Definition
Leukaemia is the uncontrolled production of white cells at different
stages of maturation. There are two main types of leukaemia depending on
the type of white cell affected. These are lymphoid leukaemia and myeloid
leukaemia. Each can further be divided into acute (where the patient falls
suddenly ill) and chronic (where the patient may have been harbouring the
disease for months and occasionally years without knowing).
Leukaemia may therefore present as Acute Lymphoid Leukaemia (ALL),
Chronic Lymphoid Leukaemia (CLL), Acute Myeloid Leukaemia (AML) and
Chronic Myeloid Leukaemia (CML).
ALL is commonest in children especially boys, CLL is commonest in the
elderly, AML and CML cut across all age groups and sexes.
CAUSES
· Usually no cause in any particular case
· Viruses e.g. Human T Lymphotrophic Virus Type 1 (HTLV-1) and Epstein
Barr Virus (EBV)
· Chemicals e.g. benzene, industrial solvents, pesticides (lindane), dyes,
· Drugs e.g. alkylating agents such as Melphalan
· Radiation
SYMPTOMS
Acute leukaemia
· Fever
· Easy fatigability
· Bruising tendencies
· Bone and joint pain especially in children
Chronic leukaemia
· Asymptomatic
· Dragging sensation left side of abdomen
· Easy satiety
HAEMATOLOGICAL DISORDERS
· Weight loss
· Generalized itch
· Lymph nodal swellings
SIGNS
Acute leukaemia
· Pallor
· Fever
· Skin and mucosal haemorrhages
· Gum hypertrophy (AML subtype 5)
· Firm, rubbery, non-tender lymph nodes (lymphoid leukaemia)
· Splenomegaly
Chronic leukaemia
· Splenomegaly, often massive in CML
· Generalized lymph node enlargement in CLL
INVESTIGATIONS
· FBC and blood film comment
· Chest X-ray
· Bone marrow aspirate and trephine biopsy
· BCR-ABL rearrangement in ALL and CML
TREATMENT
Treatment objectives
· To aim for a cure for ALL in children
· To aim for a cure for good prognostic cases of ALL in adults
· To achieve remission and prolong good quality life in AML
· To control white cell counts and symptoms and prolong good quality life
in CML
Non-pharmacological treatment
· Watchful waiting or careful observation in CLL
· Good hydration
Pharmacologic treatment
(Evidence rating: A)
Supportive treatment:
· Red cell and platelet transfusions as needed
· Antibiotics in case of infections
· Filgrastim for situations of severe neutropenia
HAEMATOLOGICAL DISORDERS
· Metoclopramide hydrochloride to counteract nausea during
chemotherapy
· Allopurinol to reduce the risk of tumour lysis syndrome
· Ranitidine or Omeprazole for gastric protection
Specific treatment:
· Refer to specialist for combination chemotherapy
Acute Lymphoid Leukaemia (ALL)
Active agent
· Vincristine, IV,
· Crisantaspase, IV, IM or SC,
· Daunorubicin, IV,
· Cyclophosphamide, IV,
· Methotrexate, IV, IT and oral,
· Folinic acid, IV and oral,
· Mercaptopurine, oral,
· Cytarabine, IV, SC and IT,
· Prednisolone, oral,
· Dexamethazone, oral,
Acute Myeloid Leukaemia (AML)
· Daunorubicin, IV,
· Cytarabine, IV, SC, IT,
· Mitoxantrone IV,
· Etoposide IV, oral,
Chronic Lymphoid Leukaemia (CLL)
· Chlorambucil oral with or without prednisolone
Alternative treatment
· Cyclophosphamide, oral,
· Combination chemotherapy with cyclophosphamide, IV or oral,
vincristine IV and prednisolone oral.
Chronic Myeloid Leukaemia (CML)
· Imatinib, oral,
Alternative treatment
· Hydroxycarbamide, oral,
· Busulphan, oral,
HAEMATOLOGICAL DISORDERS
REFER
Refer all patients to tertiary centre for further evaluation and
management. Follow-up can continue at a regional centre by a physician
under the distant guidance of a haematologist in the case of chronic
leukaemias. From the tertiary centre, patients with a good chance of cure
by bone marrow transplant and who have a stem cell donor should be
referred to the appropriate centre.