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Questions and Answers
What percentage of adult acute leukaemia is represented by AML?
What is the common symptom of bone marrow failure in leukaemia?
Which of the following is NOT a risk factor for leukaemia?
What is the typical blast cell percentage in acute leukaemia?
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Which of the following is a common complication of leukaemia?
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What is the name of the virus associated with ALL?
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What is the goal of consolidation therapy in treating ALL?
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What is the approximate 5-year survival rate for children with ALL?
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What is a common emergency that can occur in patients with acute leukemia?
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What is the percentage of adults who achieve complete remission (CR) with modern therapy for ALL?
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What is a characteristic of Chronic Myeloid Leukemia (CML)?
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What is the percentage of leukaemias that CML forms?
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What is the incidence rate of CLL?
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What is a common symptom of CLL?
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What is the typical finding on blood film in CLL?
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What is an indication to start treatment in CLL?
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What is a common autoimmune complication of CLL?
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What is a treatment option for CLL?
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What is the defining characteristic of Acute Leukemia?
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Which type of progenitor cell gives rise to B-lymphocytes and Plasma cells?
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What is the basis of FAB classification of AML?
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Which of the following cells is NOT derived from the Myeloid progenitor?
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What is the term for the accumulation of blasts in the marrow?
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What is the correct sequence of cell development from the Hematopoietic stem cell?
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What is the peak age of incidence for Chronic Myeloid Leukemia (CML)?
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What is the characteristic of the blasts in the chronic phase of CML?
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What is the primary clinical presentation of CML?
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What is the characteristic of the peripheral blood in CML?
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What is the primary diagnostic tool for CML?
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What is the first line drug in the management of chronic phase CML?
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What is the characteristic of Chronic Lymphatic Leukemia (CLL)?
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What is the median age of incidence for CLL?
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Study Notes
Leukaemia
- Definition: Progressive proliferation of immature leucocytes (blast cell >20%) that diffusely infiltrate all organs and tissues, displacing normal haematopoietic cells from the bone marrow.
- Incidence: 1:10,000 for AML (80% of adult AL) and 1:70,000 for ALL (80% of children AL).
Etiology of Leukaemia
- Genetic: Down syndrome, Fanconi anemia
- Radiation
- Chemical: benzene
- Chemotherapy
- Viruses: EBV in ALL
- Previous haematological disorders: MDS, myelofibrosis, CML
Clinical Features
- Symptoms of bone marrow failure: anaemia, thrombocytopenia, neutropenia
- Tissue infiltration: lymph node enlargement, hepatosplenomegaly, CNS involvement, lung, gum hypertrophy, skin chloromas (monocytic AML), testicular infiltration (ALL)
- Leukostasis causes infarction
- Increased uric acid
- Bone pain
Acute Leukaemia
- Accumulation of blasts in the marrow >20%
- AML: tissue/organ infiltration, bone marrow cytogenetic for prognosis and treatment, immunophenotyping of blood or marrow blasts (CD19 for ALL, CD33 for AML)
- ALL: tissue/organ infiltration, central nervous system (CNS) involvement
Classification of AML
- FAB classification: M0, M1, M2, M3, M4, M5, M6, M7 based on predominant differentiation pathway and degree of differentiation.
Specific Treatment of ALL
- Remission induction: using vincristine, prednisolone, daunorubicin, and asparaginase
- CNS prophylaxis: cranial irradiation and intra-thecal methotrexate
- Consolidation therapy: 1-2 intensification phases to reduce the risk of relapse
- Maintenance therapy: 2-3 years of 6-MP and methotrexate
- Allogeneic stem transplant: an option in young patients with high-risk disease
Prognosis of ALL
- About 75% of adults achieve complete remission (CR) with modern therapy and good supportive care
- 5-year survival rate for children is around 70%
- For adults <30% (less than 20% for those <50 years)
Emergencies in Haematological Malignancies
- In acute leukaemia: DIC with severe bleeding (AML M3), severe thrombocytopenia with ICH, neutropenic fever and septic shock, tumour lysis syndrome and acute renal failure
Chronic Myeloid Leukaemia (CML)
- Definition: clonal disorder of pluripotent stem cells
- One of the myeloproliferative disorders (MPD) that forms 15-20% of all leukaemias
- Peak age of incidence: 40-60 years
- Incidence: 1.25/100,000
- 3 phases: chronic phase (blast 5-9%), accelerated phase (blast 10-19%), blastic phase (blast 20%)
Clinical Features of CML
- Asymptomatic, discovered accidentally
- Anaemia
- Abdominal discomfort due to splenic enlargement (splenomegaly in >75%), hepatomegaly
- Sweating, fever, weight loss, gout, leukostasis (infarction)
- Signs: pallor and splenomegaly that can be huge
Investigations for CML
- Peripheral blood: Hb N or ↓, Plt: N, ↑, or ↓, WCC: ↑ (usually above 50,000/cu mm but may reach up to 500,000), shows the whole spectrum of myeloid precursors with only few blasts
- Bone marrow: hypercellular with ↑ in myeloid series
- Cytogenetics: Ph chromosome (t 9-22) is present in most patients
- PCR for BCR-ABL fusion gene
- Increased ESR, LDH, uric acid, decreased NAP score (increased in infection)
Management of CML
- Imatinib (STI 571): first-line drug in chronic phase, causes major cytogenetic response in 83% of patients
- Chemotherapy: hydroxyurea, effective in controlling leucocytic count
- IFN-alpha SC: may produce cytogenetic response (decrease the % of Ph +ve cells), may be useful in pregnant
- Bone marrow transplant: only established curative treatment
Chronic Lymphatic Leukaemia (CLL)
- Definition: uncontrolled proliferation and accumulation of lymphocytes that are mostly mature but immunologically incompetent
- Incidence increases with age, median age: 65 years, incidence: 2.5/100,000
Clinical Features of CLL
- Often asymptomatic, detected accidentally
- May complain of recurrent infection, weight loss, manifestations of BM infiltration: anaemia, neutropenia, thrombocytopenia
- G. lymphadenopathy, moderate splenomegaly, hepatomegaly
- Autoimmune AIHA, ITP may occur
Investigations for CLL
- FBC and blood film: lymphocytosis > 5X 10^9/L, mature lymphocytes
- Anaemia, neutropenia, thrombocytopenia due to BM failure are usually absent in early stage, however, they can be caused by autoimmune mechanisms
- BM: lymphocytosis > 30% with characteristic immunophenotypic markers (CD5,23)
Treatment of CLL
- Indications to start treatment: anaemia, recurrent infection, bleeding, bulky disfiguring or compressing LNs, increasing splenomegaly
- Treatment: chlorambucil with or without prednisolone, prednisolone to control AIHA, sometimes radiation to relieve compression
- Stem cell transplant
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Description
This quiz covers the definition and types of Leukaemia, a haematological malignancy. It discusses the differences between chronic and acute Leukaemia, as well as myeloid and lymphatic types.
