Podcast
Questions and Answers
Which subtype of B lineage ALL is characterized by HLA-DR+, TdT+, CD19+, and CD10-?
Which subtype of B lineage ALL is characterized by HLA-DR+, TdT+, CD19+, and CD10-?
Which category of AML includes cases with distinct numerical chromosomal abnormalities?
Which category of AML includes cases with distinct numerical chromosomal abnormalities?
What is a common clinical feature of ALL at presentation?
What is a common clinical feature of ALL at presentation?
What must be present in at least 50% of cells in 2 or more myeloid lineages to classify AML as having multi-lineage dysplasia?
What must be present in at least 50% of cells in 2 or more myeloid lineages to classify AML as having multi-lineage dysplasia?
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Which type of T lineage ALL is identified by TdT+, cytoplasmic CD3+, and CD1a/2/3+?
Which type of T lineage ALL is identified by TdT+, cytoplasmic CD3+, and CD1a/2/3+?
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Which infection is particularly common in patients with ALL?
Which infection is particularly common in patients with ALL?
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Which type of AML is particularly associated with prior exposure to alkylating agents or radiation?
Which type of AML is particularly associated with prior exposure to alkylating agents or radiation?
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Which lymphadenopathy prevalence rate is reported in patients with ALL?
Which lymphadenopathy prevalence rate is reported in patients with ALL?
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What is a characteristic of acute myeloid leukemia not otherwise categorized?
What is a characteristic of acute myeloid leukemia not otherwise categorized?
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How is acute promyelocytic leukemia identified within the WHO classification system?
How is acute promyelocytic leukemia identified within the WHO classification system?
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What is a characteristic finding in the full blood count of a patient with acute leukemia?
What is a characteristic finding in the full blood count of a patient with acute leukemia?
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Which cytochemical stain is NOT positive in acute myeloid leukaemia (AML)?
Which cytochemical stain is NOT positive in acute myeloid leukaemia (AML)?
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What condition has the highest incidence of acute lymphoblastic leukaemia (ALL)?
What condition has the highest incidence of acute lymphoblastic leukaemia (ALL)?
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Which of the following is considered a potential environmental risk factor for acute lymphoblastic leukaemia (ALL)?
Which of the following is considered a potential environmental risk factor for acute lymphoblastic leukaemia (ALL)?
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What does the presence of more than 20% myeloblasts in the bone marrow indicate?
What does the presence of more than 20% myeloblasts in the bone marrow indicate?
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Which subtype of acute lymphoblastic leukaemia is most common?
Which subtype of acute lymphoblastic leukaemia is most common?
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Which of the following leukaemias is more common in males than females?
Which of the following leukaemias is more common in males than females?
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What finding in a bone marrow aspiration typically suggests acute leukaemia?
What finding in a bone marrow aspiration typically suggests acute leukaemia?
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What blood findings are typically associated with the diagnosis of ALL?
What blood findings are typically associated with the diagnosis of ALL?
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Which treatment is generally used for CNS prophylaxis in ALL?
Which treatment is generally used for CNS prophylaxis in ALL?
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What is one of the primary supportive treatments in managing ALL?
What is one of the primary supportive treatments in managing ALL?
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Which biochemical test result is expected to be elevated prior to chemotherapy in ALL patients?
Which biochemical test result is expected to be elevated prior to chemotherapy in ALL patients?
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What is the primary goal during the remission induction phase of ALL treatment?
What is the primary goal during the remission induction phase of ALL treatment?
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What is the primary characteristic required to diagnose acute leukemia?
What is the primary characteristic required to diagnose acute leukemia?
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Which type of leukemia predominantly affects children?
Which type of leukemia predominantly affects children?
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Which of the following best describes Acute Myeloid Leukemia (AML)?
Which of the following best describes Acute Myeloid Leukemia (AML)?
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What is a common symptom of acute leukemia due to marrow failure?
What is a common symptom of acute leukemia due to marrow failure?
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Which of the following is NOT a classification of leukemia?
Which of the following is NOT a classification of leukemia?
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What proportion of patients with AML typically show chromosomal abnormalities?
What proportion of patients with AML typically show chromosomal abnormalities?
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In which demographic does the incidence of Acute Myeloid Leukemia (AML) notably increase?
In which demographic does the incidence of Acute Myeloid Leukemia (AML) notably increase?
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What is a common cause of idiopathic Acute Myeloid Leukemia?
What is a common cause of idiopathic Acute Myeloid Leukemia?
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Which statement is true regarding the prognosis of Acute Lymphoblastic Leukemia (ALL)?
Which statement is true regarding the prognosis of Acute Lymphoblastic Leukemia (ALL)?
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What percentage of acute leukemias in adults does Acute Myeloid Leukemia (AML) account for?
What percentage of acute leukemias in adults does Acute Myeloid Leukemia (AML) account for?
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Which of the following factors is associated with a poor prognosis in acute lymphoblastic leukemia (ALL)?
Which of the following factors is associated with a poor prognosis in acute lymphoblastic leukemia (ALL)?
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What is the primary characteristic of biphenotypic acute leukemias?
What is the primary characteristic of biphenotypic acute leukemias?
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What percentage of myeloblasts in a peripheral smear is required for a diagnosis of acute leukemia according to the WHO?
What percentage of myeloblasts in a peripheral smear is required for a diagnosis of acute leukemia according to the WHO?
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Which of the following statements is true regarding maintenance therapy in acute leukemia?
Which of the following statements is true regarding maintenance therapy in acute leukemia?
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Which of the following conditions is more prominent in chronic leukemias compared to acute leukemias?
Which of the following conditions is more prominent in chronic leukemias compared to acute leukemias?
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Study Notes
Acute Leukemias
- Acute leukemia is a group of malignant blood disorders
- Originates from the clonal proliferation of hematopoietic stem cells in bone marrow
- Accumulation of immature (blast) or abnormal white blood cells in both peripheral blood and bone marrow occurs
- Caused by somatic mutation and malignant transformation of BM pluripotent stem cells
- Neoplastic expansion in the BM leads to abnormal leukemic cells with impaired production of red cells, neutrophils, and platelets
- Unique morphological, cytogenetic, and immunophenotypic features are used for classification, diagnosis, and prognosis
- In acute leukemia, a diagnosis requires 20% or more blasts (immature cells) in the blood and bone marrow
Classification of Leukemia
- Categorized into acute and chronic types
- Further sub-classified based on myeloid or lymphoid origin
- Acute myeloid leukemia (AML)
- Chronic myeloid leukemia (CML)
- Acute lymphoblastic leukemia (ALL)
- Chronic lymphocytic leukemia (CLL)
AML (Acute Myeloid Leukemia)
- AML is a malignant clonal disorder of the myeloid cell line
- Characterized by accumulation of myeloblasts in blood, bone marrow, or other tissues
- Myeloblasts often show myeloid or monocytic differentiation
- Approximately 80% of AML patients have chromosomal abnormalities
- 20% or more blasts are present in blood and bone marrow
AML Epidemiology
- AML can occur at any age; incidence increases with age
- Accounts for 80% of adult acute leukemias and 20% of childhood acute leukemias
- Predominant form of congenital leukemia, often associated with Down syndrome (Trisomy 21)
- Incidence slightly higher in males compared to females
- Higher incidence in developed and industrialized countries
- Little difference in incidence between blacks and whites at any age
AML Causes
- Idiopathic (most common)
- Underlying hematologic disorders (MDS, PNH, PRV, MF)
- Exposure to chemicals (e.g., benzene, petroleum products)
- Certain drugs (cytotoxic drugs)
- Ionizing radiation
- Viral infections (HTLV-I)
- Genetic conditions (Down syndrome, Turner syndrome, Klinefelter syndrome, Fanconi anemia)
- Primary (de novo AML) or secondary to other conditions (MDS, PNH, MF, PRV) or exposure to chemical/drugs
AML Clinical Features
- Symptoms related to bone marrow suppression and organ infiltration by malignant myeloblasts
- Symptoms due to:
- Marrow failure
- Tissue infiltration
- Leukostasis (excessive WBC accumulation in circulation)
- Constitutional symptoms (fever, headaches, night sweats, anorexia, nausea, vomiting)
- Bleeding tendency (thrombocytopenia or DIC)
- Usually short duration of symptoms (weeks/months if untreated)
- Clinical findings: bone marrow infiltration, neutropenia, anemia, thrombocytopenia, medullary infiltration (marrow), extramedullary infiltration (liver, spleen, lymph nodes, central nervous system), gum hypertrophy, purpuric/petechial rashes, subcutaneous nodules,chloromas/granulocytic sarcomas
AML Classification
- Classified based on morphology of myeloblasts, molecular markers, and cytogenetic abnormalities
- Key classification systems include:
- French-American-British (FAB) classification (divides AML into 8 types; Mo-M7)
- World Health Organization (WHO) classification (divides AML into 5 categories/groups based on recurrent genetic abnormalities, multi-lineage dysplasia, therapy-related syndromes, not otherwise categorized, and ambiguous lineage)
ALL (Acute Lymphoblastic Leukemia)
- Results from accumulation of lymphoblasts in both peripheral blood and bone marrow
- Most common childhood leukemia, though it can occur in adults
- Accounts for about 76% of all leukemias diagnosed in children below 15 years
- More males are affected than females
ALL Etiology
- Etiology is unknown in most cases
- Environmental factors (ionizing radiation, chemical mutagens)
- Genetic predisposition (e.g., Down syndrome)
- Exposure to childhood infections (mainly viral)
ALL Classification
- Classified based on morphology of lymphoblasts and immunological markers
- Types:
- FAB (L1, L2, L3) - morphological classification
- Immunological classification (e.g., B-lineage: pro-B-ALL, common ALL, pre-B-ALL, B-cell ALL; T-lineage: pre-T-ALL, T-cell ALL)
ALL Morphology (FAB)
- L1: Small, homogeneous blasts
- L2: Large, heterogeneous blasts
- L3: Large, homogeneous blasts with abundant basophilic cytoplasm and vacuoles
ALL Clinical Features
- Acute presentation, often critically ill due to bone marrow failure
- Symptoms of anemia, weakness, lethargy, breathlessness, palpitations
- Infections (e.g., Staphylococcus, Pseudomonas, HSV, Candida)
- Bleeding tendencies (purpura, menorrhagia, epistaxis)
- Signs of leucostasis (hypoxia, retinal hemorrhage)
- Mediastinal involvement and CNS involvement are considerations
ALL Investigations and Diagnosis
- Full blood count and blood film (normocytic normochromic anemia, thrombocytopenia, leukocytosis with lymphoblasts)
- Bone marrow aspiration/biopsy (hypercellular marrow with >20% lymphoblasts)
- Bone marrow cytogenetics (to detect chromosomal abnormalities)
- CSF examination to look for CNS involvement
- Biochemical tests (↑ LDH, ↑ uric acid, LFTs, renal function tests)
- X-rays (to detect possible thymic or mediastinal lymph node enlargement)
- Immunophenotyping of blood or bone marrow blasts
ALL Treatment (Supportive and Specific)
-
Supportive:
- Explain diagnosis and offer counseling
- Central venous catheter for drug and blood product administration
- Transfusions of RBC and platelets
- Prophylactic and aggressive treatment of infections (antibiotics, antivirals, antifungals)
- Allopurinol to prevent hyperuricemia and tumor lysis syndrome
- Antiemetic drugs to prevent vomiting
-
Specific:
- Induction therapy to achieve complete remission (vincristine, prednisolone, daunorubicin, L-asparaginase)
- CNS prophylaxis (generally includes cranial irradiation and/or intrathecal chemotherapy)
- Consolidation therapy to reduce tumor burden and prevent relapse.
- Maintenance therapy (necessary for those not undergoing stem cell transplant)
- Allogeneic stem cell transplantation (SCT) in some cases
Prognostic Factors (AML and ALL)
-
AML:
- Cytogenetics (favorable = t(8;21), t(15;17), inv 16; unfavorable = deletions of chromosomes 5 or 7, FLT3 mutation)
- Age at diagnosis (>60 years is unfavorable)
- Onset of disease (primary or secondary)
- Bone Marrow response to induction chemotherapy (CR or <5% blasts after first course vs. >20% blasts)
-
ALL:
- TWBC (Total White Blood Cell Count) at diagnosis (low is good)
- Sex (female better prognosis)
- Age (childhood better prognosis)
- Immunophenotype (certain types of ALL have better prognoses)
- Cytogenetics (normal or hyperdiploidy is better than Ph+ or hypoploidy)
- CNS disease (absence preferable)
Biphenotypic Acute Leukemias
- A minority of cases with two distinct leukemia cell populations
- Often expressing both B-lymphoid and myeloid markers
- Variable response rates; treatment protocol selection can be problematic
Tumor Lysis Syndrome (TLS)
- Rapid tumor cell lysis causes release of intracellular substances (potassium, phosphate, nucleic acids) overwhelming the kidneys
- Can occur at presentation with chemotherapies or high-grade lymphomas
- Risk of renal failure, electrolyte imbalances, and hyperphosphatemia
- Usually treated with hydration, allopurinol, and supportive care
Additional Notes
- Presented data is from a series of presentations
- Specifics like patient demographics and detailed clinical findings depend on the specific case studies, and the summary assumes general context from the presentations.
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Description
Test your knowledge on acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) with this quiz. Explore topics such as cell markers, clinical features, and classifications of different leukemia subtypes. Answer questions about presentations and findings associated with these hematological malignancies.