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Hematology > The Acute Leukemias > Flashcards

The Acute Leukemias Flashcards

1
Q

What is ALL

A

Acute lymphoblastic leukemia
A clonal disorder of the lymphocytes(both b and t lymphocytes) in which there is an accumulation of lymphoblasts in the bm resulting in marrow failure and pancytopenia

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2
Q

Epidemiology of ALL

A

Median age of incidence- 3-5yrs
75% of cases diagnosed before the age of 10yrs
All makes up 76% of leukemia in individuals <15years with the remaining 20% in adults
Male predilection

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3
Q

Aetiology of ALL

A

Exact aetiology basis unknown, however
Ionising radiation, chemicals and infections(viral/bacterial) has been seen to play a role
Less than 5% of all has been said to be associated with inherited genetic abnormality like down syndrome, bloom syndrome, ataxia telengiectasia.
However, majority are said to be associated with acquired genetic abnormalities

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4
Q

Acquired genetic abnormalities associated with ALL

A

Change in number of chromosomes;hyperploidy and hypoploidy
Intrachromsomal rearrangements
All with ETV6-RUNX1 rearrangement
TCF3-PBX1
BCR-ABL2
MLL gene

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5
Q

Pathogenesis of ALL

A

Like most malignancies, ALL occurs following genetic alterations which results in an enhanced ability of self renewal,loss of ability to control proliferation, resistance to apoptosis etc
Also an acquired ability to resist killing by chemotherapy is seen and this particular clone are mostly involved in relapse.

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6
Q

FAB Classification of ALL

A

French American British
Based on morphology

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7
Q

ALL L1

A

Small monomorphic lymphoblasts
Regular nuclear shape
Absent or small nucleoli

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8
Q

ALL L2

A

Large and heterogeneous lymphoblasts
Irregular nuclear shape
Large conspicuous nucleolus

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9
Q

ALL L3

A

Large homogenous lymphoblasts with deep basophilic cytoplasm , cytoplasmic vacuolisation
Irregular nuclear shape
Multiple nucleoli
Burkitt cell type

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10
Q

An important diagnostic marker for ALL

A

Terminal deoxynucleotidyl transferase (tdt)

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11
Q

WHO’S classification of ALL

A

B lymphoblastic leukemia/lymphoma otherwise not specified
~ with recurrent genetic abnormalities
T lymphoblastic leukemia /lymphoma

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12
Q

Clinical features of ALL

A

L- Lymphadenopathy painless
Y
M- neutropenic fever
P
H- Hepatosplenomegaly
O- ocular swelling
B-Bone /joint pain
L
A- Anemic symptoms
S- scrotal enlargement in males
T- Thrombocytopenic bleeding
I- infection
C- CNS symptoms

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13
Q

Investigations of ALL

A

Fbc…anemia, Thrombocytopenia, Leucocytosis
Pbf…pancytopenia with varying percentage of circulating lymohoblasts
Trephine decreased myelo, erythro, mgakaryopoeisis and
Increased lymphoblasts greater than 20%
Immunophenotyping CXR Lumbar puncture

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14
Q

Differentials of ALL

A

ITP
Aplastic anemia
Viraemia
Juvenile rheumatoid arthritis

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15
Q

Treatment for ALL

A

Could be supportive or definitive
Supportive including pain medications, treatment of infant,transfusion support etc

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16
Q

Definitive treatment for ALL

A

Period of 2-3 years using drug combinations
-vincristine
-L-asparaginase
-Steroid
-Antracycline

Induction-To completely eradicate the tumor
Intensification/consolidation..following complete remission to further reduce tumor burden
Maintenance;to clear minimal residual disease
CNS directed therapy

17
Q

Philadelphia positive ALL

A

Bad prognosis
Treated using allogeneic sct
Tyrosine kinase inhibitors like imatinib, dasatinib

18
Q

Prognosis for ALL

A

Children have better prognosis than adults and children less than 1 yr
Male predilection T ALL is worser than B ALL
Presence of philadelphia chromosome
Presence of Cns disease

19
Q

Acute myeloid leukemia..Definition

A

A clonal malignant disorder of the multipotent hc that results in the accumulation of myeloblasts predominantly in the marrow and also peripheral cytopenias

20
Q

Epidemiology of AML

A

Commoner in older age groups with peak incidence around the 7th decade of life
Male predilection

21
Q

Etiology of AML

A

Same as MDS
Alkylating agents like cyclophosphamide and topisomerase II Inhibitors like etoposide
Can also arise from several hsc diseases like cml pm pv most importantly mds
Or some inherited predisposition to Malignancy like fanconi’s anemia, dyskeratosis congenita

22
Q

Pathogenesis of AML

A

Somatic mutations arising from translocation of chromosomal segments which disrupt several genes that code for proteins involved in control of cell cycle
This then results in an uncontrolled proliferation of cells with maturation arrest, resistance to apoptosis and accumulation of malignant myeloblasts in the BM

23
Q

Consumption coagulopathy with bleeding occurs in

A

Acute promuelocytic leukemia

24
Q

3 modes of classification of AML

A

FAB
WHO
Immunophenotyping

25
Q

FAB Classification of AML

A

M0- M7
M0- AML undifferentiated
M1- AML with minimal maturation
M2-AML with maturation
M3- Acute promyelocytic leukemia
M4-Acute myelomonocytic leukemia
M5-Acute monocytic/monoblastic leukemia
M6-Acute erythroid leukemia
M7-Acute megakaryoblastic leukemia

26
Q

In immunophenotypic classification of Aml, what cluster of differentiation are typically affected

A

CD13 and CD33

27
Q

Clinical features of AML

A

Tb, nf, as
Some tissue infiltration mainly in monocytic variant
-Gingival hyperplasia, granulocytic sarcoma
-bleeding due to coagulopathy in premyelocytic leukemia

28
Q

Investigations of AML

A

Sane as ALL, However myeloblasts seen instead of lymphoblasts
Myeloblasts- medium sized cells
High n:c ratio
Fairly abundant granules or a granular cytoplasm
Nucleus composed of fine, open chromatin and has 3 or more nucleoli

29
Q

Cut off for morphological diagnosis of aml

A

> 20% myeloblasts in bm
Immunopenotyping to detect cd13 and 33
Cytochemical stain which include positivity for sudan B black and myeloperoxidase
Cytogenetics to detect t(5,17),t(8,21), inv 16
LFT, RFT, CXR ECG are also important

30
Q

What differentiates aml from all

A

Cytochemical stain which shows positivity for Sudan B black and MYELOPEROXIDASE enzyme I.e myeloperoxidase staining in Aml
Presence of Auer rods also

31
Q

Treatment of AML

A

Supportive and definitive

32
Q

Definitive treatment for aml

A

Remission induction- to achieve complete remission
Doxorubicin, cytosinearabinoside with or without thioguanine

Post remission therapy

33
Q

Acute promyelocytic leukemia is treated with

A

Retinoic acid
Arsenic trioxide

34
Q

Targeted therapy for aml

A

Anti Cd33- Gemtuzumab
Anti FLT3- midostarin
Allogeneic sct to prevent relapse

Hematology (6 decks)

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