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1.24 Leukemia: in general and acute
By Dr. Weilbaecher
100
Pathology
Professional
01/24/2012

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Term
Where do leukemias originate?
Definition
the bone marrow
Term
What is a lymphoma
Definition
lymphocytic malignancy that arises in lymph nodes and extranodal lymphoid tissues
Term
Leukemias are classified by cell lineage into either...
Definition
myeloid (myelogenous) or lymphoid (lymphocytic, lymphoblastic)
Term
What are the four main categories of leukemia?
Definition
acute lymphoblastic leukemia, chronic lymphocytic leukemia, acute myeloid leukemia, chronic myelogenous leukemia
Term
What is a synonym for ALL?
Definition
lymphoblastic leukemia/lymphoma
Term
What does AML stand for?
Definition
acute myelogenous or acute myeloid leukemia
Term
What is a synonym for CML?
Definition
chronic granulocytic leukemia
Term
Ionizing radiation is associated with all leukemias except...
Definition
CLL
Term
What disorders involving chronic marrow dysfunction can cause leukemia?
Definition
myelodysplastic syndrome, chronic myeloproliferative diseases (CMPD), CML, aplastic anemia, PNH
Term
What congenital chronosomal abnormalities result in a 20 fold increase in acute leukemia?
Definition
Down's syndrome
Term
What cytochemical stains do you use to detect myeloid (granulocytic) cells?
Definition
myeloperoxidase (MPO) and sudan black B (SBB)
Term
What does LAP stand for?
Definition
leukocyte alkaline phosphatase
Term
what is the LAP level in cml?
Definition
low to absent (high in leukemoid reactions
Term
What cytochemical stain is used for hairy cell leukemia?
Definition
tartrate-resistant acid phosphatase (TRAP)
Term
Leukemic cells can be typed as..
Definition
lymphoid or myeloid, T or B lymphoid, immature or mature
Term
What CD is for B lymphoblasts
Definition
CD10 (CALLA)
Term
What leukemia is usually CD 10+?
Definition
ALL
Term
Wat are the CDs for B lymphocytes?
Definition
CD19, 20, 23
Term
What is the CD for CLL?
Definition
CD 5
Term
What is the CD marker for myeloid cells (granulocytes)?
Definition
CD13
Term
What CDs are used for stem cells and blasts with nonspecific lineage?
Definition
CD34, 117
Term
What two leukemias are both positive for CD34+?
Definition
AML and ALL
Term
What CD is characteristic of immature myeloid cells?
Definition
CD33
Term
What CD is characteristic of both T and B lymphoblasts?
Definition
TdT
Term
What leukemia is positive for TdT?
Definition
T and B lymphoblasts
Term
What is the typical marker profile of CLL?
Definition
CD 19+, CD5+, CD23+
Term
What is the typical marker profile of AML?
Definition
CD34+, CD33+, and CD13 +
Term
What is the typical marker profile of ALL?
Definition
CD34+, TdT+, CD10+, CD19+ (B-cell ALL)
Term
Are deletions a structural or numerical chromosomal alteration?
Definition
structural
Term
What is the characteristic cytogenetics of CML?
Definition
philadelphia chromosome= t(9;22)
Term
What leukemias are associated with the philadelphia chromosome?
Definition
CML and ALL
Term
What is the characteristic cytogenetics of AML-M2?
Definition
t(8;21)
Term
What are the cytogenetics of AML-M3?
Definition
t(15;17)
Term
What is the characteristic cytogenetic abnormality of AML-M4Eo
Definition
inv(16)
Term
Which leukemia is associated with hyperdiploidy?
Definition
ALL
Term
What are the molecular diagnostic tests for leukemia?
Definition
southern blot, PCR, FISH
Term
How do leukemias cause morbidity and motality?
Definition
decrease in normal hematopoietic cells by marrow replacement; invusion of organs with functional impairment; systemic manifestations of weightloss, fever, ,sweats, or metabolic alterations
Term
How do leukemias cause anemia?
Definition
marrow replacement, bleeding/hemorrhage, splenomegaly (hypersplenism)
Term
How do leukemias cause neutropenia/immune defects?
Definition
marrow replacement, impaired humoral immunity, spelnomegally
Term
Which leukemia is associated with impaired humoral immunity d/t decreased Ig?
Definition
CLL
Term
What leukemia is associated with DIC?
Definition
AML-M3
Term
Which organs are enlarged by leukemias? What types of leukemias?
Definition
splenomegally, hepatomegaly, lymphadenopathy; more prominent in chronic leukemias
Term
Name common locations of extramedullary leukemic infiltration?
Definition
skin, gums, CNS, periosteum
Term
What metabolic alterations are associated with leukemia?
Definition
acute tumor lysis syndrome and pseudohyperkalemia
Term
What is acute tumor lysis syndrome?
Definition
leukemic cells destroyed by chemotherapy release intracellular contents into blood sometimes with acute renal failure
Term
What is pseudohyperkalemia?
Definition
invitro hemolysis of leukemic cells in patients with very high WBC counts
Term
What do you call an uncontrolled proliferation of monoblasts with loss of differentiation?
Definition
acute myeloid leukemia
Term
What is the most common childhood cancer?
Definition
ALL
Term
What is the peak age of incidence of childhood acute leukemias?
Definition
2-4 yoa
Term
What are the symptoms of ALL?
Definition
bone and joint pain; infection and bleeding
Term
What leukemia can cause a mediastinal mass?
Definition
seen in half of T cell ALL
Term
What findings on physical exam for ALL?
Definition
generalized LAD and hepatosplenomegaly
Term
Where are common sites of leukemic relapse for ALL?
Definition
CNS and testes
Term
What is the most frequent childhood type of ALL?
Definition
early pre-B
Term
Describe the cytogenetic abnormalities of early pre-B ALL?
Definition
hyperdiploidy; CD10+
Term
What is the prognosis of early pre-B ALL vs. pre B ALL vs. T cell ALL?
Definition
early pre B= good
pre-B= intermediate
T cell= poor prognosis
Term
Describe the characteristic features of T cell ALL.
Definition
high WBC, mediastinal mass, male predom, early CNS involvement
Term
What are the CBC findings of ALL?
Definition
WBC elevated in 60%; normal or low in 40%
anemia, neutropenia, and thrombocytopenia
Term
What do you see on peripheral blood smear of ALL?
Definition
lymphoblast and smudge cells
Term
What percent of ALL have chromosomal abnormalities?
Definition
90%
Term
What cytogenetic abnormality of ALL has a favorable prognosis?
Definition
hyperdiploidy (>46 chromosomes)
Term
What cytogenetic abnormality of ALL is associated with a poor prognosis?
Definition
t(9;22)
Term
How does the philadelphia chromosome cause cancer?
Definition
chrom 22 has a BCR/ABL hybrid gene that produces a 190 kd oncogenic protein with tyrosine kinase activity
Term
What WBC count is a favorable prognostic factor for ALL?
Definition
<50,000
Term
What age is a favorable prognostic factor for ALL?
Definition
2-10 years old (infants and people >10 have poorer prognosis)
Term
What gender ahs a more favorable prognosis for ALL?
Definition
female
Term
What is the % of complete remission in kids versus adults?
Definition
90% in kids; 30% in adults
Term
Which leukemia forms a leukemic hiatus?
Definition
acute myeloid leukemia
Term
From what can AML arise?
Definition
CML; acute transformation of myelodysplastic syndrome; previous chemotherapy; de novo
Term
M0,1,2=
Definition
mainly of myeloblasts
Term
M3=
Definition
predominately promyelocytes
Term
M4=
Definition
both myeloblasts and monoblasts
Term
M5=
Definition
predominantly monoblasts
Term
M6=
Definition
myeloblasts and erythroblasts
Term
M7=
Definition
megakaryoblasts
Term
M0-M7 are all types of...
Definition
AML
Term
What is the FAB definition of AML?
Definition
30% blasts in blood or BM
Term
What cytogenic abnormality is associated with AML-M2?
Definition
t(8;21)
Term
How do you diagnose AML-M3?
Definition
t(15;17)
Term
What is counted in the blast count of an AML-M3?
Definition
both myeloblasts and promyelocytes
Term
Which AML has common DIC?
Definition
AML-M3
Term
Which leukemia is associated with numerous auer rods?
Definition
AML-M3
Term
How do you treat AML-M3?
Definition
ATRA (all trans retinoic acid) + chemo
Term
What is the official name for AML-M4?
Definition
acute myelomonocytic leukemia
Term
Where does AML-M4 and M5 have tissue infiltration?
Definition
gums, skin, CNS
Term
What is the official name of AML-M5?
Definition
acute monoblastic/monocytic luekemia
Term
What is the official name for AML-M6?
Definition
erythroleukemia (Diguglielmo's syndrome)
Term
What is the official name for AML-M7?
Definition
acute megakaryoblastic leukemia
Term
Which AML is associated with Down's syndrome?
Definition
AML-M7
Term
What accounts for 80% of acute luekemias in adults and is uncommon in childhood?
Definition
AML
Term
Wat are the symptoms of AML?
Definition
fatigue and weakness, infection, hemorrhage
Term
What is another name for granulocytic sarcomas?
Definition
chloromas
Term
What is a granulocytic sarcoma?
Definition
soft tissue tumor composed of neoplastic myeloid cells
Term
What is luekostasis?
Definition
intravascular clumps of leukemic cells in CNS, lungs that occur with WBC is >100,000
Term
What is the WHO definition of AML?
Definition
minimum of 20% blasts in the blood or BM
Term
HOw does WHO classify AML?
Definition
AML with recurrent cytogenetic abnormalities; AML with myelodysplastic related changes; AML, therapy related; AML, not otherwise cateogorized
Term
What percent of AML is therapy related? What is the prognosis?
Definition
10-15%, poor prognosis
Term
What are the features of PBS on AML?
Definition
leukemic hiatus and auer rods
Term
What are auer rods?
Definition
fusion/crystallization of primary granules in some blasts
Term
How do you treat acute myeloid leukemia? prognosis?
Definition
combination chemotherapy is the mainstay of management; high initial complete response rates are achieved with favorable cytogenetic types although remission is seldom permanent; only 20% of patients are alive 4 years after diagnosis
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