significance?
Hypercalcemia
227
Lymphomas differ from leukemias in that they are
Solid tumors
228
Which one of the following viruses is known to cause lymphoid neoplasms in humans
HTLV-1
229
. Loss-of-function of tumor suppressor genes increase the risk of hematologic neoplasms by:
Allowing cells with damaged DNA to progress through the cell cycle
230
Oncogenes are said to act in a dominant fashion because:
A mutation in only one allele is sufficient to promote a malignant phenotype
231
Which one of the following is NOT one of the cellular abnormalities produced by oncogenes
Acceleration of DNA catabolism
232
Example of tumor suppresor gene
TP53
233
Treatment using G-CSF during leukemia
Reduces Risk of Infection
234
Imatinib is an example of what type of leukemia treatment
Targeted therapy
235
Which one of the following is FALSE about epigenetic mechanisms
Hypermethylation of CpG islands in gene promoters result in their overactivation
236
True or False: Spleen is a hematopoetic organ
Flase - lymphoid organ
237
A 20-year-old patient has an elevated WBC count with 70% blasts, 4% neutrophils, 5% lymphocytes, and 21% mono cytes in the peripheral blood. Eosinophils with dysplastic changes are seen in the bone marrow. AML with which of the following karyotypes would be most likely to be seen?
AML with t(16;16)(p13;q22)
238
Which of the following would be considered a sign of poten tially favorable prognosis in children with ALL?
Hyperdiploidy
239
Signs and symptoms of cerebral infiltration with blasts are more commonly seen in
ALL
240
An oncology patient exhibiting signs of renal failure with seizures after initial chemotherapy may potentially develop:
Tumor lysis syndrome
241
. Disseminated intravascular coagulation is more often seen in association with leukemia characterized by which of the following mutations?
. t(15;17)(q22;q12)
242
Which of the following leukemias affects primarily children, is characterized by an increase in monoblasts and monocytes, and often is associated with gingival and skin involvement?
AML with t(9;11)(p22;q23)
243
. A 20-year-old patient presents with fatigue, pallor, easy bruising, and swollen gums. Bone marrow examination reveals 82% cells with delicate chromatin and prominent nucleoli that are CD141, CD41, CD11b1, and CD361. Which of the following acute leukemias is likely
Acute monoblastic/monocytic leukemia
244
Pure erythroid leukemia is a disorder involving
Pronormoblasts and basophilic normoblasts
245
A patient with normal chromosomes has a WBC count of 3.0 3 10^9/L and dysplasia in all cell lines. There are 60% blasts of varying sizes. The blasts stain positive for CD61. The most likely type of leukemia is
Acute megakaryoblastic
246
SBB stains which of the following component of cells
Lipids
247
The cytochemical stain a-naphthyl butyrate is a nonspecific esterase stain that shows diffuse positivity in cells of which lineage
monocytic
248
A peripheral blood film that shows increased neutrophils, basophils, eosinophils, and platelets is highly suggestive of:
CML
249
CML gene present
. t(9;22)
250
A patient in whom CML has previously been diagnosed has circulating blasts and promyelocytes that total 30% of leuko cytes. The disease is considered to be in what phase?
. Transformation to acute leukemia
251
The most common mutation found in patients with primary PV is
JAK2 V617F
252
The peripheral blood in PV typically manifests:
. Erythrocytosis, thrombocytosis, and granulocytosis
253
A patient has a platelet count of 700 x 10^9 /L with abnor malities in the size, shape, and granularity of platelets; a WBC count of 12 x 10^9 /L; and hemoglobin of 11 g/dL. The Philadelphia chromosome is not present. The most likely diagnosis is:
ET
254
Complications of ET include all of the following excep
Infections
255
Which of the following patterns is characteristic of the peripheral blood in patients with PMF?
. Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes
256
The myelofibrosis associated with PMF is a result of:
Enhanced activity of fibroblasts as a result of increased stimulatory cytokines
