Pathology Exam 3 - Hematology

Front 1

What is hematocrit? What is the normal hematocrit for men and women?

Back 1

The proportion of blood occupied by RBCs.

48% men; 38% women

Front 2

What is the normal Hgb for men and women?

Back 2

Men = 13.8 - 17.2 g/dL

Women = 12.1 - 15.1 g/dL

Front 3

What is the normal MCV?

Back 3

80 to 100 femtoliters

Front 4

What is the normal MHC?

Back 4

27 to 31 picograms per cell

Front 5

What is the normal MCHC?

Back 5

32 to 35 g/dL

Front 6

When is RDW increased?

Back 6

In iron deficiency anemia

This is due to a variation in size of RBCs in the peripheral blood

Front 7

What is considered anemia?

Back 7

Being 2 standard deviations below the mean RBC count of the general population

Front 8

What causes hypochromic microcytic anemia (4 things)?

Back 8

Iron deficiency, thalassemia, anemia of chronic disease, sideroblastic anemia

Front 9

What causes normocytic anemia?

Back 9

Sickle cell anemia, hereditary spherocytosis, acute blood loss, G6PD deficiency, hemolytic anemias

Front 10

What is a cause of teardrop-shaped RBCs?

Back 10

Myelofibrosis

Front 11

What is a cause of "target RBCs"?

Back 11

Hemoglobinopathies, such as thalassemia

Front 12

Anemia plus skeletal abnormalities indicates what kind of process?

Back 12

Hemolytic anemia w/ expansion of the bone marrow for compensation

Hemolysis of the RBCs may also cause jaundice

Front 13

Anemia plus iron overload indicates what kind of process?

Back 13

Defective erythropoiesis

Front 14

What does a direct Coomb's test measure? What does an indirect Coomb's test measure?

Back 14

Direct = antibodies/complement proteins that are bound to the surface of RBCs

Indirect = antibodies in the pts blood that target RBCs

Front 15

Which kind of immunoglobulins are the warm type? Which kind of immunoglobulins are the cold type?

Back 15

Warm = mostly IgG, rarely IgA

Cold = only IgM

Front 16

What is an acute cause of a cold-type immune hemolytic anemia?

Back 16

Infection!

Usually infectious mononucleosis or mycoplasma

Front 17

What are the symptoms of a cold-type immune hemolytic anemia caused by?

Back 17

Vascular obstruction

Front 18

What is a chronic cause of a cold-type immune hemolytic anemia?

Back 18

B-cell lymphoma; can also be idiopathic

Front 19

What is the inheritance pattern of hereditary spherocytosis?

Back 19

Autosomal Dominant

Front 20

What is the most common protein affected in hereditary spherocytosis? Name the additional proteins that can be affected?

Back 20

Most common = ankyrin

Can also involve band 3, spectrin, and band 4.2

Front 21

Splenectomy is a treatment in hereditary spherocytosis. What will you see on a blood smear after such a treatment?

Back 21

Howell-Jolly bodies

Pieces of nuclei that remain stuck in the RBC

Front 22

Which two symptoms of hereditary spherocytosis are fixed w/ splenectomy? Which two signs are not fixed?

Back 22

Fixed = anemia, hyperbilirubinemia

Not fixed = spherocytosis, increased cell osmotic fragility

Front 23

An aplastic crisis in someone who has hereditary spherocytosis is most caused by what event?

Back 23

Parvovirus infection

Front 24

On which chromosome are the beta-hemoglobin chains synthesized?

Back 24

Chromosome 11

Front 25

Which type(s) of beta-thalassemia require transfusions?

Back 25

Only b-thalassemia MAJOR

Front 26

What are the consequences of a decrease in one of the Hgb chains?

Back 26

1. Low intracellular Hgb

2. Excess of the other chain, which can lead to free chains forming insoluble aggregates which can damage membranes

Front 27

What are the common symptoms (3) of beta-thalassemia?

Back 27

1. Skeletal deformities due to marrow expansion

2. Hepatosplenomegaly

3. Iron overload due to multiple transfusions

Front 28

How do you distinguish beta-thalassemia minor from iron deficiency anemia?

Back 28

Beta-thal minor has an IRON OVERLOAD

Front 29

A CBC of a patient w/ beta-thal minor will show what?

Back 29

Decreased = Hgb, MCV, MCH, MCHC

Increased = # of RBC per liter

Normal = RDW

Front 30

What are the four (4) clinical manifestations of alpha-thalassemia?

Back 30

1. Hydrops fetalis
2. Hemoglobin H disease
3. Alpha-thal trait
4. Silent carrier

Front 31

What is Hemoglobin Bart disease?

Back 31

Tetramers of the gamma chains of Hgb

Front 32

What is Hemoglobin H disease?

Back 32

Tetramers of the beta chains of Hgb

Front 33

Compare the free chains seen in beta-thal vs alpha-thal

Back 33

Alpha-thal has better prognosis!

Non-alpha chains generally form more soluble and less toxic aggregates than those from alpha chains

Front 34

Compare the symptoms seen in beta-thal vs alpha-thal

Back 34

Alpha-thal is less severe!

1) Hemolytic anemia and 2) ineffective erythropoiesis is less severe in alpha-thal than beta-thal

Front 35

What is the normal purpose of G6PD?

Back 35

It keeps up the normal levels of NADPH

Front 36

What is the inheritance pattern of G6PD deficiency?

Back 36

X-linked recessive

Front 37

What is a sign of G6PD deficiency?

Back 37

EPISODIC hemolysis

Can be due to infections, drugs, fava beans

Front 38

In G6PD deficiency, oxidants cause the precipitation of what?

Back 38

Heinz bodies

Front 39

What kind of hemolysis is caused by the Heinz bodies in G6PD deficiency?

Back 39

Intravascular - can directly damage RBC membrane

Extravascular - macrophages may attempt to "bite" out the Heinz bodies

Front 40

What type of immunoglobulin is responsible for paroxysmal nocturnal hemoglobinemia?

Back 40

IgG

Binds RBCs and fix complement @ low temps; hemolysis presents @ high temps

Front 41

What is the usual cause of paroxysmal nocturnal hemoglobinemia?

Back 41

Viral infections!

Infectious mononucleosis, mycoplasma

Front 42

What syndrome is associated w/ iron deficiency?

Back 42

Plummer-Vinson syndrome

Loilonychia+alopecia+glossitis+esophageal webs

Front 43

What term describes an abnormal shape of RBCs?

Back 43

Poikilocytosis

Front 44

What term describes RBCs of unequal sizes?

Back 44

Anisocytosis

Front 45

When does iron absorption increase, even with increased body stores?

Back 45

If you have ineffective erythropoiesis

Front 46

In iron deficiency anemia, what do you see regarding serum iron, TIBC, and transferrin saturation?

Back 46

Serum iron is LOW
Tissue iron binding capacity is HIGH
Transferrin saturation is LOW

Front 47

What will you see in the bone marrow with aplastic anemia?

Back 47

Increased fat

Small foci of lymphocytes/plasma cells

Front 48

What is the order of development for normal erythropoiesis?

Back 48

Proerythroblast
Basophilic normoblast
Polychromatic normoblast
Orthochromic normoblast
Reticulocyte
RBC

Front 49

Which are larger, lymphoblasts or myeloblasts?

Back 49

Myeloblasts

Front 50

What describes the chromatin of myeloblasts - finely distributed, or slightly clumped?

Back 50

Finely distributed

Front 51

Which might contain Auer rods - lymphblasts or myeloblasts?

Back 51

Myeloblasts

Front 52

What describes the nucleoli of lymphoblasts - inconspicuous or prominent?

Back 52

Inconspicuous

Front 53

Describe the size, nucleus shape, nucleoli, and cytoplasm of Burkitt leukemia lymphoblasts.

Back 53

Size = large
Nucleus = oval/round (same as other lymphoblasts)
Nucleoli = prominent and multiple
Cytoplasm = abundant and basophilic

Front 54

What immunophenotype separates Burkitt leukemia from precursor B-ALL?

Back 54

Monoclonal surface IgM!

It MUST be surface, not core IgM

Front 55

What immunophenotype is characteristic of precursor T-ALL?

Back 55

CD7

It is found on all immature T cells

Front 56

Which has the best prognosis - precursor-B, precursor-T, or Burkitt leukemia? Which has the worst?

Back 56

Best = precursor-B cell leukemia (also has highest frequency)

Worst = Burkitt leukemia (also has lowest frequency)

Front 57

How does the frequency of precursor-B cell ALL change with age?

Back 57

Rate DECREASES with age

Front 58

How does the frequency of precursor-T cell ALL vary with age?

Back 58

Rate INCREASES with age

Front 59

Which translocation yields Burkitt leukemia, and thus a poor prognosis?

Back 59

t(8;14)

Front 60

t(8;14)?

Back 60

Burkitt leukemia

Front 61

What are three (3) "good prognosis" cytogenic abnormalities?

Back 61

1. Hyperdiploidy

2. Simultaneous trisomy of 4, 10, 17

3. Translocation of t(12;21) to create a TEL/AML1 fusion

Front 62

What are three (3) "poor prognosis" cytogenic abnormalities?

Back 62

1. Hypodiploidy

2. t(9;22) Philadelphia chromosome

3. t(4;11)

Front 63

t(12;21) - good or bad prognosis?

Back 63

Good!

Front 64

t(4;11) - good or bad prognosis?

Back 64

Bad!

Front 65

Which trisomy has a good prognosis in ALL/AML?

Back 65

Trisomy 4, 10, 17

Front 66

In terms of age and WBC count, what is considered "high-risk" in precursor-B ALL?

Back 66

Age > 10

WBC count > 50000 cells/uL

Front 67

What two other medical conditions would put you in "high-risk" in precursor-B ALL

Back 67

1. CNS disease

2. Testicular disease

Front 68

Name five (5) differences about precursor T-ALL compared to precursor B-ALL.

Back 68

1. Higher median WBC count
2. Higher median age
3. Higher % of male predominance
4. Mediastinal mass
5. Higher frequency of lymphadenopathy

Front 69

What are two (2) differences about Burkitt leukemia vs precursor T-ALL?

Back 69

1. Older median age

2. CNS involvement is seen in Burkitt leukemia

Front 70

What is the most common leukemia in the US?

Back 70

Chronic lymphocytic leukemia

Front 71

Describe the typical CLL patient

Back 71

Elderly & male

Front 72

What is the major cause of death in CLL?

Back 72

Infection; bone marrow becomes compromised due to infiltration by lymphocytes

Front 73

What will you see in a CBC with a CLL patient?

Back 73

Lymphocytosis and leukocytosis

Front 74

What is Richter syndrome?

Back 74

The transformation of CLL into a large-cell lymphoma (such as Hodgkin)

Front 75

What is the immunological phenotype of CLL?

Back 75

Typical B-cell markers ALONG WITH CD5!!

Front 76

In CLL, which is associated with the worse prognosis; having somatic mutations or lacking somatic mutations?

Back 76

Lacking somatic mutations is worse

Will see CD38 if it lacks somatic mutations

Front 77

When do you treat a patient for CLL?

Back 77

When they become symptomatic

Front 78

What are the most important clinical findings (2) in Hairy Cell Leukemia?

Back 78

MASSIVE splenomegaly w/o lymphodenopathy

Pancytopenia

Front 79

What is the most distinctive morphological feature in hairy cell leukemia?

Back 79

An abundant pale blue cytoplasm that appears delicate

Front 80

What is the immunophenotype of hairy cell leukemia? What CD markers are indicative?

Back 80

Is a B-cell leukemia at a more mature stage of development than CLL

CD11c and CD103 in addition to B-cell markers

Front 81

How do you diagnose hairy cell leukemia?

Back 81

With a bone marrow biopsy!

A bone marrow aspiration will only result in a "dry tap"

Front 82

Small lymphocytic lymphoma may be considered a tissue manifestation of which leukemia?

Back 82

Chronic lymphocytic leukemia

Front 83

What is the immunophenotype of small lymphocytic lymphoma?

Back 83

B-cell markers + CD5

Front 84

Is follicular lymphoma B-cell or T-cell in origin?

Back 84

B-cell

Front 85

Small lymphocytic lymphoma usually affects which age group?

Back 85

Older adults (> 60 years)

Front 86

How do you differentiate centrocytes from centroblasts?

Back 86

1. Centroblasts are larger
2. Centrocytes have an elongated nucleus, while centroblasts have an oval/round nucleus
3. Centrocytes have dark-staining chromatin, while centroblasts have pale dispersed chromatin

Front 87

Follicular lymphoma will typically have monoclonality of what kind of chain?

Back 87

Monoclonality of a single LIGHT chain

Front 88

90% of follicular lymphomas have what kind of translocation?

Back 88

t(14;18)

Places the BCL2 gene next to the heavy chain gene

Front 89

t(14;18) translocation is indicative of which lymphoma?

Back 89

Follicular lymphoma

Front 90

Diffuse Large B-cell lymphoma is a proliferation of what?

Back 90

Centroblasts

Front 91

Which lymphoma may be difficult to separate from other malignancies?

Back 91

Diffuse Large B-cell lymphoma

Front 92

What is the most common non-Hodgkin lymphoma?

Back 92

Diffuse Large B-cell lymphoma

Makes up 30-40% of cases

Front 93

What is clinically significant about diffuse large B-cell lymphoma

Back 93

It is aggressive, but curable

cyclophosphamide+adriamycin+vincristine+prednisone+rituxin

Front 94

What age group is affected by diffuse large B-cell lymphoma?

Back 94

All age groups!

Front 95

Which is more common: peripheral T-cell lymphomas or B-cell lymphomas?

Back 95

B-cell lymphomas

Front 96

What is the most common pediatric precursor lymphoblastic lymphoma?

Back 96

Precursor T-cell lymphoblastic lymphoma

Front 97

Which pediatric lymphoma is primarily supradiaphragmatic?

Back 97

Precursor B/T-cell lymphoblastic lymphoma

Front 98

Which pediatric lymphoma is primarily subdiaphragmatic?

Back 98

Burkitt lymphoma

Front 99

Are the pediatric lymphomas follicular or diffuse type?

Back 99

Diffuse

Front 100

Which pediatric lymphoma has absent/inconspicuous nucleoli w/ scanty cytoplasm?

Back 100

Precursor B/T-cell lymphoblastic lymphoma

Front 101

Which pediatric lymphoma has prominent multiple nucleoli w/ narrow rims of purple cytoplasm?

Back 101

Burkitt lymphoma

Front 102

Which five (5) factors make up the International Prognostic Index for lymphoma?

Back 102

1. Age
2. Tumor stage
3. Number of extranodal sites
4. Performance status
5. Serum LDL level

Front 103

What is found in primary granules? Which type of cells is this found in?

Back 103

Myeloperoxidase

Found in promyelocytes

Front 104

What is found in secondary granules?

Back 104

Lactoferrin

Front 105

What is the characteristic enzyme found in monocytes and monocytopoiesis?

Back 105

Myeloperoxidase

Front 106

Which lineage marker is found only on mature granulocytes (segmented neutrophils and band cells)?

Back 106

CD10

Front 107

What are lineage markers for myelopoiesis/monocytopoiesis?

Back 107

CD13, CD15, CD33, CD64

Front 108

What is the most common cause of leukopenia / neutropenia?

Back 108

Drugs!

Front 109

What is toxic granulation? When would you see this event?

Back 109

Describes persistent primary granulation

Seen in neutrophilia due to infections

Front 110

When would you see basophilia?

Back 110

Myeloid neoplasms (AML, CML)

Front 111

What is the common feature underlying all myeloid neoplasms?

Back 111

An origin from hematopoietic progenitor cells

Front 112

Bleeding diathesis in AML is common in what translocation?

Back 112

t(15;17)

Front 113

Myeloblasts have what enzyme in their granules, and thus stain positive with what?

Back 113

Myeloperoxidase positive

Stains with O-Toulidine

Front 114

Monoblasts have what enzyme in their granulues, and thus stain positive with what?

Back 114

Nonspecific esterase positive

Stains with alpha-napthyl butyrate

May or may not have MPO as well

Front 115

Define myelodysplastic syndrome

Back 115

Maturation defects associated with INEFFECTIVE HEMATOPOIESIS

Front 116

What age group does primary myelodysplastic syndrome usually occur in?

Back 116

The elderly (> 60 years)

Front 117

Describe the bone marrow in MDS

Back 117

Hypercellular

(erythrocytosis, granulocytosis, megakaryocytosis, myeloblastosis)

Front 118

In which myeloid disorder will you see a massive splenomegaly?

Back 118

Myeloproliferative neoplasms

Front 119

Define myeloproliferative neoplasms

Back 119

Proliferation of one or more of the myeloid lineages

Front 120

What are 5 types of MPN?

Back 120

1. CML
2. Polycythemia vera
3. Essential thrombocytosis
4. Primary myelofibrosis
5. Mastocytosis

Front 121

Describe the bone marrow in MPN

Back 121

Hypercellular with EFFECTIVE hematopoiesis

Front 122

What two genes are disrupted in AML? What are the genetic changes that do this?

Back 122

Disruption of CBF1alpha and CBF1beta

Caused by t(8;21) and inv(16), resp.

Front 123

What are the genetic changes associated with:
1) CML
2) Polycythemia vera
3) Essential thrombocythemia
4) Myelofibrosis
5) Mastocytosis

Back 123

1) BCR-ABL fusion
2) JAK2
3) JAK3, MPL
4) JAK2, MPL
5) c-KIT

Front 124

What are 3 good prognostic cytogenic abnormalities in AML?

Back 124

t(8;21)
inv(16)
t(15;17)

Front 125

What are 3 intermediate prognostic cytogenic abnormalities in AML?

Back 125

t(9;11)
Negative Y
Trisomy of 8 or 21

Front 126

What are 3 poor prognostic cytogenic abnormalities in AML?

Back 126

Deletion of 5 or 7
t(6;)
inv(3)

Front 127

Which type of AML can be treated with retinoic acid? How does it treat it?

Back 127

t(15;17)

It causes the neoplastic promyelocytes to differentiate into neutrophils

It will stop the bleeding diathesis associated w/ t(15;17)

Front 128

Infiltration of skin and gums in AML is seen with differentiation of which cell type?

Back 128

Monocytes

Front 129

What complication is seen in AML where the WBC count is > 100000?

Back 129

Pulmonary leukostasis

Front 130

What 3 characteristics are the WHO Classification for AML based on?

Back 130

1) Patient history
2) Cytogenetics/molecular genetics
3) Morphology

Front 131

How do you differentiate AML from a leukemoid reaction?

Back 131

Will see an elevated leukocyte alkaline phosphatase (LAP) score in leukemoid reaction

Front 132

Aside from acute vs chronic, how can differentiate b/w AML and CML?

Back 132

CML has the Philadelphia chromosome!!

Front 133

What is different about CML from the other MPNs, in regards to origin?

Back 133

CML --> arises in pluripotent stem cells

MPN --> arise in multipotential myeloid stem cells

Front 134

Describe the erythropoietin levels in primary, relative, and secondary Polycythemia Vera

Back 134

Primary --> low
Relative --> normal
Secondary --> high

Front 135

In what age group does polycythemia vera occur?

Back 135

Late middle age

Front 136

What is the age of onset of Hodgkin lymphoma, compared to non-Hodgkin lymphoma?

Back 136

Younger age of onset!

15 to 40 vs 40 to 65

Front 137

Which Hodgkin lymphoma occurs in the younger age groups?

Back 137

Nodular lymphocyte prodominant Hodgkin Lymphoma

Front 138

What is the characteristic cell in NLP Hodgkin Lymphoma?

Back 138

L&H cell, i.e. the "popcorn cell"

Front 139

NLP Hodgkin Lymphoma actually behaves like what malignancy?

Back 139

A low grade B-cell lymphoma

Front 140

What is the immunophenotype for NLP Hodgkin Lymphoma?

Back 140

CD20 and CD45

Front 141

What is the immunophenotype for Reed-Sternberg cells?

Back 141

CD30 and CD15

Front 142

Which Hodgkin lymphoma has the most favorable prognosis?

Back 142

NLPHL

Front 143

What is the second most common classic Hodgkin lymphoma?

Back 143

Mixed cellularity type

Front 144

Plasma cell myeloma usually occurs in what age group?

Back 144

The elderly (mean age of 70 years old)

Front 145

What is the most common immunologic subtype of multiple myeloma?

Back 145

IgG

Front 146

What is the immunologic subtype of Waldenstrom macroglobulinemia?

Back 146

IgM

Front 147

How can you differentiate multiple myeloma from Waldenstrom macroglobulinemia based on tissue involvement?

Back 147

Multiple myeloma has renal involvement (Bence-Jones protein)

Waldenstrom does NOT!

Front 148

Is Waldenstrom macroglobulinemia an aggressive or indolent disease?

Back 148

Indolent