Hematology Exam 2

Front 1

What are the 4 categories of leukemia?

Back 1

AML- Acute Myeloid L
ALL- Acute Lymphoblastic L
CML- Chrocic myelosytic L
CLL- Chronic Lymphocytic L

Front 2

In this leukemia, the spleen becomes the site of extra medullary hematopoeisis.

Back 2

AML

Front 3

Peripheral blood of this leukemia contains:
Blasts
Auer rods,
giant platelets
nRBCs and
Hypersegmented Segs

Back 3

AML

Front 4

To diagnose this Leukemia you need:
-200 WBC count
-Observable Hypercellular BM
- 20% or > myeloblasts

Back 4

AML

Front 5

True or False?

There are more blasts present in Chronic Leukemia than Acute Leukemia?

Back 5

False

Front 6

This cytochemical stain stains primary granules containing peroxidase and is a good indicator of AML?

Back 6

Myeloperoxidase (MPO)

Front 7

This test differentiates AML from ALL and is a strong positive test for myeloblasts.

Back 7

Myeloperoxidase (MPO)

Front 8

This cytochemical stain:
- stains phospholipids and other lipids found in primary and secondary granules of Myeloid cells.
-Negative in Lymphs
-Differentiates ALL and AML
-Sensitive for granulocyte precursor

Back 8

Sudan Black B (SBB)

Front 9

This cytochemical stain:
- stains primary granules of myeloid cells
- myeloblasts are strong positive
-neutrophils, basophils, mast cells stain pos
- eos, monos, and lymphs stain neg

Back 9

Specific Esterase (napthol AS-D chloroacetate esterase)

Front 10

This cytochemical stain:
-strong pos for monocytes
-negative reation w/ granulocytes
-used to ID monoblasts and monocytes in acute myeloblastic anemia

Back 10

Nonspecific esterase

Front 11

This cytochemical stain:
-stains an intracellular enzyme found in stem cells and immature T and B cells in BM
- indicator of Lymphoblastic leukemias

Back 11

Terminal deoxynucleotidyl transferase (TdT)

Front 12

This cytochemical stain:
- stains T lymphoblasts

Back 12

Acid phosphatase

Front 13

This cytochemical stain:
- stains B lymphocytes in hairy cell leukemia

Back 13

Tartrate resistant acid phosphatase

Front 14

This cytochemical stain:
- Stains glycoproteins and glycogen
- ALL exhibits "block staining"

Back 14

Periodic Acid-Schiff (PAS)

Front 15

This AML portrays
-Pseudo Chediak Higashi graunles
-Pseudo Pelger Huet
- Auer Rods
-good long term prognosis

Back 15

AML w/ t(8;21) (q22;q22)

Front 16

This AML portrays:
-Various stages of Monos, Eos, and other grans
- high complete remission rate
-NSE strong pos on Monos and Promonos

Back 16

AML W/ inv(16)(p13q22)

Front 17

This AML portrays:
- Auer rods
- * procoagulant/ fibrinolytic activity leads to DIC *
- hypo/hypergranular varients
-

Back 17

Acute promyelocytic leukemia w/ t(15;17)(q22;q21)

Front 18

This AML portrays:
- mainly in adults
- dyplasia in at least 2 cell lines
- a myelodysplastic syndrome
- a poor prognosis

Back 18

AML with Myelodysplasia

Front 19

This AML M1 (not otherwise categorized) portrays:
- marrow replaced with blasts
- CD 13, 33, 117
- MPO and SBB neg
- No Auer rods
- 5% of AML cases

Back 19

AML minimally differentiated

Front 20

This AML (not otherwise categorized) portrays:
- similar to minimally diff. AML except that at least 5% of blasts are MPO and SBB pos.
- Auer rods me also be present.
- 10% of AML cases

Back 20

AML without maturation

Front 21

This AML (not otherwise categorized) portrays:
- 30-45% of all AML cases
- as in all AMLs, 20% if BM cells are blasts, but also > 10% of segs are beyond the pro stage
- monocytic component is <20% of nonerythroid cells
- 50% of blasts are MPO and SBB pos
- Auer rods and blasts occur frequently

Back 21

AML with maturation

Front 22

This AML (not otherwise categorized) portrays:
- a mixture of malignant cells w/ both myelocytic and monocytic features
- contains monoblasts
- NSE strong pos
- Specific esterase is also pos
- monoblasts are MPO and SBB neg

Back 22

Acute Myelomonocytic leukemia

Front 23

This AML (not otherwise categorized) portrays:
- > 20% of BM cells are blasts w/ >80% of monocytic origin
- monoblasts are predominant but also promonocytes and monocytes

Back 23

Acute Monoblastic and Acute Monocytic leukemia

Front 24

True or False?

Acute monocytic leukemia is more common in adults, and Acute monoblastic leukemia is more common in kids.

Back 24

True

Front 25

The hallmark feature for this AML is Extramedullary dz charasterized by :
- Gum infiltration
- CNS dz
- a poor prognosis

Back 25

Acute Monocytic/blastic leukemia

Front 26

This AML (not otherwise categorized) portrays:
- usually affects Pts 50 years old or older
- more than 50% of BM cells are erythroid precursors and 30% are monoblsts.

Back 26

Erythroleukemia

Front 27

This AML (not otherwise categorized) portrays:
- usually affects Pts 50 years old or older
- >80% of BM cells are erythroid precursors
- Iron stain shows ringed sideroblasts
- Myeloblasts are MPO and SBB pos
- PSA stain shows "block" pattern in promonoblasts

Back 27

Pure erythroid leukemia

Front 28

The affects of this AML are:

- ineffective erythropoiesis
- high NRBC count in peripheral blood

Back 28

Acute Erythroid Leukemia

Front 29

This AML (not otherwise categorized) portrays:
- rarest form of AML
- occurs in children and adults
- megakaryoblasts
- diagnosis based on immunophenotyping

Back 29

Acute megakaryoblastic Leukemia

Front 30

This acute leukemia is characterized by:
- rare occurrence
- pancytopenia
- BM hyperplasia
- splenomegaly usually absent
- * BM is so packed, marrow tap results a "dry tap"

Back 30

Acute Myelofibrosis

Front 31

This Leukemia is:
- * Predominantly in children *
- a mutation of lymphoid precursor cells

Back 31

Acute Lymphoblastic Leukemia

Front 32

True or False?

L2 class ALL is more likely to occur in children and L1 class ALL is more likely to occur in adults.

Back 32

False

Front 33

In this Leukemia:
- B-lineage lymphoblasts are predominant in BM
- cells are uniformly TdT pos, HLA-DR pos, and CD10 pos

Back 33

Precursor B Leukemia

Front 34

In this leukemia:
- T-lineage lymphoblasts are predominant in BM
- TdT pos
- CD 3 and 7

Back 34

Precursor T Leukemia

Front 35

What are the 4 Chronic Myeloproliferative Disorders (CMPDs)?

Back 35

1. Chronic myelogenous leukemia (CML)
2. Myelofibrosis with myeloid metaplasia (MMM)
3. Polycythemia Vera (PV)
4. Essential thrombocythemia (ET)

Front 36

True or False?

Leukocyte alkaline phosphatase (LAP) can be high in all CMPDs except CML.

Back 36

True

Front 37

This CMPD:
- 90-95% of Pts have Philadelphia chromosome 9:22 translocation (Ph1)
- results in coding for p210, which increases tyrosine kinase activity, leading to suppressed apoptosis, excess cell production

Back 37

Chronic Myelogenous Leukemia

Front 38

True or False?

One characteristic of CML is that it is a normocytic, normochromic anemia.

Back 38

True

Front 39

Diagnosis of this CMPD consists of FISH test to detect BCR/ABL, or cytogenetics for Ph1 chromosome.

Back 39

CML

Front 40

Treatment of this CMPD includes:
- myelosupressive therapy
- leukaphoresis
- Tyrosine kinase inhibitor

Back 40

CML

Front 41

How do you tell the difference between a leukamoid reaction and CML?

Back 41

- Leukamoid does not contain PH1
- Eos and Basos are increased in CML, but not leukamoid
- LAP is decreased in CML but increased in leukamoid

Front 42

This CMPD:
- usually occurs in ages 60-70
- BM is hypercellular w/ hyperplasia
- clonal disorder with over-production of RBCs, WBCs, and platelets
- *RBCs are much more sensitive to EPO than usual
- increased blood viscosity
- * 90% have JAK 2 mutation *

Back 42

Polycythemia Vera (PV)

Front 43

In this CMPD:
- The most significant findings are increased RCM, splenomegaly, and JAK 2.

Back 43

PV

Front 44

The major cause of death in untreated Pts with this CMPD is hemorrhage and thrmbosis.

Back 44

PV

Front 45

This rare CMPD:
- is characterized by marked thrombosytosis and abnormal platelet function.
- increased PLT production due to hypersensitive megakaryocytes cytokines and TPO

Back 45

Essential Thrombocythemia (ET)

Front 46

Characteristics of this rare CMPD include:
- Hgb count no higher than 13 g/dL
- absence of BM fibrosis (no tear cells)
- presence of stainable iron in BM

Back 46

ET

Front 47

Thrombosis in microvasculature resulting in erythromelagia of extremities is indication of.......

Back 47

ET

Front 48

Factors that cause "reactive" type of this CMPD include:
- acute hemorrhage
- post splenectomy
- post operative
- inflammation
- hemolytic anemia

Back 48

ET

Front 49

The goal of treatment for this CMPD is to prevent vaso-occlusion and hemorrhage. Usually by prescribing aspirin, platelet phoresis, myelosuppressive drugs.

Back 49

ET

Front 50

PLT is >600 x 10^9/L in this CMPD.

Back 50

ET

Front 51

Platelet count needs to be under what value to successfully prevent the effects of ET?

Back 51

< 400 x 10^9/L PLT

Front 52

This CMPD:
- is characterized by BM fibrosis, proliferation of megakaryocytes and granulocytes, and EM hematopoiesis
- normal hematopoiesis is blocked as BM becomes more * fibrotic *
- results in EM hematopoiesis and splenomegaly

Back 52

Myelofibrosis with Myeloid Metaplasia

Front 53

Many tear drop RBCs can be observed in this CMPD. (They are due to splenic and BM fibrosis). Dwarf megakaryocytes, and giant platelets are also observed.

Back 53

Myelofibrosis with Myeloid Metaplasia

Front 54

In this CMPD,
- Hgb < 10mg/dL
- MB draw usually results in a dry tap

Back 54

MMM

Front 55

Treatment of this CMPD is hydroxyurea to control:
- leukocytosis
- thrombocytosis
- organomegaly

Back 55

MMM

Front 56

True or False?

Lymphoma originates in the BM and is noted in the general circulation

Back 56

False, Lymphoma originates in the lymphatic system.

Front 57

True or False?

Leukemia originates in the lymphatic tissue.

Back 57

False, Leukemia originates in the BM.

Front 58

What are the 7 Lymphoproliferative Disorders (LPDs)?

Back 58

- Chronic Lymphocytic Leukemia (CLL)
- Hairy Cell Leukemia
- Sezary Syndrome
- Prolymphocytic Leukemia
- Hodgkin's/ Non-Hodgkin's Lymphoma
- Multiple Myeloma
- Waldenstrom's Macroglobulinemia

Front 59

This LPD is caused by a clonal proliferation of B cells and exhibits exclusively small lymphocytes and smudge cells on a smear and a WBC count >100,000 x 10^9/L

Back 59

Chronic Lymphoproliferative Leukemia

Front 60

This LPD is characterized by fragile mononuclear cells with hair-like projections of the cytoplasm and are diagnostic with the TRAP test. (CD 19, 20, 22, 11c, 25, 103)

Back 60

Hairy Cell Leukemia

Front 61

Symptoms of this LPD include a abdominal pain ( indicative of a massive spleen), Thrombocytopenia, Pancytopenia, and dry tap and treated by therapeutic splenectomy.

Back 61

Hairy Cell Leukemia

Front 62

This LPD is considered the leukemic phase of cutaneous T cell lymphoma.

Back 62

Sezary cells

Front 63

Sezary cells are pathognomonic of what lymphoma?

Back 63

Cutaneous T cell Lymphoma

Front 64

This rare LPD is a variant of CLL and has a majority of circulating prolymphocytes. Also has a strong CD 20 and Sig activity.

Back 64

Prolymphocytic Leukemia

Front 65

This LPD is the most common lymphoma in young males and also seen in people over 50.

Back 65

Hodgkin's Lyphoma

Front 66

The presence of large, multi-nucleated cells that resemble and owls eye are called ___________ cells and are indicative of ______________?

Back 66

Reed-Sternberg cells

Hodgkin's Lymphoma

Front 67

This LPD is 3x more common than Hodgkin's Lymphoma and may present as painless cervical lymphnode involvement.

Back 67

Non-Hodgkin's Lymphoma

Front 68

This LPD is a disorder of the plasma cells, and causes a spike in the gamma region on a EP test.

Back 68

Multiple Myeloma (MM)

Front 69

In this LPD plasma cells are accelerated by cytokine IL-6 and contain colorless inclusions called Russell bodies or other crystalline inclusions.

Back 69

Multiple Myeloma (MM)

Front 70

Flame cells are present in the IgA version of this LPD.

Back 70

Multiple Myeloma

Front 71

In this LPD serum calcium levels are elevated due to increased osteoclast activity (bone break down) and may lead to kidney failure/ stones.

Back 71

Multiple Myeloma

Front 72

Monoclonal gammopathy, serum free light chains, Rouleaux formation, increased ESR, and Bence-Jones Proteins are all signs of which LPD?

Back 72

Multiple Myeloma

Front 73

What is the treatment for Multiple Myeloma?

Back 73

Chemo/ radiation
Thalidomide

Front 74

What complication of Multiple Myeloma occurs in late Dz state and is characterized by increased plasma cells in circulating blood?

Back 74

Plasma Cell Leukemia

Front 75

This LPD is characterized by an overproduction of IgM from abnormal B cells.

Back 75

Waldenstrom's Macroglobulinemia

Front 76

True or False:

In Waldenstrom's Macroglobulinemia, IgM interferes with platelets by causing a platelet satallitism.

Back 76

False, The IgM impedes PLT function by coating the platelets.

Front 77

Hyperviscosity syndrome, or the slow flow of blood that causes CNS symptoms, is an indicator of of which LPD?

Back 77

Waldenstrom's Macroglobulinemia

Front 78

Cryoglobulins may be found in which LPD, and lead to what phenomenon characterized by cold hands and feet?

Back 78

Waldenstrom's Macroglobulinemia

Raynaud's Phenomenon

Front 79

This Glycoprotein is made in the liver to transport iron in the serum.

Back 79

Transferrin

Front 80

This iron is the storage form of iron in the body, found mostly in the RES.

Back 80

Ferritin

Front 81

What are the 3 broken-down components of Hemoglobin?

Back 81

-Globin
- Iron
- Protoporphyrin

Front 82

What are the 3 routes that lead to Anemia?

Back 82

1. Dec. production of RBC or Hgb
2. Inc. destruction of RBC or Hgb
3. Loss of RBCs

Front 83

True or False?

1/3 of iron in the body is found in Hgb and the other 2/3 is "tissue iron".

Back 83

False, 2/3 is in Hgb and 1/3 is "tissue iron".

Front 84

What is hemosiderin?

Back 84

It is precipitated ferritin found primarily in the liver. Can be seen with heatoxylin-Eosin or Prussian Blue stain.

Front 85

True or False?

100% of iron from RBCs is recycled.

Back 85

True

Front 86

Describe how dietary iron gets converted to ferritin in the body.

Back 86

1. Ingested as Fe3+,
2. converted to Fe2+ by stomach acid
3. Fe2+ absorbed in duodenum and oxidized back to Fe3+.
4. Fe3+ combines with apoferritin to for ferritin

Front 87

What are the three stages of iron deficiency?

Back 87

1. Depletion of iron stores.
2. Iron-deficient erythropoiesis
3. Iron-deficiency anemia

Front 88

What are some causes of Iron deficiency anemia?

Back 88

- nutritional deficiency
- gastrointestinal bleeding
- other kinds of hemorrhage
- menorrhagia
-hemolysis

Front 89

This phenomenon, known as Koilonychia, is a common symptom of what microcytic anemia?

Back 89

Iron deficiency anemia (IDA)

Front 90

Delta aminolevulinic acid + EPO + vit. B6 lead to the production of what?

Back 90

Protoporphyrin

Front 91

In inherited sideroblastic anemia, because Pts are aminolevulinic synthetase deficient, they cannot create what important element of RBCs?

Back 91

Protoporphyrin

Front 92

What is the word for a clinical disorder that results in tissue damage from excess iron?

Back 92

Hemachromatosis

Front 93

True or False?

Gene mutations are the reason for the chromosomal abnormalities of Alpha Thalassemia.

Back 93

False, gene deletions are the cause.

Front 94

What subclass of Alpha Thalassemia has no alpha globin chains present?

Back 94

Barts hydrops fetalis

Front 95

Why is Beta Thalassemia not recognizable in an infant until they are older?

Back 95

B/c the absent beta chains do not affect Hgb F, which is the predominant Hgb for infants. It is only when the Hgb F levels decrease and the Hgb A levels increase that you would observe affects.

Front 96

Describe the concept of the osmotic fragility test.

Back 96

RBCs in thalassemia have a decreased osmotic fragility because of the lack a certain globin chain. When normal RBCs are put in a hypotonic solution, fluid follows the concentration gradient into the RBC to the point of cell lysis. Because cells in thalassemia have more cell surface area, they lyse at a much more hypotonic solution than normal RBCs.

Front 97

True or False?

Hgb H dz has 2 functional alpha chains.

Back 97

False, Hgb H only has 1 functional alpha chain.

Front 98

True or False?

Beta Thal it a homozygous disorder?

Back 98

True

Front 99

True or False?

The reason for the genetic abnormalities in Beta Thal are from gene mutations.

Back 99

True

Front 100

In what microcytic anemia do Pts compensate by producing 60-95% of Hgb F

Back 100

Beta Thalassemia

Front 101

What is the risk factor for transfusion in a Pt with Beta Thal?

Back 101

It could lead to iron overload.

Front 102

True or False?

Hgb levels are very high in Beta Thal?

Back 102

False, Hgb levels are around 6-9 g/dL

Front 103

What test is used to screen for Hgb F?

Back 103

Acid Elution Test (Kleihauer and Betke stain), Hgb F picks up Eosin stain and is observed.

Front 104

What dz is the acid elusion test used to identify?

Back 104

Hereditary Persistence of Fetal Hgb (HPFH),

Front 105

What microcytic anemia is characteristic of these "golf ball" cells?

Back 105

Hgb H syndrome (1 functional alpha chain)

Front 106

Which hemoglobins are fetal?

Back 106

1. Hgb Gower I
2. Hgb Gower II
3. Hgb Portland

Hint 106

there are 3 of them.