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Week 1- cancer > Acute and Chronic Leukemias > Flashcards

Acute and Chronic Leukemias Flashcards

1
Q

Myelogenous Leukemias

A

Acute Myelogenous Leukemia (AML)
Gazillion subtypes

Chronic Myelogenous Leukemia (CML

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2
Q

Lymphocytic/ Lymphoblastic Leukemias

A

Acute Lymphoblasic Leukemia (ALL)
B cell
T cell

Chronic Lymphocytic Leukemia (CLL)

Hairy Cell Leukemia

Adult T cell Leukemia
HTLV-1

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3
Q

Leukemias by age

A 
0- adolescent (0-14):  ALL
Adolescent to adult (15-59):  AML
Adult(40-60+):  AML/CML
Elderly adult (>60):  CLL
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4
Q

Acute Myelogenous Leukemia

A

Young to middle aged adults

Maturational arrest in granulocyte development, leading to proliferation of malignant precursor cells

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5
Q

Clinical features of AML

A 
EVIDENCE OF MARROW FAILURE
Anemia
Thrombocytopenia
Neutropenia
Leukocytosis with ↑↑ blasts
diffuse bone marrow infiltration

Auer rods can be present

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6
Q

Signs and symptoms of AML

A

Fatigue
Bleeding/ecchymoses
DIC (APL)
Gum infiltration (monocytic)

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7
Q

Acute Promyelocytic Leukemia

A 
Corresponds to FAB M3 
WHO APML with t(15;17)(q22;q12)- TRANSLOCATION 15:17
5-8% of AML
Any age, but usually 3-5 decades of life
Frequently presents with DIC
Rapid doubling time
Often has large numbers of Auer rods
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8
Q

Acute Promyelocytic Leukemia treatment and prognosis

A

Treat with ATRA and alkylating agent

80-90% remission

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9
Q

Acute Monocytic Leukemia

A

Auer rods are rare
GUM INFILTRATION is frequently the presenting sign
Diagnosis hinges on immunophenotype (CD markers)

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10
Q

Chronic Myelogenous Leukemia

A

15% of adult leukemias
May be related to radiation or benzene
Pluripotent
t(9;22) BCR-ABL fusion gene … aka Philadelphia chromosome
Protracted chronic phase, sometimes many years, followed by BLAST CRISIS

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11
Q

Philadelphia Chromosome

A

De novo mutation

NOT specific for CML

BCR-ABL1 fusion gene produces a constitutively ACTIVE TYROSINE KINASE, leading to dysregulation of proliferation and normal apoptosis

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12
Q

Chronic Myelogenous Leukemia Clinical Features

A 
WBC >5x109/L
Characteristic diff, with FULL SPECTRUM OF GRANULOCYTES REPRESENTED 
<10% BLASTS
Fever, night sweats, weight loss
Splenomegaly
Bleeding/ecchymoses
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13
Q

What is blast crisis

A

Progression to acute leukemia

> 20% blasts

Large aggregates in BM and extramedullary tissue

Usually fatal

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14
Q

CML treatment and prognosis

A

The prognosis depends on response to TKI’s (e.g. imatinib; aka Gleevec)

With allogenic stem cell transplant, 5-year progression-free survival 80-95%

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15
Q

B Cell Acute Lymphoblastic Leukemia

A 
Most common leukemia in children
80-85% of ALL are B-cell
75% of cases involve children under 6 yo
Primitive, undifferentiated lymphoblasts
Express CD10, tdt (flow cytometry)
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16
Q

Differentiating CML from Leukemoid reaction

A

Leukocyte Alkaline phosphatase is low in CML and high in Leukemoid reaction

17
Q

Clinical Features of B cell Acute Lymphoblastic Leukemia

A

Marrow failure: anemia, thrombocytopenia, neutropenia
WBC can be low, normal, or extremely high
Extramedullary involvement common in CNS, LN, spleen, liver, testis

18
Q

B cell ALL prognosis (and favorable factors)

A 
90-95% complete remission
FAVORABLE FACTORS: 
age 1-10 
t(12;22)/ETV-RUNX1
Hyperdiploidy (>52 chromosomes)
19
Q

T cell Acute Lymphoblastic Leukemia

A

Frequently presents with mediastinal mass or acute leukemia
15-20% of childhood leukemias
CD10-, tdt+

20
Q

Chronic Lymphocytic Leukemia

A

Clonal B cell neoplasm – light chain restricted (kappa vs lambda)
OVERALL, MOST COMMON HUMAN LEUKEMIA
Most common LA in elderly
Mature, virgin B-cells (CD19+,CD20+); blasts <10%
Aberrant co-expression of CD5 and CD23

21
Q

Chronic Lymphcytic Leukemia clinical features

A 
WBC 10-100 x 109/L
Small mature lymphs
Clumped chromatin
Smudge cells
Lymphoid aggregates in bone marrow
22
Q

Prognosis of CLL- which genes are favorable and unfavorable

A

Mutated IGVH (FAVORABLE): median survival 293 months

ZAP-70 and CD38 expression are UNFAVORABLE

2-8% develop DLBCL (Richter Transformation) – median survival <1 yr

Indolent disease – treatment usually withheld, but Chlorambucil if treatment is warranted

23
Q

Hairy Cell Leukemia

A 
Neoplasm of memory B cells
Older Caucasian males
Indolent disease
Pancytopenia, monocytopenia*
Splenomegaly
CD11c, CD25, CD103
24
Q

Clinical Finding in Hairy Cell Leukemia

A

Frequent splenic red pulp sequestration – splenomegaly
Diffuse bone marrow involvement – fried egg appearance
Dense reticulin network – “dry tap”

25
Q

Adult T cell Leukemia

A 
CD4+ T cell neoplasm caused by HTLV-1
Floret, or "clover leaf" cells
LA, hepatosplenomegaly, lymphocytosis
Rapidly progressive and fatal within 1 yr
Endemic to:
Japan
West Africa
Caribean

Week 1- cancer (8 decks)

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