Brainscape's Knowledge GenomeTM

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


See full index

Hematology > White Cell Disorders Overview > Flashcards

White Cell Disorders Overview Flashcards

1
Q

What are the three major clues you can bet from a bone marrow biopsy to determine a WBC disorder ?

A
  1. What the abnormal cells look like 2. The immunophenytype of the abnormal cells 3. The Genotype of the abnormal cells.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the normal contents of a core marrow biopsy ?

A

Erythroid, Myeloid, and Magakaryocytes.

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

Is there iron in the bone marrow ?

A

Yes, on a histiocytic biopsy there will be ferritin in the stain.

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

What are the 5 tumor markers for B cells

A

CD-45 CD-79 CD-20 IgG Kappa IgG Lambda

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

What are the 5 common tumor markers for T cells ?

A

CD-45 TcR (CD-3) CD-7 CD-4 CD-8 (For the most part below ten)

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

What is the process for immunophenotyping WBC’s ?

A

The same as with RBC’s except you have to lyse the RBC’s first.

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

If a patient presents with a low white count, anemia, and thrombocytopenia what is this commonly called ?

A

Pancytopenia

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

What do the different regions on this flow cytometry readout correspond to ?

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

What does this region on the flow cytometry chart correspond to ?

A

This region is blasts

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

How do you know what protein cells in the blood are expressing ?

A

Immunophenotyping

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

What do you think this stain means ?

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

What is the difference between normal staining and immunostaining ?

A

The immunostain has an antibody that will bind to a specific tumor marker displayed on the surface of the WBC

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

Philedelphia Chromosome

A

Chronic Mylogenous Leukemia from a 9:22 translocation which most commonly shows up on FISH or traditional cytogenic examination.

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

IF Cytogenetic and Fish turn up normal and you still suspect the patient has CML what test should you perform next ?

A

PCR based DNA and RNA sequencing can turn up a NPM-1 mutation

Complete genome sequencing from two different samples, one from the leukemia cells and one from the skin biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How many point mutations can lead to AML ?

A

720: 12 in the coding regions and up to 52 in the noncoding regions. You must always ask yourself if this is relevant to the pathogenesis or an independent mutation

**This is inefficent because typically only 4 are found in clinically significant cases.

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

What are the 4 lineages that can cause malignancies in the marrow ?

A
  1. Niave Lymphocytes
  2. Megakaryocytes
  3. Erythroid Lineage
  4. Myleoid Lineage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What causes acute leukemia ?

A

Rapidly proliferating bone marrow stem cells which will be indicated by blasts in the peripheral blood and bone marrow

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

What caues myeloproliferative disease ?

A

Chronically proliferating clones which differentiate into terminally circulating blood cells

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

What causes myelodysplastic syndromes ?

A

Poorly functioning clones

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

If you have a mutation in the Myeloid lineage that causes Myeloid clones to proliferate indefinetly what is this called ?

A

Acute Myeloid Leukemia

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

What will rapidly proliferating lymphoblastic clones in the peripheral blood indicate ?

A

Acute Lymphoblastic Leukemia

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

What will rapidly proliferating hematopoietic stem cells in the marrow indicate ?

A

Acute Undifferentiated leukemia of mixed phenotypes

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

Should you ever see blasts outside the bone marrow ?

A

No, Never this will indicate Lymphoblastic Lymphoma or Myleoid Sarcoma

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

What should you think if you see proliferating blasts in the marrow and in the peripheral cells ?

A

Marrow blast number over 20 % in the marrow will indicate acute leukemia

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

What is the blast immunophenotype ?

A

CD 34 +

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

What is the myleoid blast phenotype ?

A

CD 34+ / CD 33+

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

What is the immunophenotype of lymphoid blasts ?

A

CD 19+ / CD 10+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Can leukemias be diagnosed with immunophenotypes alone ?

A

No because often the cancer will express mixed immunophenotypes but is a usefull tool.

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

Blasts in Marrow and Blood Stream

t(15;17) (q22;q12)

A

PML-RARA

AML Subtype

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

Blasts in Marrow and Blood Stream

t(8;21) (q22;q22)

A

RUN X1-RUNXT1

AML Subtype

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

Blasts in Marrow and Blood Stream

t( 12;21) (p13, q22)

A

TEL-AML-1

ALL

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

Blasts in Marrow and Blood Stream

Cytogenetic testing negative

A

FLT3 Mutation (+)

AML Subtype

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

When you see blasts in the marrow and blood steram what should you do ?

A

What is the blast count ?

What is the blast morphology ?

What is the immunophenotype of the blast ?

What is the genetic makeup of the blast ?

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

In AML what are the two classes of translocations and mutations ?

A

Class 1 provide a proliferative and Survival advantage

Class 2 provide impaired differentiation and apoptosis

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

What are the class 1 Mutation Translocations ?

A

FLT3-ITD

FLT3-TKD

KIT

RAS

PTPN-11

JAK-2

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

What are the class 2 mutation translocations ?

A

PML-RARA

Runx1-Run1x1t1

CBFB-Myh11

MLL Fusions

CEBPA

NPM-1

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

What is the Gilland Hypothesis for AML ?

A

It takes two classes of mutations or translocations to get AML

38
Q

What is the arrow pointing to ?

A

An Aeur Rod, This is a classical smear for Acute Myleoid Leukemia with an t(8;21) (q22, a22) Run X1- Run1X1

The smear shows myelocytes with some maturation with crystalization of the granule contents forming Auer Rods

**Remember these require a class 1 and a class 2 mutation

39
Q

Describe the morphology you see in this stain ?

A

This stain repersents Big Blasts, some of which can be cleaved to show bat wing blasts, with Many cytoplasmic granules and Auer rods in the sacs.

40
Q

What does CD-34 Cd-17 indicate on a immunophenotype ?

A

Blasts

41
Q

CD-13 CD-33 +

A

Granulocytes

42
Q

CD14 CD11b ?

A

Monocytes

43
Q

You hace blasts, monocytes, and Granulocytes in the peripheral smear, what kind of Leukemia is this ?

Notice the eosinophilia

A

Acute Myeloid Leukemia

44
Q

What do you see on this peripheral smear ?

A

The morphology of these cells is Granulocytic, or Monocytic or Monoblastic

45
Q

NPM-1+

CEBPA+

But The Cytogenetic Testing Comes Back Normal

A

Acute Myleoid Leukemia

46
Q

t(15;17) q22,12

A

PML RARA

AML subtype

47
Q

t(8;21) (q22,q22)

A

RUNx1-RUNTX1

AML subtype

48
Q

t(12;21) (p13,q22)

A

TEL AML -1

ALL Subtype

49
Q

What is the Gilliand Hypothesis

A

It requires two mutations for a neoplastic conversion into AML

AML AML AML

50
Q

How would you detect PML-RARA

RunX1- RunX1T1

CBFP-MYH11

MLL Fusions ?

A

These all are detected by Cytogenetic analysis

51
Q

t(8;21) with (q22, a22)

CD34+ HLA-Dr+ CD13+ CD 33 weak

A

There are auer rods, that combined with the cell markers CD34 CD 13 HLA-Dr indicate this is Acute Myleoid Leukemia

Auer Rods AML

52
Q

What are the B stem cell markers ?

A

CD-34 and TdT

53
Q

What are the lymphoid precursor markers in B cell development ?

A

CD-19 and CD-10

54
Q

What are the Pre-B cell markers ?

A

Cyto u and CD-20

55
Q

What transcription factors regulate progression from Stem Cells to Lymphoid Precursors ?

A
56
Q

Patient is presenting with DIC, Thrombocytopenia

CD 33+ CD 13+

t(15;17)

A

In the blood we see big blasts with cytoplasmic granules in the neutrophils and auer rods.

The t(15;17) will indicate a PML-RARA (Retinoic Acid Receptor)

Prognosis Good

Acute Myleoid Leukemia

57
Q

What transcription factor regulates transmission from PreB1 to PreB2

A
58
Q

Can Lymphoid Markers ever express myleoid markers ?

A

Yes they can express CD 13 and CD33

59
Q

t(9;22) (q34; q11.2)

A

BCR-ABL 1

60
Q

t(v;11q23)

A

MLL rearranged

61
Q

t(12;21) (p13, q22)

A

TEL AML-1 ETV6 RUNX1

62
Q

t(5;14) (q31, q32)

A

IL-3 IgH

63
Q

In leukemia what is the difference between Hyperdiploid and Hypodiploid

A

Hyperdiploid has over 5-0 chromosomes in the tumor and better prognosis

64
Q

t(9;22) (q34, q11.2)

CD10+ CD19+ TdT+

A

The smear shows large agranular blasts

CD10, CD19, TdT show you that is its Lymphoid

This is Acute Lymphocytic Leukemia

BCR-ABL prognosis is very poor for Leukemia

65
Q

What two cancers is the BCR-ABL mutation present in ?

A

ALL and CML

66
Q

IFKZ mutation is mutated in 84 percent of what type of cancer ?

A

Acute Lymphocytic Leukemia

67
Q

t(v;11q23)

What will this mutation do ?

A

This mutation will lead to ALL, MLL, and AML. it fuses a transcription regulator

68
Q

What does the t(12;21) (p13, q22) transmutation do ?

A

TEL-AML-1 (ETV-6 RUNX-1)

Usually associated with ALL. This is the ALL that has a 90% cure rate.

69
Q

t(14 q11; 10q24) with TdT+, CD3+, CD5+, Myleoid or B cell antigens

A

T-ALL most have a very poor prognosis

Most have a translocation of a oncogene to a TCR promoter. This is a common motif for Lymphoid oncogenesis

70
Q

What are myeloproliferative diseases ?

A

A disease characterized by chronically proliferating clones which differentiate into circulating blood cells.

71
Q

What disease is characterized by all stages of granulocyte maturation present in the blood smear ?

A

Chronic Mylogenous Leukemia

72
Q

What disease is characterized by monocytes, promonocytes, and weird hybrids between monocytes and granulocytes ?

A

Chronic Myelomonocytic Leukemia

73
Q

What diseases are characterized by elevated RBC counts ?

Elevated Platelet counts?

A

Polycytemia Vera

Essential Thrombocytopenia or primary myelofibrosis

74
Q

How can you distinguish between Myeloproliferative disease and sepsis ?

A

Myeloproliferative diseases have the myleoid buldge and sepsis does not

75
Q
A

There is more Metas than myelocytes so this is a case of Sepsis

76
Q

What is this a case of ?

A

More Myelocytes than metas is Myeloproliferative disease

77
Q
A

This is CML and the peripheral smear shows Granulocyte precursors

78
Q

What is mastocytosis ?

A

Mast cells similiar to basophils but longer lived and non circulating

79
Q

How do mast cell neoplasms normally present ?

What is the most common site of mast cell neoplasms ?

A

As cutaneous lesions in kids, Uticaria Pigmentosum

The bond marrow but often other organs can be involved

80
Q

This immunophenotype is positive for Tryptase , CD 117, CD25

What kind of mutation will be likely with this presentation

A

Mastocytosis, notice the aggregates of band looking cells with round spindel shaped, and sometimes containing eosinophillia.

cKIT is common in this presentation

81
Q

What type of mutation is present in Polycytemia Rubivera ?

What do these patients present with ?

A

Jak-2 Mutations are present in over 95 precent

Thrombosis, Hypertension Stroke and MI

82
Q

Increased RBC’s ?

Increased Platelets ?

Increased Platelets ?

A

Polycytemia Vera

Essential Thrombocytemia

Primary Myelofibrosis

83
Q

The patient is presenting with thrombosis, hypertension and stroke with a Jak-2 Mutation

A

Polycytemia Vera

84
Q

What do you see in this smear ?

A

A greatly increased number of platelets and Megakaryocytes

This is essential thrombocytopenia

85
Q

What can you see in this peripheral smear ?

A

Increased Megakaryocytes bizarre shapes and clustering Fibrosis.

This is Primary Myelofibrosis

86
Q

What can myelodysplastic syndrome progress to ?

A

Acute Leukemia

87
Q

When you see unexplained pancytopenia and this on the peripheral smear, what will you suspect ?

A

Refractory Cytopenia It can also present with ringed cyteroblasts

88
Q

What kind of cell is this ?

With a 5q deletion what disease will this cause ?

A

Mononuclear Megakaryocyte

MDS

89
Q

What is the abnormal spear associated with ?

A

Refractory Cytopenia

90
Q
A

Hematology (12 decks)

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