 |
| Common symptoms of leukemia |
The majority of patients with acute leukaemia present with symptoms reflecting inadequate hematopoiesis secondary to infiltration of the bone marrow by leukaemia cells, symptoms due to tissue infiltration by leukaemic cells, the consequences of a high WBC or substance release from the tumours cells.
Investigation
- Blood count. Hb and platelets low, WBC raised.
- Blood film. Blast cells seen.
- Bone marrow aspirate.
- Chest X-ray. Mediastinal widening often present in T lymphoblastic leukaemia.
- Cerebrospinal fluid examination.
- Coagulation profile
Supportive care forms the basis of treatment whether for cure or palliation:
- Avoidance anemia - repeated transfusion.
- Prevention or control of bleeding
- Treatment of infections
- Prophylactically: Antibiotics, antifungals, antivirals
- Therapeutically - Control of hyperuricaemia with hydration, prophylactic allopurinol and rasburicase.
The initial requirement of therapy is to return the peripheral blood and bone marrow to normal (complete remission) using induction chemotherapy. Successful remission induction is always followed by further treatment (consolidation)
ACUTE MYELOID LEUKAEMIA (AML)
Treatment with curative intent is undertaken in the majority of adults below the age of 60 years, provided there is no significant c-morbidity. Treatment success reflects the cytogenic pattern.
Risk groups:
- Low risk - chemotherapy (anthracyclines)
- Intermediate risk - consolidation chemotherapy after intial remission, followed by allogenic transplantation
- High risk - stem cell transplant in complete remission 1.
ACUTE PROMYELOCYTIC LEUKAEMIA (APML)
APML is characterized by the translocation t(15;17). There is an almost invariable coagulopathy, which was a major cause early death. The discovery of all trans-retinoic-acid (ATRA) was a major breakthrough. APML is treated with ATRA combined with several courses of chemotherapy. Transplantation may be necessary either if the leukaemia is not eliminated at the molecular level or following recurrence and reinduction therapy. Arsenic trioxide is used with resistant or relapsed disease.
ACUTE LYMPHOBLASTIC LEUKAEMIA (ALL)
ALL may present in leukaemic phase with significant marrow involvement or may present as localized disease, typically a mediastinal mass. Remission induction is undertaken with combination chemotherapy including vincristine, a glucocorticoid, anthracycline and asparaginase. Intensive consolidation of remission with variable numbers of chemotherapy cycles , including high dose methotrexate. In patients with high-isk features or an HLA-matched sibling donor, allogenic transplantation is recommended upon achieving first complete remission.
ALL needs central nervous systems directed therapy. Prophylaxis should be given with intrathecal chemotherapy as soon as blasts are cleared from blood. Depending upon risk this may be continued for up to 2 years and complemented by high doses of systemic cytosine arabinoside and methotrexate.
Additionally after intensive induction and consolidation maintenance therapy for 2 years is required to reduce the risk of disease reoccurrence.
Prognosis
The prognosis of ALL in childhood is up tp 80% being alive without recurrence at 5 years. Failure occurs most frequently in those with high presentation blast counts or a t(9;22) translocation.
Adults, remission rate is 70-80%. Between 30-40% of patients continue in durable first remission, resulting in approximately 25-30% overall patients cure.
Imatinib used in conjuction with chemotherapy increases the response rate and quality of reponse in patients with the t(9;22) translocation and ALL.
0 comments :
Post a Comment