Leukemia Treatment and Classification

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%).