Leukemia Treatment and Classification

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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?

50%

> 80% (correct)

70%

90%

What is the classification system for Acute Myeloblastic Leukemia (AML)?

FAB: M0-M7 (correct)

WHO: M1-M6

FAB: M0-M5

WHO: M1-M7

What is the prognosis for Acute Myeloblastic Leukemia (AML) patients with Monosomy 7?

No effect on prognosis

Uncertain prognosis

Good prognosis

Poor prognosis (correct)

What is the treatment for Acute Myeloblastic Leukemia (AML)?

Daunorubicin, Ara-C, Etoposide Signup and view all the answers

What is the cure rate for Acute Myeloblastic Leukemia (AML) patients treated with chemotherapy alone?

50% Signup and view all the answers

What is the significance of WBC count > 100,000/cmm in Acute Myeloblastic Leukemia (AML)?

Poor prognosis Signup and view all the answers

What is the significance of the loss of dental lamina dura in jaw X-rays?

Osteolytic lesions Signup and view all the answers

What is the primary treatment approach for the disease?

Chemotherapy Signup and view all the answers

What is the age group with a worse prognosis?

> 13 years Signup and view all the answers

What is the significance of CSF cytology in investigations?

Detection of cytology in CSF Signup and view all the answers

What is the characteristic of lymph nodes in Hodgkin Lymphoma?

Firm and rubbery Signup and view all the answers

What is the significance of B symptoms in Hodgkin Lymphoma?

Important for staging Signup and view all the answers

What is the age range for the young adult form of Hodgkin Lymphoma?

15-34 years Signup and view all the answers

What is the significance of urinary uric acid levels in investigations?

Detection of kidney dysfunction Signup and view all the answers

What is the characteristic of Burkitt lymphoma in terms of growth rate?

It has a 24hr doubling time Signup and view all the answers

What is thecommonest childhood malignant tumour in Nigeria?

Endemic Burkitt lymphoma Signup and view all the answers

What is the role of C-Myc oncogene in Burkitt lymphoma?

It is critical in regulation of cell proliferation Signup and view all the answers

What is the typical age range for Burkitt lymphoma?

2-16 years Signup and view all the answers

What is the characteristic cytological feature of Burkitt lymphoma?

Medium sized cells with basophilic cytoplasm containing fat vacuoles Signup and view all the answers

What is the clinical feature of Burkitt lymphoma in the jaw?

Loosening of teeth Signup and view all the answers

What type of cells are present in Reed-Sternberg cells?

Lymphocytes, histiocytes, and plasma cells Signup and view all the answers

What is the most common type of Acute Leukaemia in childhood?

Acute Lymphoblastic Leukaemia Signup and view all the answers

What is the main diagnostic test for Hodgkin lymphoma?

LN Biopsy Signup and view all the answers

What is the age range for Non-Hodgkin Lymphoma?

5-15yrs Signup and view all the answers

What is the chemotherapy regimen for Non-Hodgkin Lymphoma?

CHOP Signup and view all the answers

What is the definition of Acute Myeloblastic Leukaemia according to WHO?

Blasts >20% or clonal cytogenetic abnormalities Signup and view all the answers

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.