Br Pathology: White Blood Cell Disorders

Front 1

What is Chediak-Higashi syndrome?

Back 1

a rare autosomal recessive disorder that affects multiple systems of the body, and arises from a mutation in the lysosomal trafficking regulator gene, LYST. Phagolysome does not form

Front 2

What type of bacterial infection is common in someone with a WBC disorder but not a normal person?

Back 2

coagulase negative staph

Front 3

Do hot or cold abscesses form in someone with white blood cell disorders? What is a sign in the oral cavity of someone with WBC disorder?

Back 3

1) cold because the immune system is deficient
2) gingivitis

Front 4

What is Jobs syndrome? What are the features of people that get it and what type of abscess is present?

Back 4

autosomal recessive disorder of neutrophils, characterized by abnormal chemotaxis leading to "cold" soft tissue abscesses due to Staphylococcus aureus. Patients have red hair, a leonine face, chronic eczema, and increased IgE (hyperimmune E syndrome).

Front 5

On a molecular level what is going on in Job's syndrome?

Back 5

defect in chemotaxis; ↑ IgE

Front 6

How is a leukomoid reaction characterized molecularly?

Back 6

a.Absolute leukocyte count usually >50,000 cells/mm3

Absolute count = % leukocytes × total WBC count

■May involve neutrophils, lymphocytes, or eosinophils

Front 7

What are few causes of leukomoid reaction? Is it benign or malignant?

Back 7

(1) Perforated appendicitis (neutrophils)
(2) Whooping cough (lymphocytes)
(3) Cutaneous larva migrans (eosinophils)

Leukemoid reaction is benign, exaggerated leukocyte response to infection

Front 8

Who does leukoerythroblastic occur in? What is due to?

Back 8

woman > 50 years of age: usually due to metastatic breast cancer. May also be due to fibrosis

Front 9

leukoerythroblastic is characterized how on blood smear?

Back 9

(1) Myeloblasts, progranulocytes
(2) Nucleated RBCs, tear drop RBCs (if fibrosis is present)
Note: presence of immature cells

Front 10

When does neutrophilic leukocytosis occur?

Back 10

Neutrophilic leukocytosis: neutrophil count > 7000 cells/mm

Front 11

Neutropenia occurs at what level?

Back 11

neutrophil count < 1500 cells/mm

Front 12

What are few causes of neutrophilic leukocytosis?

Back 12

1) corticosteriods, catecholamines, lithium
2) decreased activation of neutrophil adhesion molecules
3) appendicitis
4) bacterial infection (staph)

Front 13

What can cause neutropenia?

Back 13

less than 1500
1) Aplastic anemia
(2) Immune destruction
■systemic lupus erythematosus (SLE)
(3) Septic shock
Note that endotoxin increases adhesion molecules for neutrophils to migrate out of blood making their concentration lower

Front 14

When does eosinophilia occur?

Back 14

eosinophil count > 700 cells/mm3

Front 15

What are causes of eosinophilia?

Back 15

■(1) Type I hypersensitivity reaction
■Examples-bronchial asthma, reaction to penicillin, hay fever
■(2) Invasive helminthic infection
■(a) Examples-strongyloidiasis, hookworm infection
■(b) Pinworms and adult ascariasis do not have eosinophilia (noninvasive).
■(3) Polyarteritis nodosa (type III)
■(4) Addison's disease (cortisol deficiency)

Front 16

will strongyloides or pinworms produce eosinophilia? What about adult ascariasis? What about hookworms?

Back 16

strongyloides hook worms because pinworms and adult ascariasis are non-invasive

Front 17

What is polyarteritis nodosa?

Back 17

is a vasculitis of medium-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease. type III hypersensitivity.

Front 18

At what level is basophilia seen? When is it seen?

Back 18

>110 cell/mm3

◦Chronic myeloproliferative disorders (e.g., polycythemia vera, ET, CML, MMM)

Front 19

When does lymphocytosis occur?

Back 19

lymphocyte count > 4000 cells/mm3 (adult); >8000 cells/mm3 (child)

Front 20

What are bacterial and viral causes of lymphoscytosis?

Back 20

1) CMV and EBV
2) whooping cough (B pertusis)

Front 21

What drug can induce lymphocytosis? What autoimmune condition can?

Back 21

1) phenytoin
2) graves

Front 22

What organisms can induce atypical lymphocytosis? What drug?

Back 22

(1) Infection
■Examples-mononucleosis, viral hepatitis, CMV infection, toxoplasmosis
(2) Drugs (e.g., phenytoin)

Front 23

How are atypical lymphocytes characterized?

Back 23

1) Antigenically stimulated lymphocytes (CD8)
2) Prominent nucleoli and abundant blue cytoplasm

Front 24

Where does EBV begin and where does it progress? What is end result?

Back 24

1) EBV initially replicates epithelial cells in oropharynx.
2) Infection spreads to B cells in lymph nodes
(a) Attaches to CD21 receptors on B cells.
(b) Causes B-cell proliferation and increased synthesis of IgM antibodies
(c) Virus remains dormant in B cells.

Recurrences may occur.

Front 25

Someone with a tonsilar exudate has hepatosplenomegaly, fatigue, generalized painful lymphadenopathy, is started on ampicillin and quickly develops a rash. What do they have?

Back 25

EBV

Front 26

EBV develops a rash. Why?

Back 26

drug reaction to ampicillin

Front 27

What are common lab findings in someone with EBV? specifically what are the tests done, what are they for, etc....?

Back 27

1) Atypical lymphocytosis
(a) Usually more than 20% of the total WBC count
(b) Atypical lymphocytes are antigenically stimulated CD8 cells
2) Positive heterophil antibody test
(a) Initial screening test
(b) Detects IgM antibodies against horse (most common), sheep, and bovine RBCs
*Heterophile antibodies: IgM antibodies directed against horse, sheep, bovine
(c) Sensitivity 87%, specificity 91%
(3) Antiviral capsid antigen (VCA) antibodies

RBCs Anti-VCA-IgG/IgM: excellent test if screening test is negative
(a) High sensitivity and specificity
(b) Develops early in the infection
(c) Persists for life

(4) Anti-early antigen (EA) antibodies
■Increased with chronic infections
(5) Anti-Epstein Barr nuclear antigen (EBNA) antibodies
(a) High sensitivity and specificity
(b) Develops late in the infection
(c) Persists for life
(6) Increased serum transaminases from hepatitis
■Jaundice is rare.

Front 28

When does lymphopenia occur?

Back 28

lymphocyte count < 1500 cells/mm3 (adult); <3000 cells/mm3 (child)

Front 29

List conditions where lymphopenia occurs?

Back 29

(1) HIV
(2) Immunodeficiency
Lymphopenia in HIV: lysis of CD4 helper T cells by the virus
(a) DiGeorge syndrome (T-cell deficiency)
(b) Severe combined immunodeficiency (B- and T-cell deficiency)
(3) Immune destruction (e.g., SLE)
(4) Corticosteroids (apoptosis)
Corticosteroids produce neutrophilic leukocytosis, eosinopenia, and lymphopenia.
(5) Radiation
■Lymphocytes most sensitive cells to destruction by radiation

Front 30

what white blood cell is most susceptible to radiation?

Back 30

lymphocytes

Front 31

When does monocytosis occur? (level)

Back 31

> 800 cells/mm3

Front 32

What is the eitiology of monocytosis?

Back 32

Chronic infection
■Examples-tuberculosis, subacute infective endocarditis

Autoimmune disease
■Examples-rheumatoid arthritis, cirrhosis

Malignancy
■Examples-carcinoma, malignant lymphoma

Front 33

Is leukemia more common in men or women?

Back 33

men

Front 34

List several risk factors for developing leukemia?

Back 34

a.Chromosomal abnormalities
■Examples-Down syndrome, chromosome instability syndromes
b.Ionizing radiation
■Example-nuclear plant explosion
c.Chemicals
■Example-benzene for myeloid leukemia
d.Alkylating agents
■Particularly busulfan
e.Chronic myeloproliferative diseases
■Example-polycythemia vera
f.Paroxysmal nocturnal hemoglobinuria
g.Cigarette smoking
h.Immunodeficiency diseases
■Example-Wiskott-Aldrich syndrome

Front 35

The use of busulfan puts a person at risk for?

Back 35

leukemia

Front 36

benzenes put a person at risk for developing what?

Back 36

myeloid leukemia

Front 37

What does Paroxysmal nocturnal hemoglobinuria put someone at risk for?

Back 37

leukemia

Front 38

Is leukemia more common in adults or children?

Back 38

adults

Front 39

In children what is the most common cancer?

Back 39

acute lymphoblastic leukemia

Front 40

In an adult aged 15-60 what is the most common leukemia?

Back 40

AML

Front 41

What is the most common leukemia in the 40-60 year old group? Second most?

Back 41

AML then CML

Front 42

What is the most common leukemia after 60?

Back 42

CLL

Front 43

In acute or chronic leukemia does a block in the cell proliferation cycle occur early?

Back 43

acute is early and chronic is in later stages of cell development. Note there is some maturation in chronic leukemia

Front 44

Where does leukemia arise?

Back 44

in bone marrow

Front 45

What are the clinical findings in acute leukemia?

Back 45

1.Abrupt onset of signs and symptoms
a.Fever usually from infection
b.Bleeding from thrombocytopenia
c.Fatigue from anemia

2.Metastatic disease
a.Hepatosplenomegaly
b.Generalized painless lymphadenopathy
c.Central nervous system (CNS) involvement
d.Skin involvement: T cell leukemias
■Especially in ALL

CNS, testicle involvement: ALL
■Especially T-cell leukemias

e.Testicles
■Especially in ALL

4.Bone pain and tenderness
◦Due to bone marrow expansion by leukemic cells

Front 46

What will a peripheral white cell count show in someone with acute leukemia?

Back 46

a.Below 10,000 cells/mm3 (normal) to >100,000 cells/mm3
b.Blast cells usually present
■Examples-myeloblasts, lymphoblasts, monoblasts

Front 47

What type of anemia is present in acute leukemia?

Back 47

Normocytic to macrocytic anemia
◦Macrocytic if folate is depleted in production of leukemic cells

Front 48

Bone marrow findings in acute leukemia show what?

Back 48

key finding of blasts >20% in bone marrow

Front 49

What are common clinical findings in someone with chronic leukemia?

Back 49

a.Insidious onset
b.Slightly more common than acute leukemia
c.Hepatosplenomegaly
d.Generalized painless lymphadenopathy

Front 50

How would a peripheral white cell count taken from someone with chronic leukemia differ from someone with acute leukemia? What other tests distinguish the two?

Back 50

1)they would be very similar, i.e. Below 10,000 cells/mm3 (normal) to >100,000
2) in CL the bone marrow is hypercellular with <10% blasts and a blood smear shows some maturation. Both are normocytic to macrocytic depending on the folate concentration

Front 51

Someone is suspected of having CL. What will their platelet count be?

Back 51

could be high (40%) or low (60%). This distinguishes it from AL where they are always low

Front 52

What leukemia has the worst 5 year survival rate?

Back 52

1.Acute lymphoblastic leukemia: 87% 5-year survival rate
2.Acute myelogenous leukemia: 21% 5-year survival rate
3.Chronic lymphocytic leukemia: 75% 5-year survival rate
4.Chronic myelogenous leukemia: 89% 5-year survival rate

Front 53

Myeloid disorders are?

Back 53

neoplastic stem cell disorders
◦May involve one or more stem cell lines

Front 54

What are the 3 types of myeloid disorders?

Back 54

a.Chronic myeloproliferative disorders
b.Myelodysplastic syndrome
c.Acute myeloblastic leukemia

Front 55

What is the most common chronic myeloproliferative disorder?

Back 55

polycythemia vera

Front 56

What are the chronic myeloproliferative disorders?

Back 56

a.Polycythemia vera
Polycythemia vera: most common chronic myeloproliferative disorder
b.Chronic myelogenous leukemia
c.Myeloid metaplasia with myelofibrosis
d.Essential thrombocythemia

Front 57

What can polycythemia vera lead to?

Back 57

a.Splenomegaly
b.Propensity for reactive bone marrow fibrosis ("spent phase")
c.Propensity for transformation to acute leukemia

Front 58

What is the RBC count equal to?

Back 58

RBC count = RBC mass/PV

Front 59

What is the most common polycythemia? What happens to the RBC count, PV, RBC mass, O2 sat, and EPO?

Back 59

Relative polycythemia: ↑ RBC count; ↓ PV; normal RBC mass, Sao2, EPO
Note that there is no increase in bone marrow production

Front 60

What is absolute polycythemia? What are the types?

Back 60

Increase in bone marrow production of RBCs
■Increased RBC count and RBC mass
1) appropriate and inappropriate

Front 61

What causes appropriate absolute polycythemia?

Back 61

(a) There is a hypoxic stimulus for EPO release.
(b) Examples-primary lung disease, cyanotic congenital heart disease, high altitude

Front 62

What are the blood work findings in someone with appropriate absolute polycythemia?

Back 62

↑ RBC mass, EPO; normal PV; ↓ Sao2; ↑ RBC count; Normal PV

Front 63

What causes inappropriate absolute polycythemia?

Back 63

Ectopic release of EPO from renal cell carcinoma

Front 64

What are the blood values in someone with ectopic inappropriate absolute polycythemia?

Back 64

↑ RBC mass, EPO; normal PV, normal Sao2
Increased RBC count

Front 65

How is relative polycythemia corrected?

Back 65

water replacement

Front 66

nonectopic inappropriate absolute polycythemia is also known as?

Back 66

polycythemia vera

Front 67

In polycythemia vera What is mutated?

Back 67

Clonal expansion via mutation in JAK2 gene on 9p

Front 68

The JAK2 gene on 9p is seen what disorders?

Back 68

polycythemia vera, myelofibrosis and myeloid metaplasia or essential thrombocythemia

Front 69

In polycythemia vera what is increased?

Back 69

Increased production of RBCs, granulocytes (neutrophils, eosinophils, basophils), mast cells, and platelets

Front 70

What are manifestations seen in polycythemia vera?

Back 70

(1) Hepatosplenomegaly
(2) Ruddy (plethoric) face
Due to vessel congestion
(3) Thrombotic events
(a) Due to hyperviscosity related to increased RBC count
(b) Examples-hepatic vein thrombosis, dural sinus thrombosis, retinal vein thrombosis
(4) Impaired CNS circulation
(a) Headache
(b) Blurred vision
(c) Retinal vein engorgement
(d) Vertigo
(e) Transient ischemic attack
(f) Stroke
(5) Signs of increased histamine released from mast cells
*pruritis is a sign after bathing, mast cell release of histamine
■Very common initial complaint; mast cells degranulate with change in skin temperature
(b) Peptic ulcer disease
■Histamine stimulates production of gastric acid.
(6) Gout
(a) Increased breakdown of nucleated cells with release of purines
(b) Purines are converted to uric acid.

Front 71

What are the lab findings in someone with polycythemia vera?

Back 71

↑ RBC mass, ↓ EPO; ↑ PV, normal Sao2,

Front 72

What are the major criteria to diagnose polycythemia vera? What are the minor criteria?

Back 72

1) Major criteria
(a) Increased RBC mass: >36 mL/kg, >32 mL/kg in women
(b) Normal Sao2 (>92%)
(c) Splenomegaly
(2) Minor criteria
(a) Absolute leukocytosis: >12,000 cells/mm3
(b) Thrombocytosis: >400,000 cells/mm3
(c) Increased serum leukocyte alkaline phosphatase: >100 score
(d) Increased serum vitamin B12: >900 pg/mL or vitamin B12 binding protein > 2200 pg/mL
(3) Hypercellular bone marrow with fibrosis in later stages

Front 73

How is polycythemia treated?

Back 73

phlebotomy, hydroxyurea (inhibits ribonucleotide reductase), interferon alpha

Front 74

What age groups are susceptible to CML? what are risk factors?

Back 74

40 and up

benzene and radiation exposure

Front 75

What is the pathogenesis behind CML?

Back 75

(1) Neoplastic clonal expansion of the pluripotential stem cell
■This stem cell has the capacity to differentiate into a lymphoid or myeloid stem cell.
(2) t9;22 translocation of ABL proto-oncogene
(a) Proto-oncogene fuses with the break cluster region (BCR) on chromosome 22 (BCR-ABL fusion gene).
(b) Chromosome 22 with translocation is called Philadelphia chromosome

Front 76

On physical exam of someone with CML what would you expect to find?

Back 76

Hepatosplenomegaly and generalized painless lymphadenopathy
■Due to metastasis

Front 77

At the cellular level in the final phase what characterizes CML? Are there Auer rods?

Back 77

CML blast crisis: myeloblasts or lymphoblasts; no Auer rods

Front 78

A CBC is done on a person with CML. What do you expect?

Back 78

(1) Peripheral WBC count 50,000 to 200,000 cells/mm3
(a) Myeloid series in all stages of development
(b) Basophilia
(2) Normocytic to macrocytic anemia
■Macrocytic if folate is depleted in the production of leukemic cells
3) Thrombocytosis in 40% to 50% of cases and thrombocytopenia in remaining

Front 79

On bone marrow biopsy A person has a blast count of less than 10%. Do they have CML or AML?

Back 79

CML

Front 80

What leukemias is the philidalphia chromosome in? How can the chromosome be lost from the body?

Back 80

ALL and CML

treat with interferon alpha

Front 81

What is the most sensitive and specific test for CML?

Back 81

BCR-ABL fusion gene

Front 82

What is decreased leukocyte alkaline phosphatase low in?

Back 82

CML and neoplastic granulocytes and present in benign granulocytes.

Front 83

How is CML treated and what is the 5 year prognosis?

Back 83

(1) Imatinib mesylate
■(a) Oral tyrosine kinase inhibitor
■(b) <35% Philadelphia chromosome t(9:22) positive cells after treatment
(2) Allogenic stem cell transplantation

Prognosis ∼90% 5-year survival rate

Front 84

When does MMM occur? What is the most common finding in this age group?

Back 84

1) Myelofibrosis and myeloid metaplasia Occurs in patients > 50 years old
(2) Most common cause of splenomegaly in this group

Front 85

What is the pathogenesis of MMM? What occurs?

Back 85

Most due to mutation of JAK2 gene on short arm of chromosome 9
■Same mutation may manifest as polycythemia vera or thrombocythemia.
(3) Ineffective erythropoiesis, dysplastic megakaryocytes (pawn cells), immature granulocytes, reactive myelofibrosis
■Marrow fibrosis occurs earlier than in the other chronic myeloproliferative diseases.
4) Hematopoiesis moves to the spleen, liver, and other sites (extramedullary hematopoiesis)

Front 86

What 3 things characterize MMM?

Back 86

marrow fibrosis; massive splenomegaly; extramedullary hematopoiesis

Front 87

A 55 year old female presents with massive splenomegaly, pleural effusion onl left from splenic infarcts and portal hypertension. What do they have?

Back 87

MMM

Front 88

What does the blood work of someone with MMM show? bone marrow show?

Back 88

1) Bone marrow fibrosis
(2) Peripheral WBC count 10,000 to 50,000 cells/mm3
(3) Normocytic anemia

Front 89

What does a blood smear in someone with MMM show?

Back 89

a) Tear drop cells (damaged RBCs)
b) Leukoerythroblastic reaction
c) Platelet count is variable.
■Platelets have abnormal morphology

Front 90

Is leukocyte alkaline phosphatase high or low in MMM? CML? polycythemia vera?

Back 90

1) normal to high
2) low
3) high

Front 91

How is MMM treated?

Back 91

(1) Hydroxyurea
(2) Interferon-α

Front 92

What is the pathogenesis in ET?

Back 92

dysplastic/nonfunctional platelets; ↑ platelets; mutation of JAK2 gene

Front 93

Some clinical findings in ET are?

Back 93

(1) Bleeding
(a) Usually gastrointestinal with concomitant iron deficiency
(b) Platelets nonfunctional
(2) Splenomegaly

Front 94

Blood work in a patient with ET would show?

Back 94

(1) Thrombocytosis
(a) Platelets > 600,000 cells/mm3;
(b) Platelet morphology is abnormal.
(2) Mild neutrophilic leukocytosis
(3) Basophilia
(4) Hypercellular bone marrow with abnormal megakaryocytes

Front 95

How is ET treated?

Back 95

hydroxyurea

Front 96

Who does MDS predominately occur in and what age group?

Back 96

◦Usually occurs in men between 50 and 80 years old

Front 97

How is MDS characterized? How is it classified?

Back 97

1) cytopenias; hypercellular marrow
2) Classification
■(a) Refractory anemia
■(b) Refractory anemia with ringed sideroblasts
■(c) Chronic myelomonocytic leukemia
■(d) Refractory anemia with excess blasts in transformation

Front 98

What does MDS occasionally transform into?

Back 98

AML

Front 99

What is seen in the blood work of someone with MDS? What is unique?

Back 99

Severe pancytopenia
(1) Normocytic to macrocytic anemia
■Dimorphic RBC population (microcytic and macrocytic)
(2) Leukoerythroblastic reaction
3) ringed sideroblasts

Front 100

Bone marrow biopsy of someone with MDS would show? What does it mean when 40% myeloblasts are seen?

Back 100

(1) Ringed sideroblasts (nucleated RBCs with excess iron)
(2) Myeloblasts < 20%
■If >20%, disease is progressing to AML.

Front 101

What age group is AML common in?

Back 101

Usually occurs between 15 and 59 years of age

Front 102

What is the chromosomal abnormality in acute promyelocytic leukemia (M3)

Back 102

t(15;17)

Front 103

How are myeloblasts characterized on microscopy in someone with AML? What stages are they present in?

Back 103

Splinter-shaped to rod-shaped structures in the cytosol of myeloblasts
■Auer rods are fused azurophilic granules

M2 and M3

Front 104

How is AML treated?

Back 104

a.Induction therapy: cytarabine + daunorubicin
b.Consolidation therapy: aggressive chemotherapy with or without radiation
c.Maintenance therapy: cytarabine

Front 105

What is seen in the M2 rating in AML? What age group is affected?

Back 105

Most common type (30-40% of cases). Auer rods present. 15-59-year-old age bracket

Front 106

What is seen in the M3 rating in AML? What can retinoic acid do in this stage?

Back 106

Numerous Auer rods. DIC is invariably present. t(15;17) translocation. Abnormal retinoic acid metabolism: high doses of all-trans-retinoic acid may induce remission by maturing cells

Front 107

ALL is most common in what age groups?

Back 107

a.Most common leukemia and cancer in children (newborn to 14 years of age)

Front 108

What are the subtypes of ALL and which is most common?

Back 108

(1) Early pre-B-cell ALL (80%)
(2) Pre-B-, B-, and T-cell ALL

Front 109

In early pre-B cell ALL what markers are present? What mutation has a favorable prognosis?

Back 109

1) a.Positive marker studies for common ALL antigen (CALLA, CD10)
2) Positive marker studies for terminal deoxynucleotidyl transferase (TdT)
3) t(12;21) offers favorable prognosis

Front 110

What are the markers in T-cell ALL?

Back 110

◦CD10 negative and TdT positive

Front 111

What are clinical findings in early pre-B cell ALL? What about T cell ALL?

Back 111

a.Metastatic sites similar to those of AML
b.B-cell types
■Commonly metastasize to the CNS and testicles
c.T-cell type
■Presents as anterior mediastinal mass or acute leukemia

Front 112

What is seen on blood work and bone marrow in someone with ALL?

Back 112

a.Peripheral WBC count 10,000 to 100,000 cells/mm3
■Over 20% lymphoblasts in peripheral blood
b.Normocytic anemia with thrombocytopenia
c.Bone marrow findings
■Bone marrow often totally replaced by lymphoblasts

Front 113

What is the induction, consolidation and maintanence therapy in ALL?

Back 113

a.Induction therapy: vincristine, prednisone, l-asparaginase
b.Consolidation therapy: aggressive chemotherapy with or without radiation
c.Maintenance therapy: methotrexate + 6-mercaptopurine

Front 114

What is adult T-cell leukemia associated with?

Back 114

Malignant leukemia associated with human T-cell leukemia virus (HTLV-1)

Front 115

How can adult T-cell leukemia present?

Back 115

malignant lymphoma

Front 116

What genetic abberation occurs in adult T cell leukemia? Which T cell type proliferates?

Back 116

Activation of TAX gene, which inhibits the TP53 suppressor gene

CD4 cells

Front 117

What are clinical findings in adult T cell leukemia?

Back 117

skin lesions; lytic bone lesions with hypercalcemia; a.Hepatosplenomegaly and generalized lymphadenopathy

Front 118

Why does hypercalcemia occur in adult T cell leukemia?

Back 118

1) Due to lymphoblast release of osteoclast-activating factor
2) Associated with hypercalcemia

Front 119

What is seen on blood exam in someone with adult T cell leukemia? What is seen in bone marrow?

Back 119

a.Peripheral WBC count 10,000 to 50,000 cells/mm3
(1) Over 20% lymphoblasts
(2) Positive CD4 marker study
(3) Negative for TdT
b.Normocytic anemia and thrombocytopenia
c.Bone marrow findings
■Replaced by CD4 lymphoblasts

Front 120

What is the most common leukemia? What age group is it most common in?

Back 120

CLL
a.Occurs in individuals > 60 years old

Front 121

What is the most common cause of generalized lymphadenopathy in those >60 years old?

Back 121

CLL

Front 122

What is the pathogenesis of CLL?

Back 122

◦Neoplastic disorder of virgin B cells (B cells that cannot differentiate into plasma cells)

Front 123

What are clinical findings in someone with CLL?

Back 123

a.Generalized lymphadenopathy
b.Metastatic sites similar to those of AML
c.Increased incidence of immune hemolytic anemia
■Both warm (IgG) and cold (IgM) types

Front 124

Is the WBC count high or low in CLL? What about lymphblasts?

Back 124

a.Peripheral WBC count 15,000 to 200,000 cells/mm3
b.Lymphoblasts < 10%

Front 125

What disease are smudge cells seen in? They are prone to bacterial infections. Why?

Back 125

1) CLL
2) hypogammaglobulinemia

Front 126

What are bone marrow findings in CLL?

Back 126

(1) Usually completely replaced by neoplastic B cells
(2) Lymphoblasts < 10%

Front 127

How is CLL treated?

Back 127

◦Chlorambucil is a nitrogen mustard alkylating agent

Front 128

What type of leukemia is hairy cell leukemia? Who is it most common in?

Back 128

B cell leukemia

◦Most common in middle-aged men

Front 129

Where is the primary site of cell replication in hairy cell leukemia?

Back 129

spleen primary site for neoplastic cells. 90% have splenomegaly

Front 130

What is the only leukemia that does not have lymphadenopathy?

Back 130

hairy cell leukemia

Front 131

What are common clinical findings in hairy cell leukemia?

Back 131

hepatosplenomegaly

Autoimmune vasculitis and arthritis

Front 132

What cells are deficient in hairy cell leukemia? What cell features characterize hairy cell leukemia?

Back 132

all of them pancytopenia

Leukemic cells have hair-like projections

Front 133

What leukemia is Positive tartrate-resistant acid phosphatase (TRAP) stain?

Back 133

hairy cell leukemia

Front 134

How is hairy cell leukemia treated?

Back 134

◦Drugs of choice are purine analogs; e.g., 2-chloro-2 deoxyadenosine