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Questions and Answers
What is the cure rate for children with Acute Myeloblastic Leukemia (AML) and Down Syndrome when treated with chemotherapy alone?
What is the classification system for Acute Myeloblastic Leukemia (AML)?
What is the prognosis for Acute Myeloblastic Leukemia (AML) patients with Monosomy 7?
What is the treatment for Acute Myeloblastic Leukemia (AML)?
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What is the cure rate for Acute Myeloblastic Leukemia (AML) patients treated with chemotherapy alone?
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What is the significance of WBC count > 100,000/cmm in Acute Myeloblastic Leukemia (AML)?
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What is the significance of the loss of dental lamina dura in jaw X-rays?
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What is the primary treatment approach for the disease?
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What is the age group with a worse prognosis?
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What is the significance of CSF cytology in investigations?
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What is the characteristic of lymph nodes in Hodgkin Lymphoma?
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What is the significance of B symptoms in Hodgkin Lymphoma?
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What is the age range for the young adult form of Hodgkin Lymphoma?
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What is the significance of urinary uric acid levels in investigations?
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What is the characteristic of Burkitt lymphoma in terms of growth rate?
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What is thecommonest childhood malignant tumour in Nigeria?
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What is the role of C-Myc oncogene in Burkitt lymphoma?
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What is the typical age range for Burkitt lymphoma?
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What is the characteristic cytological feature of Burkitt lymphoma?
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What is the clinical feature of Burkitt lymphoma in the jaw?
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What type of cells are present in Reed-Sternberg cells?
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What is the most common type of Acute Leukaemia in childhood?
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What is the main diagnostic test for Hodgkin lymphoma?
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What is the age range for Non-Hodgkin Lymphoma?
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What is the chemotherapy regimen for Non-Hodgkin Lymphoma?
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What is the definition of Acute Myeloblastic Leukaemia according to WHO?
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Study Notes
Treatment of Leukaemias
- Remission in leukaemia is achieved with chemotherapy, and prognosis is better with non-B non-T cell morphology.
- Good centres have an 80% cure rate in ALL.
Acute Myeloblastic Leukaemia
- Clinical features of AML are similar to ALL, with additional features such as chloromas, scalp swellings, and gingival hyperplasia.
- Classification of AML is based on WHO and FAB criteria (M0-M7).
- M7 megakaryoblastic leukaemia is frequently observed in Trisomy 21.
- Treatment of AML involves chemotherapy with Daunorubicin, Ara-C, and Etoposide (DAE), and stem cell transplantation.
Prognostic Factors in AML
- Age is not a consistent prognostic factor.
- Caucasians have a better prognosis than African-Americans.
- Poor prognosis is associated with monosomy 7, WBC count > 100,000/cmm, secondary AML, and poor outcome in M7 patients without Down syndrome.
- Recent studies suggest a good outcome with M3: Acute Promyelocytic Leukaemia.
- Prognosis in AML is improved with chemotherapy alone (50% cure) or chemotherapy with bone marrow transplantation (70% cure).
Childhood Lymphomas and Leukaemias
- Lymphomas are proliferative malignant disorders of lymphocytes and/or macrophage system.
- Classification of lymphomas includes Hodgkin, Non-Hodgkin, and Burkitt.
Burkitt Lymphoma
- Burkitt lymphoma is a malignant lymphoma of B lymphocyte origin.
- Endemic Burkitt lymphoma occurs in tropical Africa, while sporadic Burkitt lymphoma occurs in the Far East, Central and South America, and Europe.
- HIV-associated Burkitt lymphoma is a type of Burkitt lymphoma.
- Burkitt lymphoma is the fastest growing tumour in man, with a doubling time of 24 hours.
Endemic Burkitt Lymphoma
- Endemic Burkitt lymphoma is the commonest childhood malignant tumour in Nigeria.
- Aetiology of endemic Burkitt lymphoma involves EB virus, malaria infection, and chromosomal translocation 8;14, 8;22, 2;8.
- C-Myc oncogene (on chr 8) is critical in regulating cell proliferation.
Pathology of Burkitt Lymphoma
- FNAB cytology shows medium-sized cells with basophilic cytoplasm, fat vacuoles, and multiple nucleoli.
- Histology shows a "starry-sky" appearance.
Clinical Features of Burkitt Lymphoma
- Age range is 2-16 years, with a peak at 7 years.
- Male-to-female ratio is 1.7:1.
- Sites affected include the jaw (maxilla or orbit), abdomen, CNS, and other sites.
- Clinical features include jaw swelling, loosening of teeth, and facial tumour.
Staging and Investigations of Burkitt Lymphoma
- Staging includes A (solitary extra-abdominal site), AR (completely resected intra-abdominal tumour), B (multiple extra-abdominal sites), C (intra-abdominal tumour + facial tumour), and D (intra-abdominal tumour + sites other than facial).
- Investigations include tumour aspirate for cytology, FBC, bone marrow, E/U, Cr, uric acid, Ca, and P.
Treatment and Prognosis of Burkitt Lymphoma
- Treatment involves chemotherapy, radiotherapy, and surgery.
- Chemotherapy agents include IV cyclophosphamide, IV vincristine, IV cytosine arabinoside or methotrexate, and IT cytosine arabinoside or methotrexate.
- Prognosis is dependent on extent, stage, and age, with a survival rate of 80-95% in some reports.
Hodgkin Lymphoma
- Hodgkin lymphoma is a progressive, painless enlargement of lymph nodes.
- Three forms of Hodgkin lymphoma exist: childhood form (15 years), young adult form (15-34 years), and older adult form (>55 years).
- Age peaks are 15-35 years and >50 years.
Clinical Features of Hodgkin Lymphoma
- Male-to-female ratio is 1.7:1.
- Clinical features include painless, non-tender, firm, rubbery cervical or supraclavicular lymphadenopathy.
- Nodal or extranodal manifestations include airway obstruction, hepatic dysfunction, and systemic symptoms.
Pathology of Hodgkin Lymphoma
- EBV is involved in the aetiology of Hodgkin lymphoma.
- Reed-Sternberg cell and other lymphocytes, histiocytes, and plasma cells are present.
Investigation and Management of Hodgkin Lymphoma
- Diagnosis is made by LN biopsy.
- Staging investigations include FBC, LFT, CXR, imaging Abd/Chest, and B marrow.
- Treatment involves chemotherapy (MOPP, ABVD, COPP) and radiotherapy.
Non-Hodgkin Lymphoma
- Age range is 5-15 years, with a male-to-female ratio.
- Clinical features include abdominal, nodal, and extranodal manifestations.
- Treatment involves chemotherapy (CHOP) and irradiation.
Acute Leukaemias
- Acute leukaemias represent a clonal expansion and arrest at a specific stage of normal myeloid or lymphoid haematopoiesis.
- Acute leukaemias account for 97% of childhood leukaemias and consist of Acute Lymphoblastic Leukaemia (75%), Acute Myeloblastic Leukaemia (20%), and Acute Undifferentiated Leukaemias (25%).
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Description
This quiz covers the treatment and classification of leukemia, including acute myeloblastic leukemia and ALL. It also discusses cell morphology and differentials.
