9. Acute leukemias Flashcards
Define Leukemias
They are a group of disorders characterized by the accumulation of malignant white cells in the bone marrow and blood
What are the four classifications of Leukemia
Acute lymphoid Leukemia
Acute myeloid Leukemia
Chronic Lymphoid Leukemia
Chronic Myeloid Leukemia
Describe the onset of Acute Leukemias
Aggressive disease in which malignant transformation occurs in the haemopoietic cell or early progenitors (blast cells)
What is the most common clinical feature of Acute Leukemias
Bone marrow failure
What causes bone marrow failure in Acute Leukemia
Caused by accumulation of blast cells in addition to organ infiltration
Define Acute Leukemia
The presence of over 20% of blast cells in the blood or bone marrow at clinical presentation
What are the two subdivisions of Acute Leukemias
Acute Myeloid Leukemia (AML)
Acute Lymphoblastic Leukemia (ALL)
What is the Morphological difference between ALL and AML
In ALL the blasts show no differentiation (with the exception of B-cell) whereas in AML some evidence of differentiation to granulocytes or monocytes is seen
What causes Acute Lymphoblastic Leukemia
An accumulation of lymphoblasts in the bone marrow and is the most common malignancy of childhood
What is the most common malignancy of childhood
Acute Lymphoblastic Leukemia
What is the incidence of Acute Lymphoblastic Leukemia (ALL)
3-7 yrs
Based on morphology, what are the three subtypes of ALL (Acute Lymphoblastic Leukemias)
L1
L2
L3
Describe L1 ALL
Uniform, small blast cells with scanty cytoplasm
Describe L2 ALL
Type comprise larger blast cells with more prominent nucleoli and cytoplasm and more heterogeneity
Describe L3 ALL
Blasts that are large with prominent nucleoli, strongly basophilic cytoplasm and cytoplasmic vacuoles
What are the four subtypes of ALL based on Immunophenotype
Early pre B
Pre B
B- cell
T- cell
What are the two main features of ALL(Acute Lymphoblastic Leukemia)
Bone Marrow Failure
Organ Infiltration
What are three effects of Bone Marrow Failure
Anaemia
Neutropenia
Thrombocytopenia
What are three clinical features of Anemia
Pallor
Lethargy
Dyspnoea
What are three clinical features of neutropenia
Fever
Malaise
Mouth, throat, skin, respiratory, perianal infections
List four clinical features of Thrombocytopenia
Spontaneous bruises
Purpura
Bleeding gums
Menorrhagia
What are five common manifestations of Organ infiltration
Tender bones Lymphadenopathy Moderate Splenomegaly Hepatomegaly Meningeal Syndrome
What are five symptoms of Meningeal syndrome
Headache Diplopia Blurred vision Nausea Vomiting
What are the results of haematologic tests in ALL and AML
Normochromic
Normocytic
Thrombocytopenia
Total White Cell count may be decreased, normal or increased
What are the results of Blood film Examination in ALL and AML
Variable number of blast cells
What are the features of the bone marrow in ALL and AML
Hypercellular with >20% leukemia blasts
What is the result of Lumbar Puncture in ALL and AML
Spinal fluid has increased pressure
Spinal fluid contains leukemic cells
What is the result of Biochemical tests for ALL and AML
Raised serum uric acid
Raised serum lactate dehydrogenase
List some differential diagnosis for ALL
AML Aplastic anemia Marrow infiltration by other malignancy Infections Juvenile Rheumatoid Arthritis
B- ALL always has which ALL morphology
L3
What are two types of treatment for ALL
General Supportive therapy
Specific Therapy
Describe Specific Therapy of ALL
Chemotherapy
Radiotherapy
What is the aim of Remission Induction
Type of Specific Treatment for ALL
Rapidly kill most of the tumour cells and get the patient into remission.
Which drugs are used for remission induction treatment of ALL
Prednisolone or Dexmethasone (in children)
Vincristine
Asparaginase
Describe Intensification (consolidation) treatment for ALL
Uses high doses of multidrug
chemotherapy in order to completely reduce or eliminate the tumour burden to very low levels
What percentage of Acute Lymphoblastic Leukemias occur in children
80%
What percentage of Acute Lymphoblastic Leukemias occur in adults
20%
What percentage of Acute Myeloid Leukemias occur in children
20%
What percentage of Acute Myeloid Leukemias occur in adults
80%
What are 5 risk factors for leukemia
Ionizing radiation Benzene Down's Syndrome Chronic Smoking Klinefelter syndrome
T- ALL is most common in which sex
Males
What is the prognosis of ALL in children
Usually good
~90% of children can expect to be cured
What is the prognosis of ALL in adults
Poor
Adults over 70 yrs have <5% cure rate
What is the common form of Leukemia in adults
Acute Myeloid Leukemia AML
What fraction of childhood leukemias does Myeloid Leukemia form
10-15%
What are the two main types of Acute Myeloid Leukemia
Primary AML
Secondary AML
What is the genetic abnormality seen in Primary AML
Chromosomal translocations of t(8;21)
And t(15;17)
What is the prognosis of primary AML
Good
What are the clinical features of AML
Anaemia
Thrombocytopenia
neutropenia
List one problem that is unique to AML and not ALL
Haemorrhagic syndrome
associated with the AML M3 variant
Haemorrhagic syndrome unique to AML is associated with which variant
M3 variant
How is Haemorrhagic Syndrome of AML treated
Multiple platelet transfusions
Replacement of clotting factors with fresh frozen plasma
CASE HISTORY//
6 yr old female with fever for 3 weeks, less active, easily tired, bleeding gums and easy bruising for 1 week
Physical Examination
- pale, febrile, tenderness over sternum
- multiple haemorrhagic lesions
- cervical nodes enlarged, non- tender
- spleen enlarged
BLOOD RESULTS//
Hb 6.0 g/dl blood film - normochromic / normocytic
Platelets : 12 x 10^9/L
WBC : 85 x 10^9/L
Chest X ray : enlarged nodes
Bone marrow: 90% blasts with few erythroid and megakaryocyte precursors
Leukemia
