Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Blood and Lymph > Unit II > Flashcards

Unit II Flashcards

1
Q

Hematologic malignancies share the common characteristic that they arise from ______ ____ that came from a transformed cell of marrow derivation

A

clonal populations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

leukemia

A

malignancy of hematopoetic cells, where the chief manifestation is involvement of the blood and marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

lymphoma

A

a malignancy of hematopoetic cells, derived from lymphocytes or their precursors, which presents primarily as a solid mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Extramedullary myeloid tumor

A

(granulocytic sarcoma)-a malignancy of hemaotpoetic cells, derived from myeloid cells or their precursors (granulocytes, monocytes, etc) which presents primarily as a solid mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Persistant findings of chromosomal translocation are important to note because:

A

1) their persistence allows them to be used as diagnostic markers for certain hematological malignancies
2) Their persistent presence suggests they play critical role in development of the hematological malignancy they are associated with

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What might be one explanation of the frequent translocations in lymphocytes?

A

genomic instability during immunoglobulin/T cell receptor rearrangement in B and T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

3 viruses known to play a role in lymphomas:

A

1) Epstein-Barr virus (EBV)
2) Human T cell leukemia virus-1 (HTLV-1)
3) Kaposi sarcoma herpesvirus/Human herpesvirus-8 (KSV/HHV-8)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Acute Leukemias

A

Usually due to rapid accumulation of (usually) immature cells in the marrow. They often replace many normal marrow cells, resulting in cytopenias. Most can be classified as Acute Myeloid Leukemia or Acute Lymphoblastic Leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Myelodysplastic syndromes

A

a group of conditions where a clonal population derived from a neoplastic HSC takes over marrow, and is NOT capable of making normal blood cells in one or more lineages (dysplasia). Categorized by falling peripheral blood cell counts. Often MDS can progress to AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Myeloproliferative Neoplasms

A

neoplastic clonal proliferations of the marrow where the clone makes normal functioning blood cells, usually in multiple lineages, but makes too many of them in one or more lineages.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What causes the signs and symptoms of acute leukemia?

A

When immature leukemic cells accumulate in the bone marrow, they displace normal hematopoetic elements.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Chromosomal abnormalities may be found in __% of AML patients and ___% of ALL patients

A

95

90

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Different types of AML:

A

granulocytic, monocytic, erythroid, megakaryocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Risk factors for acute leukemia

A
  • previous chemo, esp. with DNA alkylating agents and topoisomerase II inhibitors
  • tobaccos smoke
  • ionizing radiation
  • benzene exposure
  • Syndromes: Down, Bloom, Fanconi anemia, ataxia-telangiectasia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Signs and symptoms of acute leukemia

A

fatigue, malaise, dyspnea, easy bruisability, weight loss, bone pain, anemia and pallor, thrombocytopenia, hemorrhage, ecchymoses, petechia, fundal hemorrhage, fever and infection, adenopathy, hepatosplenomegaly, mediastinal mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Most common age for ALL

A

75% of cases of ALL occur in children under age 6

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

In order to diagnose ALL, lymphoblasts must be identified. This is done with :

A

immunophenotyping

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Immunophenotyping markers to diagnose ALL

A

CD34-marks immature lymphobasts, but often expressed by myloblasts.
TdT-expressed by immature lymphocytes only

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Does B-ALL or T-ALL account for more cases of ALL?

A

B-ALL accounts for 80-85% of all cases of ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

B lymphoblasts express Be lineage antigens:

A

CD19, CD22, CD79a

usually do not express mature B cell markers such as CD20

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Recurrent cytogenetic abnormalities of B-ALL

A

1) t(9,22) BCR-ABL1
2) t of 11q23, MLL
3) t(12;21) ETV6-RUNX1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Rank the prognoses of the most common B-ALL cytogenetic abnormalities

A

t(12;21) > t 11q23 MLL > t(9;22)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What age group does T-ALL occur in most commonly?

A

adolescents and young adults and males more than females

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How does T-ALL often present?

A

with a component of lymphoblastic lymphoma (T-LBL), often manifesting as a large mediastinal mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

T lymphoblasts express T-lineage antigens ___, ___, and/or ____. They may express both ___ and ___ concurrently, or may express just one/neither. They often express T-lineage antigens only seen in immature T cells, such as ____and ___

A

CD2, CD3, CD7
CD4, CD8
CD99, CD!a

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is the prognosis for ALL in children? In adults?

A

Good prognosis in children, complete remission rate 95%, 80% cure rate. In adults, disease is much worse. Complete remission 60-80%, cure rate of <50%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Prognostic factors for all ALLs

A 
Age (worst for infants, >10)
WBC count (more=worse)
Slow response to therapy, disease left after treatment
hyperdiploidy=good, hypodiploidy=bad
T cell worse
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Who does AML occur in most commonly?

A

average age id 65, rare in children and young adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

the diagnosis of AML is usually based on:

A

identifications of increased myeloblasts accounting for 20% or more of nucleated cells in the marrow or peripheral blood. (exception: if one can document presence of Auer rods)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Auer rods

A

Indicate AML and are found in myeloblast

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Myeloblasts often express myeloid antigens such as

A

CD117 (C-kit), myeloperoxidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Recurrent AML cytogenteic abnormalities and prognosis

A

1) t(8;21) RUNX!-RUNX1T1-Good
2) inv (16) or t(16;16) CBFB-MYH11-good
3) t(15;17) PML-RARA
- This is APL-best prognosis of AMLs with ATRA
4) t(1;22) RBM15-MKL1-good
5) abnormalities of 11q23 MLL-Poor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

In rare cases, APL can give rise to:

A

disseminated intravascular differentiation (DIC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Which AML cytogenetic abnormality is associated with infants with Down syndrome?

A

AML with t(1;22) RBM15-MKL1, usually showing megakaryoblastic differentiation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Two most common types of therapy related AML (t-AML):

A

1) secondary to alkylating agents or radiation

2) secondary to topoisomerase II inhibitors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Molecular findings that are the most important prognostic factors in AML, NOS

A

FLT3 ITD (- prognosis)
NPM1 (+)
CEBPA (+)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Myelodysplastic syndrome

A

group of conditions where the marrow is replaced by a malignant clone, derived from a transformed stem cells or progenitor cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Characteristics of Myelodysplastic syndrome (MDS

A

1) ineffective hematopoesis (clone can’t make functioning blood cells-they often die before leaving marrow and look dysplastic
2) risk of transformation to AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Median age of Primary (idiopathic) MDS

A

70, usually occurs after age 50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Secondary MDS (t-MDS) occurs:

A

as a part of the spectrum of therapy-realted AML, generally 2-8 years after therapy with DNA alkylating agents or ionizing radiation

41
Q

Secondary MDS (t-MDS) karyotype

A

generally complex, whole or partial deletions of chromosomes 5 and/or 7

42
Q

MDS is generally diagnosed after noticing:

A

1) persistant peripheral cytopenia in one or more lineages that cannot be otherwise explained (highly likely if advanced age and 2+ cytopenias). If there is a cytopenia in a single lineage, it is not usually MDS

43
Q

MDS diagnosis can be made in setting of persistent cytopenia with wine of the following modalities:

A

1) Morphological evidence of dysplasia
2) Increased myeloblasts, but less than 20% of blood and marrow cells
3) presence of a clonal cytogenetic abnormality

44
Q

dyshematopoiesis

A

When more than 10% of the cells in one lineage (erythroid cells, granulocytes, megakaryocytic) appear dysplastic.

45
Q

dyshematopoiesis can be sub classified as:

A

Dyserythropoiesis, Dysgranulopoiesis, Dysmegakaryopoiesis

46
Q

DYSERYTHROPOIESIS morphological characteristics

A

Megaloblastoid Chromatin Patterns in
RBC Precursors, Nuclear Irregularities
(irregular contour, irregular shape, nuclear budding, multiple nuclei) in RBC precursors, Prominent Ring Sideroblasts

47
Q

MORPHOLOGIC EVIDENCE OF:

DYSGRANULOPOIESIS

A

POOR CYTOPLASMIC GRANULATION (Hypogranular), Abnormal Nuclear Segmentation (neutrophils with 2 lobes- pseudo-Pelger-Huet, because they resemble the bilobed nuclei seen in the neutrophils of patients with Pelger-Huet anomaly, a benign congenital condition)

48
Q

MORPHOLOGIC EVIDENCE OF:

DYSMEGAKARYOPOIESIS

A

Small, often hypolobated or non-lobated nuclei

49
Q

The presence of one of the following MDS- related abnormalities within the clone is DIAGNOSTIC of MDS:

A
  • complex karyotype with whole or partial deletions of chromosomes 5 and/or 7
  • isolated deletion 5q
  • trisomy 8
50
Q

In a patient with suspected MDS, but negative for elevated myeloblasts and negative for a clonal cytogenetic abnormality, and only having morphologic evidence of dysplasia, be sure to exclude potential causes of secondary myelodysplasia. These include:

A
  • VITAMIN DEFICIENCY (B12, folate, etc.)
  • TOXIN EXPOSURE (e.g. heavy metals)
  • EXPOSURE TO CERTAIN DRUGS
  • VIRAL INFECTIONS
51
Q

MDS is classified into two types:

A

Low and high grade MDS

52
Q

Characteristics of low grade MDS

A

Myeloblasts account for <2% of blood cells

53
Q

Characteristics of high grade MDS

A

Myeloblasts account for 5-19% of marrow cells, and/or 3-19% of blood cells

54
Q

Types of low grade MDS:

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

Refractory Cytopenia with Multilineage Dysplasia (RCMD)

55
Q

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

A
  • Low grade MDS with dysplasia in ONE lineage
  • Mostly cases of refractory anemia, rarely may be refractory neutropenia or refractory thrombocytopenia
  • Relatively good prognosis: - Median survival >5 years
  • Only 2% of cases transform to AML by 5 years
56
Q

Refractory Cytopenia with Multilineage Dysplasia (RCMD)

A
  • Low grade MDS with dysplasia in 2 or more lineages
  • Worse prognosis than RCUD:
    • Median survival: 2.5 years
    • 10% of cases transform to AML by 2 years
57
Q

Types of High grade MDS

A

Refractory Anemia with Excess Blasts-1 (RAEB-1)

Refractory Anemia with Excess Blasts-2 (RAEB-2)

58
Q

Refractory Anemia with Excess Blasts-1 (RAEB-1)

A
  • 5-9% blasts in marrow, and/or 2-4% blasts in blood
  • Relatively dismal prognosis: - Median survival of 16 months
    • 25% of cases will transform to AML
59
Q

Refractory Anemia with Excess Blasts-2 (RAEB-2)

A
  • 10-19% blasts in marrow, and/or 5-19% blasts in blood
  • Very dismal prognosis:
    • Median survival of 9 months
    • 33% of cases will transform to AML
60
Q

Myeloproliferative neoplasms (MPNs)

A
  • Clonal hematopoietic neoplasm arising from a transformed hematopoietic stem cell
  • neoplastic clone partially or entirely replaces the normal marrow cells in multiple lineages
  • neoplastic clone gives rise to increased numbers of normal (not dysplastic) blood cells in one or more lineages
61
Q

Myeloproliferative neoplasms usually occur in the following age group:

A

middle aged-elderly adults, rare in children

62
Q

Common themes of MPNs:

A

1) Early disease=increase in 1+ blood cell types, increased marrow cellularity
2) splenomegaly and/or hepatomegaly
3) Usually insidious onset
4) W/o treatment, progress to excessive marrow fibrosis, resultant bone marrow failure and/or transformation to acute leukemia (much less common for MPNs than for MDS)

63
Q

Without treatment, myeloproliferative neoplasms progress to….

A

-excessive marrow fibrosis with resultant bone marrow failure - -transformation to acute leukemia (much less common for MPNs than for MDS)

64
Q

Historically, MPNs were classified based on the _____________________, and the appearance of the marrow on biopsy

A

type(s) of cells being overproduced

65
Q

Important types of myeloproliferative neoplasms

A 
Chronic Myelogenous Leukemia (CML)
Polycythemia Vera (PV)
Primary Myelofibrosis (PMF)
Essential Thrombocythemia (ET)
66
Q

Chronic myelogenous leukemia often manifests as

A

persistent neutrophilic leukocytosis from a CBC. Some are symptomatic at diagnosis. Others have non-specific signs/symptoms, including night sweats, weight loss, splenomegaly, and anemia

67
Q

Typical age of CML diagnosis

A

Typical age at diagnosis: 40-60; rare in children and young adults

68
Q

Stages of CML are ____ and _____. when are people usually diagnosed?

A

chronic phase, blast phase. Large majority of CML cases are diagnosed in the initial phase of disease, chronic phase

69
Q

Characteristics of chronic phase CML

A
  • Leukocytosis from neutrophilia - Increased basophils in blood
  • Often increased platelets in blood
  • Hypercellular bone marrow with granulocytic hyperplasia (neutrophilic granulocytes in peripheral blood increased)
70
Q

Appearance of megakaryocytic in chronic phase CML bone marrow biopsy

A

Frequent Small Megakaryocytes with Non-Lobated Nuclei

71
Q

Definition of blast phase of CML

A

defined by 20% or more blasts in the marrow or blood (essentially, CML-blast phase is CML that has transformed to acute leukemia)

72
Q

Types of blasts present in marrow and blood CML blast phase

A

In 70% of cases of CML-blast phase, the blasts are myeloblasts
30% of cases, the blasts are lymphoblasts

73
Q

How can CML progress from chronic–> blast phase?

A

Directly from chronic phase–> blast phase
or
through an intermediate phase known as accelerated phase

74
Q

CML translocation, how it differs from ALL

A

breakpoint in the major breakpoint cluster region of BCR (M-BCR), giving rise to a 210kd fusion protein (p210).

Ph+ ALL, the BCR breakpoint is usually in the minor breakpoint cluster region (m-BCR), giving rise to a 190kd fusion protein (p190)

75
Q

The p190 mRNA transcripts are detectable in most cases of CML, why?

A

Splicing of 210kD protein can give rise to 190kd protein if it spliced

76
Q

BCR-ABL1 fusion gene can be documented by

A

Conventional cytogenetics (karyotype) - FISH (BCR-ABL fusion probes) - RT-PCR (BCR-ABL mRNA transcripts)

77
Q

Polycythemia Vera is chiefly characterized by

A
  • by an increase in RBC mass (erythrocytosis)

- Usually, also see increased neutrophils and platelets in the blood, and trilineage hyperplasia in the marrow

78
Q

What will a bone marrow biopsy of Polycythemia Vera show?

A

often shows clusters of bizarre megakaryocytes

79
Q

Genetic factors from Polycythemia Vera

A

Essentially all cases of PV contain an activating mutation of JAK2, usually a V617F point mutation.

80
Q

If a patient has a persistent erythrocytosis in the absence of a demonstrable JAK2 mutation, consider the possibility of a secondary (reactive) erythrocytosis. Secondary erythrocytosis may be seen in:

A
  • Smokers (due to carboxyhemoglobin)
  • Chronic hypoxia
  • Certain hemoglobin disorders
  • Other conditions
81
Q

Phases of Polycythemia Vera, when is it usually diagnosed

A

polycythemic phase (diagnosis probably), spent phase

82
Q

Characteristics of spent phase PV

A

post-PV myelofibrosis. Spent phase is characterized by extensive marrow fibrosis with a corresponding fall in blood cell counts.

83
Q

Initial presenting symptoms in PV patients include:

A
  • headaches - dizziness
  • plethora (dusky, reddish skin)
  • pruritus (itching)
  • paresthesias (“pins and needles” sensations) - splenomegaly (70%); hepatomegaly (40%)
84
Q

Most serious complication of PV

A

are venous or arterial thrombosis, leading to complications such as DVT, MI, and stroke. Most common cause of death!

85
Q

What thromboses are particularly indicative of PV?

A

Thrombosis of the mesenteric vein, portal vein, or splenic vein should always raise the possibility of PV

86
Q

Prognosis and risk of transformation to acute leukemia, and main therapeutic methods for PV

A

relatively good prognosis (without chemotherapy, risk of transformation to acute leukemia is very low), median survival times of 10-20 years
Main therapeutic methods are: - serial phlebotomy
-aspirin therapy (to help prevent clots)

87
Q

Primary Myelofibrosis

A

an MPN characterized by granulocytic and megakaryocytic hyperplasia, but no erythrocytosis.

88
Q

Genetic findings of Primary Myelofibrosis

A

JAK2 mutations are present in around 50% of PMF cases

89
Q

Stages of Primary myelofibrosis

A

prefibrotic stage and fibrotic stage

90
Q

Characteristics of prefibrotic stage of primary myelofibrosis

A
  • hyper cellular marrow- granulocytic and megakaryocytic hyperplasia - large, bizarre megakaryocytes in marrow - markedly increased platelets in blood - usually increased neutrophils in blood
91
Q

Characteristics of fibrotic stage of primary myelofibrosis

A
  • reticulin fibrosis of the marrow
  • Leukoerythroblastosis of the blood
  • Falling blood cell counts
  • Enlargement of organs (liver, spleeen, lymph nodes, others) due to extramedullary hematopoiesis
92
Q

Peripheral smear of primary myelofibrosis

A
  • immature granulocytes and immature red cells
  • Tear drop-shaped RBCs (dacrocytes) in blood
  • fibrosis giving the marrow cells a “streaming” appearance
  • Entrapped bizarre megakaryocytes suggest an MPN
  • reticulin fibrosis
93
Q

Primary myelofibrosis:

  • MST if diagnosed in fibrotic stage
  • Most deaths from
  • Transform to AML?
A
  • MST=5yrs
  • Most deaths due to bone marrow failure
  • minority of cases may transform to AML
94
Q

Essential Thrombocytopenia

A

an MPN characterized by a persistent thrombocytosis. It lacks the marrow granulocytic hyperplasia often seen in PMF, and the atypical megakaryocytes in ET are even larger than those in PMF

95
Q

Genetic components of essential thrombocythemia

A

JAK2 mutations are present in around 50% of ET cases

96
Q

Essential thrombocythemia marrow characteristics

A

Clusters of Very Large Megakaryocytes in Marrow

97
Q

Symptoms of Essential thrombocythemia

A

50% of ET patients are asymptomatic

  • TIAs, digital ischemia, arterial or venous thromboses
  • spelnomegaly UNCOMMON
98
Q

Essential thrombocythemia

  • MST
  • Transform to AML?
A

ET is indolent disease, with median survivals of over 10 years
Only rarely does ET transform to AML, or progress to myelofibrosis

Blood and Lymph (3 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions