acute leukaemia Flashcards
what is it?
proliferation of abnormal progenitors with block in differentiation/maturation causing a rapidly progressive clonal malignancy of the marrow/blood with maturation defects
excess of blasts in either the peripheral blood or bone marrow
decrease/loss of haemopoitic reserve
types of acute leukaemia?
Acute Myeloid Leukaemia (AML)
o More common in the elderly >60 years
o May be de novo or secondary
Acute Lymphoblastic Leukaemia (ALL)
o ALL is a malignant disease of primitive lymphoid cells (lymphoblasts)
o Most common childhood cancer
how do acute leukaemias present?
(more commonly than chronic leukaemia) with failure of normal marrow function
how does ALL (acute lymphoblastic leukaemia) present?
o due to marrow failure (anaemia, infections, bleeding)
o leukaemic effects:
- high count with obstruction of circulation,
- involvement of areas outside the marrow and blood (extra-medullary) e.g. CNS, testis
o Bone pain
how does AML (acute myeloid leukaemia) present?
o Presentation can be similar to ALL (marrow failure)
o Subgroups of AML may have characteristic presentation
- Coagulation defect - DIC in acute promyelocytic leukaemia
- Gum infiltration
how is it investigated?
- Apply principles from normal haemopoiesis to identify abnormal
- Blood count and film
- Coagulation screen
- Bone marrow aspirate
- Diagnosis is important as AML and ALL have different treatment
what findings on blood count and film suggest acute leukameia?
reduction in normal
presence of abnormal - abnormal cells with high nuclear:cytoplasmic ratio
what investigations are carried out on bone marrow aspirate to diagnose acute leukaemia?
o Morphology
o Immunophenotype (by flow-cytometry) – lineage specific proteins on the cell surface?
- Lineage specific proteins and immunophenotyping are needed for a definitive diagnosis and has largely replaced cytochemistry
o Cyto/molecular genetics
- Diagnostic utility
- Prognostic significance
o Trephine (piece of bone) enables better assessment of cellularity and helpful when aspirate sub-optimal
how is acute leukaemia cured?
multiagent chemotherapy
How is ALL cured?
o Can last up to 2-3 years
o Different phases of treatment of varying intensity
o Targeted treatments in certain subsets
how is AML cured?
o Between 2-4 cycles of chemotherapy (5-10 days of chemotherapy followed by 2-4 weeks of recovery)
o Prolonged hospitalisation
o Target treatments in subsets
problems of marrow suppression
• Anaemia • Neutropenia o Infections – severity and duration - Gram negative bacteria can cause fulminant life-threatening sepsis in neutropenic patients - Bacterial infections are common but patients are susceptible to fungal infections • Thrombocytopenia o Bleeding - Purpura - Petechiae
complications of treatment?
- Nausea and vomiting
- Hair loss
- Liver, renal dysfunction
- Tumour lysis syndrome (during first course of treatment)
- Infection
- Late effects (eg loss of fertility, cardiomyopathy with anthracyclines)
infections which can be caused by treatment?
o Bacterial: empirical treatment with broad spectrum antibiotics (particularly covering Gram negative organisms) as soon as neutropenic fever (IMPORTANT!)
o Fungal (if prolonged neutropenia and persisting fever unresponsive to anti-bacterial agents) (IMPORTANT!)
o Protozoal e.g PJP (more relevant in ALL therapy)
what is the prognosis?
- Many patients will go into remission (<5% marrow blasts with recovery of normal haemopoiesis)
- Unfortunately remissions might not be durable depending on the type of acute leukaemia and many patients will relapse
- Some patients die of treatment-related toxicity