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Hema Lec 2 > Hematologic Malignancies > Flashcards

Hematologic Malignancies Flashcards

1
Q

neoplasm of lymphoblasts committed to the B-cell lineage.

A

precursor B-cell ALL

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2
Q

neoplasm of lymphoblasts committed to the T-cell lineage.

A

Precursor T lymphoblastic leukemia/lymphoblastic
lymphoma (precursor T-cell ALL)

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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)

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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)

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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)

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6
Q

Lymphadenopathy and hepatomegaly are present in %

A

75%

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7
Q

ALL: LABORATORY DATA
Total leukocyte count

A

elevated in 60% to 70% of
patients; ranging from 50 to 100 × 10
9/L.

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8
Q

ALL: LABORATORY DATA
Approx. 25% of patients exhibits

A

Leukocytopenia

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9
Q

ALL: LABORATORY DATA

predominance of _____

A

Blast Cells

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10
Q

ALL: LABORATORY DATA

close to lymphoblasts, lymphocytes & smudge cells

A

100%

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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

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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

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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

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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

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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

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16
Q

median age of onset of CHRONIC LYMPHOCYTIC LEUKEMIA / SMALL LYMPHOCYTIC LYMPHOMA

A

65 years

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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

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18
Q

Characterizarion of B-CLL

A

CD5+
CD19+
CD23+ monoclonal B cells

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19
Q

most consistent finding which is present in approximately 50% of patients in chromosomal alteration

A

trisomy of chromosome 12

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20
Q

associated with B-CLL
chromosomal alteration

A

translocation of chromosomes 8 and 14

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21
Q

Chromosomal alterations:
translocation of chromosomes 9 and 22

A

Ph observed in non-T and non-B types

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22
Q

B-cells display the classic surface
immunoglobulin (SIg) markers.

A

CD19
CD20
CD24
CD5

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23
Q

CHRONIC LYMPHOCYTIC LEUKEMIA /
SMALL LYMPHOCYTIC LYMPHOMA
also frequently present.

A

Hepatosplenomegaly

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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)

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25
Q

appearance of fine, hair-like, irregular cytoplasmic projections that are characteristic of lymphocytes in this disease

A

Hairy cell leukemia

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26
Q

Isoenzyme 5 is a —- present in large amounts in the hairy cells of HCL

A

pyrophosphatase

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27
Q

—– may show less than complete
resistance to L(+) tartaric acid and stain faintly positive

A

atypical lymphocytes of infectious mononucleosis, CLL and lymphosarcoma

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28
Q

HAIRY CELL LEUKEMIA

The cytochemical features of HCL include a
strong acid phosphatase reaction that is not
inhibited by

A

tartaric acid or tartrate-resistant acid
phosphatase (TRAP) stain.

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29
Q

Isoenzyme —- is present
in HCL

A

5

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30
Q

Immunological Markers of HCL (typical type)

A

CD19+
CD20+
CD22+
CD24+
CD25+

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31
Q

display strong surface immunoglobulin (SIg).

A

HAIRY CELL LEUKEMIA

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32
Q

Chromosomal alterations in CHRONIC LYMPHOCYTIC LEUKEMIA / SMALL LYMPHOCYTIC LYMPHOMA

A

13q deletion
11q deletion
trisomy of chromosome 12
17p deletion
translocation of chromosomes 8 and 14
translocation of chromosomes 9 and 22( Ph)

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33
Q

Clinical Signs & Symptoms for Chronic Lymphocytic Leukemia

A

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.

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34
Q

Serum electrophoresis of Chronic Lymphocytic Leukemia shows:

A

hypogammaglobulinemia

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35
Q

more aggresive type of HCL

A

HAIRY CELL LEUKEMIA VARIANT

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36
Q

Result in differential diagnosis of vHCL from typical HCL is cytochemical staining (TRAP).

A

vHCL: negative
HCL : positive

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37
Q

Poor prognosis in immunophenotyping using flow cytometry

A

vHCL

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38
Q

presently considered as a type of chronic leukemia. The cells are also B lymphocytes, just like the classic HCL.

A

Hairy Cell Leukemia Variant

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39
Q

very rare form of HCL-V that shows a slightly more prominent nucleoli than the typical HCL.

A

Japanese Variant

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40
Q

used to differentiate similar morphology in japanese variant of HCL

A

polyclonal B cell lymphocytosis

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41
Q

PROLYMPHOCYTIC LEUKEMIA is a malignancy of B prolymphocytes affecting ____________

A

blood
bone marrow
spleen

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42
Q

Characterized by a large number of small lymphocytes with scant cytoplasm and the immature features of prolymphocytes in the peripheral blood.

A

PROLYMPHOCYTIC LEUKEMIA

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43
Q

Prolymphocytes must exceed—— of lymphoid cells in the peripheral blood.

A

55%

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44
Q

Immunological Markers of prolymphocytic leukemia

A

CD19+
CD20+
CD24+
CD22+
Cells display strong SIg.

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45
Q

a malignant bone marrow–based, plasma cell neoplasm associated with abnormal protein production.

A

MULTIPLE MYELOMA (PLASMA CELL MYELOMA)

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46
Q

a malignant bone marrow–based, plasma cell neoplasm associated with abnormal protein production.

A

MULTIPLE MYELOMA (PLASMA CELL MYELOMA)

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47
Q

an increased number of plasma cells in the peripheral blood and should be considered a form of multiple myeloma and not a separate entity.

A

Plasma cell leukemia

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48
Q

Multiple myeloma sually evolves from an asymptomatic premalignant stage of clonal plasma cell proliferation called

A

monoclonal gammopathy of undetermined significance (MGUS)

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49
Q

Age onset of multiple myeloma

A

between 40 and 70 years
peak incidence in the seventh decade of life.

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50
Q

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.

A

Multiple myeloma

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51
Q

compensatory decrease in synthesis and increase in catabolism of normal immunoglobulins.

A

Multiple myeloma

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52
Q

may develop as a result of bone marrow failure in multiple myeloma

A

granulocytopenia

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53
Q

Electrophoresis of serum in multiple myeloma usually demonstrates the overproduction of

A

IgM 19Sveedbeg units

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54
Q

Protein seen in patients with multiple myeloma

A

Bence- Jones protein.

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55
Q

a B-cell neoplasm characterized by lymphoplasmo-proliferative disorder with infiltration of the bone marrow and a monoclonal immunoglobulin M (IgM) protein

A

WALDENSTRÖM PRIMARY MACROGLOBULINEMIA

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56
Q

increased viscosity which requires plasmapheresis to alleviate symptoms

A

Waldenstrom macroglobulinemia

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57
Q

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.

A

Factor 88 or MYD88 gene

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58
Q

Heavy Chain Diseases is a rare syndromes characterized by the production of the

A

gamma, alpha, mu heavy chains of immunoglobulin and soft tissue tumors

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59
Q

Lymphoproliferative disorders associated with ____ chain

A

gamma

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60
Q

Lymphoproliferative disorders often resembles

A

PLL or plasmacytoma

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61
Q

may resemble CLL

A

mu heavy chain disease

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62
Q

associated with MALT lymphoma.

A

alpha chain disease

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63
Q

a LPN characterized by secretion of a truncated gamma chain without light chain binding sites

A

Gamma heavy chain disease

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64
Q

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.

A

Alpha heavy chain disease

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65
Q

usually with CLL, with vacuolated plasma cells in the BM

A

u heavy chain disease

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66
Q

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

A

LYMPHOMAS

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67
Q

post-thymic neoplastic disorder of T cells associated with retroviral infection by the human T lymphotropic virus type 1( HTLV-1).

A

Adult T Cell Leukemia/ Lymphoma ( ATLL)

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68
Q

Human T lymphotropic virus type 1 (HTLV-1) Is transmitted via

A

placental circulation, breastfeeding, blood transfusion, or sexual contact.

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69
Q

medium to large sized lymphocytes, have accentuated, convoluted nuclei, coarsely clumped chromatin, and deeply basophilic cytoplasm. “ Flower cell” is coined for this morphology.

A

ATLL cells

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70
Q

ATLL immunophenotype is generally consistent with

A

T helper cells:
CD3 and CD4 are expressed
CD25 and CCR4 are highly expressed
CD7 and CD8 are absen

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71
Q

Indication of prognostic significance in ATLL can be used as a tumor marker for assessing disease status.

A

soluble form of IL-2

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72
Q

Treatment for HTLV-1 virus involves the combination of

A

interferon alpha and azidothymidine

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73
Q

Anti- CCR4 monoclonal antibody

A

mogamulizumab

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74
Q

reported to effect long-term survival.

A

Allogeneic stem cell transplantation

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75
Q

Aggressive form of cancer of mature B cells

A

Burkitt Lymphoma/ Leukemia (BL)

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76
Q

3 types of Burkitt Lymphoma

A

endemic
sporadic
HIV associated

Responsive to chemotherapy

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77
Q

mostly patients are asymptomatic. A separate scoring system, Fabry International Prognostic Index (FIBI) has been developed to aid in decision –making.

A

Follicular Lymphoma

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78
Q

Treatment for follicular lymphoma

A

combination chemotherapy like that used in other forms of NHL

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79
Q

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.

A

Mantle cell lymphoma (MCL)

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80
Q

the most common form of NHL, 25%-30% belong to this type.

A

Diffuse Large B cell Lymphoma (DLBCL)

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81
Q

large with a diffuse pattern in the lymph node.

A

DLBCL cells

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82
Q

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.

A

immunochemistry and genetic testing

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83
Q

Translocation involving the BCL6 gene —– of patients

A

30%

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84
Q

gene, a marker associated with t(14;18) and FL occurs in 20%to 30% of DLBCL and may complicate dx.

A

Translocation of the BCL2

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85
Q

typically associated with Burkitt leukemia/ lymphoma is rearranged in 10% of DLBCL patients.

A

MYC gene

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86
Q

standard chemotherapy has improved patient outcomes.

A

anti-CD20 monoclonal antibody and rituximab

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87
Q

indolent B cell lymphoma associated with chronic antigen stimulation either in the setting of infection or autoimmunity

A

Marginal Zone Lymphoma

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88
Q

3 subtypes of Marginal Zone Lymphoma

A

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

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89
Q

T cell disorder associated with large pleomorphic cells, some small and others are medium sized.

A

Anaplastic Large Cell Lymphoma (ALCL)

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90
Q

ALCL expresses

A

CD30

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91
Q

ALCL: common in younger population, better prognosis

A

ALK Positive

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92
Q

ALCL: common in older patients; associated with breast implants

A

ALK negative

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93
Q

ack defining features that would place them in another category

A

Peripheral T Cell Lymphoma- Not Otherwise Specified (PTCL-NOS)

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94
Q

rearrangement of TCR disorder

A

post-thymic T cells

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95
Q

Peripheral T Cell Lymphoma- Not Otherwise Specified (PTCL-NOS): Immunophenotypically abnormal cells

A

T cell antigen mismatch with the absence of CD7 or CD5 as the most common finding

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96
Q

Treatmentfor PTCL-NOS

A

combination chemotherapy

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97
Q

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.

A

Hodgkin Lymphoma

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98
Q

Hallmark cell for Hodgkin Lymphoma

A

Reed -Sternberg cell

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99
Q

malignant lymphocytes arrested at certain stages of maturation, classified by Rappaport system: usually neoplasm of B cell, fatal.

A

Non-Hodgkin Lymphoma

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100
Q

Hodkin Disease persistent defect in the cellular immunity with abnormalities in

A

T lymphocytes
IL-2 production
increased sensitivity to suppressor monocytes
normal T suppressor cells.

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101
Q

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.

A

Aneuploidy

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102
Q

a recurring numerical abnormality in aneuploidy

A

chromosomes 1, 2, 5, 12, and 21

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103
Q

Most frequent type of NHL is

A

diffuse large B-cell lymphoma.

104
Q

that expresses high levels of genes characteristic of germinal center, B-cell–like lymal germinal center B cells

A

Germinal center, B-cell–like lymphoma

105
Q

which expresses genes characteristic of mitogenically activated blood B cells

A

Activated B-cell–like lymphoma

106
Q

has a heterogeneous gene expression that suggests it includes more than one subtype of lymphoma

A

type 3 diffuse large B-cell lymphoma

107
Q

Leukemic phase of cutaneous T-cell lymphoma, mycosis fungoides. Affects older males, usually.

A

Sezary Syndrome

108
Q

Sezary Syndrome affects the skin and involves primarily

A

T lymphoctes

109
Q

Sezary Syndrome is positive for

A

CD2, CD3, CD4 antigens

110
Q

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

A

Sézary cell

111
Q

Treatment for sezary syndrome

A

steroid, nitrogen mustard, or phototherapy. Advanced stage is incurable

112
Q

derived from mature or post-thymic T cells.

A

Mature T-Cell & NK-Cell Neoplasms

113
Q

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

A

Burkitt lymphoma

114
Q

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

A

Burkitt lymphoma

115
Q

Receptor for EBV

A

CD21

116
Q

dark blue cytoplasm, with multiple vacuoles creating a starry sky pattern

A

Burkitt cell

117
Q

an MPN arising from a single genetic translocation in a pluripotential HSC( hemocytoblast) producing a clonal overproduction of the myeloid cell line.

A

CHRONIC MYELOID LEUKEMIA

118
Q

The clonal origin of HSC in CML was verified in studies of female heterozygous for

A

G-6PD

119
Q

20% of all cases of leukemia is represented by

slightly common in men than in women

A

CHRONIC MYELOID LEUKEMIA

120
Q

a tyrosine kinase inhibitor that has changed prognosis and treatment for CML

A

Imatinib

121
Q

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

A

Tyrosine kinase

122
Q

present in proliferating HSCs and their progeny in CML and must be identified to confirm the diagnosis.

A

Ph chromosome

123
Q

discovered that Ph is a reciprocal translocation between the long arms of chromosomes 9 and 22.

A

1973 Rowley

124
Q

ABL1 proto-oncogene

A

band q34 of chromosome 9 to the breakpoint cluster region(BCR) of band q11 of chromosome 22

125
Q

Unique chimeric gene

A

BCR-ABL1

126
Q

Found in the Ph chromosome, and also identified with Ph chromosome-positive ALL.

A

BCR-ABL1 fusion gene

127
Q

transcribes and translates to a p185/p190 protein is present in 50% of Ph chromosome positive

A

minor chimeric BCR-ABL1 gene

128
Q

Lab findings in CML

A

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
Q

Several diseases appear clinically similar to CML but do not exhibit the Ph chromosome and express only

A

pseudo-Gaucher cells.

130
Q

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.

A

Chronic Neutrophilic Leukemia ( CNL) i

131
Q

involves a comparable expansion of monocytes, including functional monocytes

A

Chronic Monocytic leukemia

132
Q

classified by WHO as myelodysplastic/ myeloproliferative diseases because of the overlap in clinical, laboratory, or morphologic findings.

A

Juvenile Myelomonocytic Leukemia and Adult Chronic Myelomonocytic leukemia-

133
Q

These work by inhibiting transcription of DNA to RNA by alkylation

A

Alkylating agents

134
Q

combination with 6-thioguanine was used to reduce tumor burden.

A

busulfan

135
Q

improve patient survival treatment for CML

A

Hydroxyurea and 6-mercaptopurine

136
Q

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

A

Interferon-alpha

137
Q

autologous/ allogeneic HSCs- curative, esp. in patients below 55 y/o.

A

BM and stem cell transplantation

138
Q

followed by transplantation of mobilized normal progenitor cells that exhibit CD34+ surface markers.

A

Ablative chemotherapy

139
Q

bind the abnormal BCR-ABL1 protein, blocking the constitutive tyrosine kinase activity and reducing signal transduction activation

A

Use of synthetic proteins

140
Q

MDS was referred to as

A

refractory anemia, smoldering anemia,oligoblastic leukemia, or preleukemias.

141
Q

Cooperative Leukemia Study Group proposed terminology and a specific set of morphologic criteria to describe what are known as myelodysplastic syndromes(MDS).

A

1982- FAB

142
Q

a new classification that included molecular, cytogenetic, and immunologic criteria in addition to morphologic features.

A

1997- WHO

143
Q

group of acquired clonal hematologic disorders characterized by progressive cytopenias in the peripheral blood, reflecting defects in erythroid, myeloid, and /or megakaryocytic maturation.

A

MDS

144
Q

Median age of diagnosing MDS

A

76 y/o

145
Q

precursor state for many hematologic disorders, including MDS. 10% of patients older than 65, and about 20% older than age 90 have CHIP.

A

Clonal hematopoiesis of indeterminate potential (CHIP)

146
Q

interact with MDS

A

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
Q

MDS types of mutation

A

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
Q

2 morphologic findings common to all types of MDS

A

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
Q

may be responsible of ineffective hematopoiesis in MDS. Increased in early stage of the disease, when blood cytopenias are evident

A

Disruption of apoptosis

150
Q

most common is the presence of oval macrocytes,macro-ovalocyes especially when these cells are seen in normal Vit B12, and folate values.

A

DISERYTHROPOIESIS

151
Q

most common is the presence of oval macrocytes,macro-ovalocyes especially when these cells are seen in normal Vit B12, and folate values.

A

DISERYTHROPOIESIS

152
Q

When progression to leukemia is apparent, apoptosis is

A

decreased
increased neoplastic cell survival and expansion of the abnormal clone

153
Q

DISERYTHROPOIESIS Blood: most common is the presence of

A

oval macrocytes,macro-ovalocyes especially

154
Q

Hypochromic microcytes in the presence of adequate iron stores

A

central pallor more than 1/3

155
Q

Dimorphic red blood cell (RBC) population

A

hypochromic cells and normochromic cells.

156
Q

DNA particles, sign of impaired erythropoiesis

A

Howell –jolly bodies

157
Q

DNA particles, sign of impaired erythropoiesis

A

Howell –jolly bodies

158
Q

-red cell with siderotic granules- iron granules stained by Perl’s or Prussian Blue

A

siderocytes

159
Q

RBC precursors with more than 1 nucleus or abnormal nuclear shapes

A

BM

160
Q

hallmark cell of MDS

A

Ring sideroblast

161
Q

Megaloblastoid cellular development in the presence of n —– values

A

ormal Vit. B12 and folate/ folic acid

162
Q

BM may exhibit

A

hypoplasia or hyperplasia

163
Q

is a common finding in dysplastic BM.

A

Monocytic hyperplasia

164
Q

Morphologic Evidences of Dysmegakaryopoiesis:

A

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
Q

not sufficient evidence for MDS, because several other conditions can cause similar morphologic features

A

Dysplasia

166
Q

cause pancytopenia and dysplasia

A

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
Q

Refractory cytopenia with unilineage dysplasia to MDS with single lineage dysplasia (MDS-SLD)

A

MDS WITH SINGLE LINEAGE DYSPLASIA

168
Q

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.

A

MDS WITH MULTILINEAGE DYSPLASIA (MDS-MLD)

169
Q

an erythroid precursor with at least 5 iron granules per cell,

A

DISERYTHROPOIESIS

170
Q

MDS WITH RING SIDEROBLASTS

A

mutation in the spliceosome gene SF3B1.

171
Q

accounts for 3%-10% of all MDS cases and has a median age of presentation of 71.

A

MDS-RS with single lineage dysplasia-

172
Q

accounts for 3%-10% of all MDS cases and has a median age of presentation of 71.

A

MDS-RS with single lineage dysplasia-

173
Q

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.

A

MDS-RS with multilineage dysplasia-

174
Q

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.

A

MDS-RS with multilineage dysplasia-

175
Q

Ttrilineage cytopenias
Significant dysmyelopoiesis, dysmegakaryopoiesis, or both, are common in MDS-EB.

A

MDS WITH EXCESS BLASTS

176
Q

Trilineage cytopenias
Significant dysmyelopoiesis, dysmegakaryopoiesis, or both, are common in MDS-EB.

A

MDS WITH EXCESS BLASTS charactarestcs

177
Q

the only WHO recognized MDS with a defining cytogenetic abnormality.

A

MDS WITH ISOLATED DEL deletion of 5q (5q-)

178
Q

specific changes necessary for classification into other MDS subtypes.

A

MDS, UNCLASSIFIABLE (MDS-U)

179
Q

Childhood MDS increased frequency of specific inherited gene mutations such as

A

RUNX1, SOS1, GATA2. ANKRD26 and others.

180
Q

In Chronic Myelomonocytic Leukemia(CMML) there is absence of:

A

absence of BCR/ABL1 fusion genes
absence of rearrangements involving PDGFRA, PDGFB, FGFR1 OR PCM1-JAK2

181
Q

characterized by leukocytosis with morphologically dysplastic neutrophils and their precursors. Basophilia may be present but is not a prominent feature. Multilineage dysplasia is common.

A

Atypical Chronic Myeloid Leukemia, BCR/ABL1 Negative

182
Q

clonal disorder of granulocytes and monocyte cell lines. It affects 1 month to 14 years of age.

A

Juvenile Myelomonocytic Leukemia

183
Q

The mutation is in SF3B1 and JAK2 V617F

A

MDS/MPN with Ring Sideroblasts and Thrombocytosis (MDS/MPN-RS-T)-

184
Q

cases which meet the criteria for MDS/MPN but do not meet the aforementioned subcategories.

A

MDS/MPN, Unclassifiable

185
Q

Cytogenetics

A

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
Q

Molecular Alterations

A

SF3B1, ASXL1, SRSF2,DNMT3A, and RUNX1.

187
Q

confers facorable prognosis

A

SF3B1

188
Q

negative prognosis and predicts higher risk of AML

A

TP53

189
Q

3 different ways in which epigenetics may facilitate oncogenesis

A

methylation of CpG islands
histone modification
alteration of micro RNA expression

190
Q

3 different ways in which epigenetics may facilitate oncogenesis

A

methylation of CpG islands
histone modification
alteration of micro RNA expression

191
Q

Treatment for the low risk groups( to overcome deficient BM production)

A

Supportive therapy

192
Q

Examples of support therapy

A

blood transfusion
erythroid stimulating agents- EPO
thrombopoietin- TPO
G-CSF
prophylactic antibiotics
iron chelation

193
Q

thymocyte globulin
cyclosporine
anti- thymocyte globulin
They decrease risk of leukemic transformation to AML

A

Immunosuppressive therapy

194
Q

50% of cases has remission.

A

Lenalidomide

195
Q

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

A

HSC transplantation-

196
Q

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

A

HSC transplantation-

197
Q

During the second trimester of fetal development, the primary site of blood cell production is the

A

Liver

198
Q

organs is responsible for the maturation of T lymphocytes and regulation of their expression of CD4 and CD8?

A

Thymus

199
Q

The best source of active bone marrow from a 20 year old would be:

A

Iliac Crest

200
Q

Physiologic programmed cell death is termed:

A

Apoptosis

201
Q

Which organ is the site of sequestration of platelets?

A

sheep

202
Q

Which organ is the site of sequestration of platelet

A

thymus

203
Q

Which one of the following morphologic changes occurs during normal blood cell maturation?

A

Condensation of nuclear chromatin

204
Q

cells is a product of the common lymphoid progenitor

A

T Lymphocyte

205
Q

growth factor is produced in the kidneys and is used to treat anemia associated with kidney disease?

A

EPO

206
Q

Which one of the following cytokines is required very early in the differentiation of a hematopoietic stem cell

A

FLT3 ligand

207
Q

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

A

Extramedullary hematopoiesis in the liver and spleen

208
Q

MDS are most common in which age group?

A

Older than 50 years

209
Q

What is a major indication of MDS in the peripheral blood and bone marrow?

A

Dyspoiesis

210
Q

An alert hematologist should recognize all of the following peripheral blood abnormalities as diagnostic clues in MDS EXCEPT:

A

Target cells

211
Q

For an erythroid precursor to be considered a ring sideroblast, the iron-laden mitochondria must encircle how much of the nucleus?

A

1/3

212
Q

According to the WHO classification of MDS, what percentage of blasts would constitute transformation to an acute leukemia?

A

20%

213
Q

A patient has anemia, oval macrocytes, and hypersegmented neutrophils. Which of the following tests would be most efficient in differential diagnosis of this disorder?

A

Vitamin B12 and folate levels

214
Q

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?

A

Supportive therapy; lenalidomide if the disease progresses

215
Q

Which of the following is LEAST likely to contribute to the death of patients with MDS?

A

Neuropathy

216
Q

Into what other hematologic disease does MDS often convert?

A

SAML

217
Q

Chronic myelomonocytic leukemia is classified in the WHO system as

A

MDS/MPN

218
Q

Non-Hodgkin lymphoma can be best differentiated from reactive disorders by:

A

Blood film review

219
Q

Which laboratory test is most suggestive of autoimmune hemolytic anemia in a patient with CLL?

A

Lymphocyte count

220
Q

What is the best test or method for determining if a clonal population of T cells is present in a specimen?

A

Karyotyping

221
Q

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

A reactive condition
Lymph node biopsy

222
Q

If not treated, which of the following would generally be associated with the best outcome?

A

Burkitt lymphoma

223
Q

What do CLL and myeloma have in common?

A

Light chain restriction

224
Q

In Hodgkin lymphoma the Reed-Sternberg cell and _________ are malignant.

A

popcorn cells

225
Q

In most cases the diagnosis of lymphoma relies on all of the following except:

A

Molecular analysis

226
Q

Which of the following is present in monoclonal gammopathy of underdetermined significance?

A

Hypercalcemia

227
Q

Lymphomas differ from leukemias in that they are

A

Solid tumors

228
Q

Which one of the following viruses is known to cause lymphoid neoplasms in humans

A

HTLV-1

229
Q

. Loss-of-function of tumor suppressor genes increase the risk of hematologic neoplasms by:

A

Allowing cells with damaged DNA to progress through the cell cycle

230
Q

Oncogenes are said to act in a dominant fashion because:

A

A mutation in only one allele is sufficient to promote a malignant phenotype

231
Q

Which one of the following is NOT one of the cellular abnormalities produced by oncogenes

A

Acceleration of DNA catabolism

232
Q

Example of tumor suppresor gene

A

TP53

233
Q

Treatment using G-CSF during leukemia

A

Reduces Risk of Infection

234
Q

Imatinib is an example of what type of leukemia treatment

A

Targeted therapy

235
Q

Which one of the following is FALSE about epigenetic mechanisms

A

Hypermethylation of CpG islands in gene promoters result in their overactivation

236
Q

True or False: Spleen is a hematopoetic organ

A

Flase - lymphoid organ

237
Q

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?

A

AML with t(16;16)(p13;q22)

238
Q

Which of the following would be considered a sign of poten tially favorable prognosis in children with ALL?

A

Hyperdiploidy

239
Q

Signs and symptoms of cerebral infiltration with blasts are more commonly seen in

A

ALL

240
Q

An oncology patient exhibiting signs of renal failure with seizures after initial chemotherapy may potentially develop:

A

Tumor lysis syndrome

241
Q

. Disseminated intravascular coagulation is more often seen in association with leukemia characterized by which of the following mutations?

A

. t(15;17)(q22;q12)

242
Q

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?

A

AML with t(9;11)(p22;q23)

243
Q

. 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

A

Acute monoblastic/monocytic leukemia

244
Q

Pure erythroid leukemia is a disorder involving

A

Pronormoblasts and basophilic normoblasts

245
Q

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

A

Acute megakaryoblastic

246
Q

SBB stains which of the following component of cells

A

Lipids

247
Q

The cytochemical stain a-naphthyl butyrate is a nonspecific esterase stain that shows diffuse positivity in cells of which lineage

A

monocytic

248
Q

A peripheral blood film that shows increased neutrophils, basophils, eosinophils, and platelets is highly suggestive of:

A

CML

249
Q

CML gene present

A

. t(9;22)

250
Q

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?

A

. Transformation to acute leukemia

251
Q

The most common mutation found in patients with primary PV is

A

JAK2 V617F

252
Q

The peripheral blood in PV typically manifests:

A

. Erythrocytosis, thrombocytosis, and granulocytosis

253
Q

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:

A

ET

254
Q

Complications of ET include all of the following excep

A

Infections

255
Q

Which of the following patterns is characteristic of the peripheral blood in patients with PMF?

A

. Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes

256
Q

The myelofibrosis associated with PMF is a result of:

A

Enhanced activity of fibroblasts as a result of increased stimulatory cytokines

Hema Lec 2 (1 decks)

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