Leukemia

Question 1

what is the hallmark of acute leonic ukemia?

Answer 1

PANCYTOPENIA w/ circulating blasts

Question 2

What are the diff types of Leukemia?

Answer 2

• acute lymphoblastic (ALL)
• Acute Myeloid (AML)
• Chronic myeloid Leukemia

Question 3

who mostly get AML?

Answer 3

adulthood- median age of onset is 60 years

Question 4

ALL

Answer 4

75% of all new leukemia

• M>F, peaks at age 2-5 years
• more common in white

Question 5

What are the two types of ALL?

Answer 5

B-Lineage or T-linage

Question 6

B-Lineage?

Answer 6

Burkitt Leukemia (good prognosis)

Question 7

CF of ALL

Answer 7

• secondary to bone marrow failure or infiltration of leukemic cells into normal tissie
• fever
• pallor
• petechia or ecchymoses
• gingival bleed/epistaxis/menorrhagia
• lethargy/malaise
• anorexia
• bone or joint pain

Question 8

dx evaluations of ALL

Answer 8

confirmed w. blasts on peripheral smear, bone marrow aspirate or both

• lumbar puncture to evaluate for CNS involvemnt
• may see mediastinal

Question 9

what is the gold standard of ALL dx?

Answer 9

bone marrow bx and aspirate

Question 10

what are ALL pts in risk of?

Answer 10

tumor lysis syndomre

-hyperuriciemia, hyperphosphatemia, hyperkalemia

Question 11

tx of of ALL

Answer 11

chemo over 28 days

vincristine, steroids, intrathecal methotrexate, asparaginase

Question 12

AML

Answer 12

accounts for 15-20% of childhood leukemias

-relatively high in neonatal peroid

Question 13

Clinical findings of AML

Answer 13

similar to ALL

-may develop soft-tissue tumore called a chloroma in spinal cord, brain or on the skin

Question 14

dx of AML

Answer 14

bone marrow bx, cytogenetic studes,

-auer rods

Question 15

tx of AML

Answer 15

more intensive then ALL

• needs myelosuppressive chemotheraphy
• induction therapy include an anthracycline w. cytosine arabinoside

Question 16

Prognosis of AML

Answer 16

overall cure rate-50%

-better outcomes for pts who receive matched sibling stem cell transplant

Question 17

What is CML

Answer 17

clonal disorders of the pluripotent stem cell- characterized by excess of proliferation of the late progenitor or relatively mature myeloid compartments

“Too many white cells are made in the bone marrow—specifically myeloid cells”

Question 18

CML epidemilogy

Answer 18

o Usually occur in young to middle age adults (medium age 42). CML accounts for 7-20% cases of leukemia.

Question 19

CML PP

Answer 19

feedback loop is shut off and you just keep making more neutrophils

Question 20

what are the phases of CML

Answer 20

chronic, acclererated, and acute

Question 21

Chronic CML

Answer 21

<15% blast in BM or peripheral blood, can be years in the making

Question 22

Accelerated CML

Answer 22

cytogenic clonal evolution

Question 23

Acute CML

Answer 23

 blast crisis when blasts compromise >30% of BM cells or peripheral blood. Extra medullary involvement. Spleen can start making cells, pleural effusions  start making cells.

Question 24

signs and sx of CML

Answer 24

o	Fever  without infection
o	Bone pain  expansion of blood cells in the bone marrow
o	LUQ pain secondary to enlarged spleen
o	Fatigue
o	Weakness
o	Night sweats
o	Bleeding and bruising
o	Petechiae 
o	Splenomegaly (often can feel it in the pelvis)

Question 25

Lab findings of CML

Answer 25

o Peripheral blood smear  promyelocytes, myelocyte, polys, basophils. Almost all WBCs are mature or maturing myeloid cells.
o WBC  ↑↑, markedly left-shift myeloid series but low % pros and blasts.
o RBC  normal, no anemia.
o Philadelphia Chromosome  BCR/ABL gene present in >95% of cases
o HCT  ↓
o PLT  ↑
o HALLMARK: leukocytosis, with a median WBC scount of 150,000 cells/mL
o Hypercellular bone marrow with a left shift

Question 26

tx of CML

Answer 26

allogenic BM transplantation

tx w/ chemo:

• Imatinib  can live a decade or more if they take their medication.
• Dasatinib
• Interferon, often along with Cytarabine, Hydroxyurea

Question 27

Chronic Lymphocytic Leukemia

Answer 27

clonal disorder of B lymphocytes involving the bone marrow and peripheral blood

-may also infiltrate lymphatic tissues and hematopoetic organs such as the liver, spleen, and bone marrow

Question 28

CLL epidemiology

Answer 28

most common leukemia, dz of older ppl, Male, >50

Question 29

PP of CLL

Answer 29

clonal proliferation and accumulation of mature-appearing B lymphoctyes in blood and lymphoid tissue

slow growing and indolent
17p chromosomal deletion w/ worsen prognosis

Question 30

CF of CLL

Answer 30

-asx
-fatigue
-drenching night sweats
-weight loss
frequent and persistent infxn
lymphandenoapthy
skin infxn/shingles
hepatomegaly and splenomegaly

Question 31

What sydrome can occur w/ CLL?

Answer 31

Richter’s- when an isolated node transforms inot aggressive, large-cell lymphoma

Question 32

Lab findings of CLL

Answer 32

o WBC  HIGH, >20,000/µL (hallmark)
o Anemia
o PLT  ↓
o Low levels of gamma globulin

Question 33

what are the hallmarks and pathognomonic CLL?

Answer 33

peri. blood smear- mature, small lymphocytes and smudge cells
* isolated lymphocytosis w/ a leukocytosis of > 20,000

Question 34

tx of CLL

Answer 34

o Observation
o Chemotherapy  Rituximab
o Bone Marrow Transplant
o Tumor lysis prophylaxis  allopurinol, 300 mg/day, hydration, diuretics
o Radiation  to control bulky adenopathy
o Surgery
o Complete remission rates of 25-50% can be achieved with initial treatment regimens. Relapse rates are high