Usera: Acute and Chronic Leukemias Flashcards
Who gets ALL?
children (0-14yo)
**75% of cases in children under 6yo
Who gets AML?
adolescents to middle-aged adults (15-59)
Who gets AML/CML?
adult 40-60+
Who gets CLL?
elderly adults
Where are cells arrested in acute myelogenous leukemia?
maturation arrest in granulocyte development, leads to proliferation of malignant precursor cells
What are 4 clinical features of AML (acute myelogenous leukemia)?
- anemia
- thrombocytopenia
- neutropenia
- leukocytosis with increased blasts
What are 4 signs and symptoms of acute myelogenous leukemia?
- fatigue
- bleeding/bruising
- DIC
- gum infiltration
What are auer rods? What cells are they unique to?
primary granules; unique to myeloid lineage
Acute promyelocytic leukemia makes up 5-8% if AML. What chromosome translocation is common?
t(15:17)(q22:q12)
Who usually gets acute promyelocytic leukemia (APML)?
any age, usu 3-5th decade of life
What are some clinical features of acute promyelocytic leukemia?
frequently presents with DIC (acute hypercoagulative state)
rapid doubling time
large numbers of Auer rods
What genes are translocated in a t(15;17) translocation in pts with APL?
PML and RARA (retinoic acid receptor)
What do you use to treat acute promyelocytic leukemia?
ATRA and alkylating agent
**80-90% remission
What is a characteristic feature of acute monocytic leukemia? Are auer rods common?
gum infiltration (malignant cells deposit themselves in the gingiva); no auer rods are rare
What chromosome abnormality is associated with chronic myelogenous leukemia?
de novo mutation in Philadelphia chromosome
**balanced translocation in (9;22) BCR-ABL fusion gene
Explain how the de novo mutation in the Philadelphia chromosome leads to chronic myelogenous leukemia.
BCR-ABL fusion gene produces a tyrosine kinase which is ALWAYS active, leading to dysregulation of proliferation and normal apoptosis
What are some clinical features of chronic myelogenous leukemia (CML)?
all the different granulocytes present; fever, night sweats, weight loss; splenomegaly; bleeding/ecchymoses <10% blasts
What are characteristics of the blast phase crisis in chronic myelogenous leukemia?
progression to acute leukemia
>20% blasts
large aggregates in bone marrow and extramedullary tissue
usu fatal
What does the prognosis of chronic myelogenous leukemia depend on?
the response to TKI’s (ex: imatinib aka Gleevec)
What is the most common leukemia in children?
B cell acute lymphoblastic leukemia
Do most cases of acute lymphoblastic leukemia affect T cells or B cells?
most affect B cells (80-85%)
What cell markers are expressed in acute lymphoblastic leukemia?
CD10 (B cell marker) and tdt
Describe what the cells look like in acute lymphoblastic leukemia.
primitive, undifferentiated lymphoblasts; cells are smaller than myeloblasts; no auer rods;
What are some clinical features of acute lymphoblastic leukemia?
- marrow failure: anemia, thrombocytopenia, neutropenia
- WBC may be low, normal, or very high
- commonly spreads to CNS
Where does acute lymphoblastic leukemia most commonly spread to?
CNS
What are 3 factors that give a good prognosis for ALL?
- ages 1-10 = best prognosis
- t(12;22)
- hyperdiploidy (really diverse, crazy cells are more susceptible to chemo)
What do T cell acute lymphoblastic leukemias frequently present with?
mediastinal mass or acute leukemia
What are the T cell markers for T cell acute lymphoblastic leukemias?
CD10-, tdt+
Overall, this is the most common human leukemia
chronic lymphocytic leukemia
What type of leukemia is CLL (chronic lymphocytic leukemia)? What is unique about this leukemia?
clonal B cell neoplasm, only light chains; unique to B cells
What are the two markers present in chronic lymphocytic leukemia?
CD5 and CD23
**combo of T cell marker (CD5) and B cell marker (CD23)
What are some morphological features of chronic lymphocytic leukemia?
high white cell count
small mature lymphocytes w clumped chromatin and occasional smudge cells
What is the prognosis for chronic lymphocytic leukemia?
good! indolent disease - treatment is usu withheld bc these patients are likely to live
What is the UNFAVORABLE marker that you should know for chronic lymphocytic leukemia?
ZAP-70
Mutation in this gene is FAVORABLE in CLL
IGVH
What develops in 2-8% of cases of chronic lymphocytic leukemia? What is this referred to as?
lymphomas;
Richter transformation - bad prognosis - less than 1 year :(
Who gets hairy cell leukemia?
older caucasian males
What are two features to remember in hairy cell leukemia?
- monocytopenia (or pancytopenia)
2. splenomegaly (cancer cells infiltrate red pulp of spleen)
What markers are present in hairy cell leukemia?
CD11, CD25, CD103
Where does hairy cell leukemia usually infiltrate?
the RED pulp of the spleen - causes splenomegaly
What does the bone marrow look like in hairy cell leukemia?
fried egg appearance
What is the bone marrow biopsy like in hairy cell leukemia?
dry tap - can’t get good bone marrow sample
This leukemia is a CD4 T cell neoplasm caused by HTLV; it is endemic in Japan, West Africa, and the Caribbean; causes hepatosplenomegaly and lymphocytosis; rapidly progressive and fatal
adult T cell leukemia
What are the characterstic cells in adult T cell leukemia?
clover leaf cells
