Myeloid Leukaemias Flashcards
What is acute myeloid leukaeia?
Neoplastic proliferation of blast cells derived from the myeloid cell lineage. Rapidly progressing disease that must be treated urgently with death within 2 months if left untreated. 20% 3-year survival after treatment.
What are the risk factors for AML?
Increasing age Chemotherapy (e.g. for lymphoma) Myelodysplastic states (heterogenous group if disorders that result in marrow failure, most are primary but can occur secondary to chemo or radiotherapy, 1/3 transform to acute leukaemia) Radiation Down’s syndrome
How is AML classified?
Based on the cause of the leukaemia • Recurrent genetic abnormalities • Multilineage dysplasia secondary to myelodysplastic state • Therapy related • Other
What are the clinical features of AML?
How is it differentiated from ALL?
Marrow failure – anaemia, thrombocytopaenia and leukopenia (although WCC can be normal or raised)
DIC – in a certain subtype of AML that releases thromboplastin
Infiltration – Hepatomegaly, splenomegaly, gum hypertrophy and skin involvement
CNS involvement at presentation is rare.
Auer rods differentiate it from ALL on biopsy
AML can cause fever in itself
Leukostasis
How is AML investigated?
FBC Blood film Bone marrow biopsy and aspiration Immunophenotyping Cytogenetic analysis
How is AML managed?
Walking exercises to relieve fatigue
Chemotherapy – very intensive leading to prolonged states of neutropenia and thrombocytopenia
If remission or during refractory period Allogenic HLA matched bone marrow transplant
Prophylaxis for bacteria, viral and fungal infection is given during treatment.
Allopurinol for tumour lysis syndrome
Keep well hydrated
What complications can occur from AML and during treatment?
Be wary of infection especially of candida and aspergillus infections.
Be wary of leukostasis especially if very high WCC
What is chronic myeloid leaukaemia?
Uncontrolled clonal proliferation of myeloid cells. Type of myeloproliferative disorder.
What are the risk factors for CML?
It occurs most commonly between 40-60yrs
Male (slight)
Rare in childhood
Lack of the Philadelphia chromosome (BCR-Abl oncogene due to t(9:22) has a worse prognosis however it is usually present in >80%
What are the clinical features of CML?
Weight loss Tiredness Fever Sweats Raised urate leading to Gout (purine breakdown) Marrow failure leading to Anaemia (although can be normal) and Thrombocytopenia Splenomegaly and Hepatomegaly Hypercellular bone marrow
What investigations should be done for someone with CML?
FBC (high WCC due to neutrophils and metamyelocytes) note steroids cause neutrophilia
Blood film
Bone marrow biopsy and aspiration
Cytogenetic analysis of blood or bone marrow for Philadelphia chromosome
What are the 3 phases of CML?
Chronic – lasting months or years with few if any symptoms
Accelerated with increasing symptoms, spleen size and difficulty controlling counts
Blast transformation – progression to AML
How is CML managed?
Hydroxycarbamide often used – note increased MCV
Imatinib – personalised treatment for those who are Ph positive (BCR-ABL tyrosine kinase inhibitor). There are new 2nd gen versions which can offer a deeper treatment
Lymphoblastic transformation should be treatment as ALL and myeloblastic transformation with chemotherapy although this rarely achieved remission and allogenic stem cell transplant is the only cure.
What differentials should be considered for someone with a raised neutrophil count?
Bacterial infection Inflammation MPNs Drugs (steroids) Disseminated malignancy
What differentials should be considered for someone with a lowered neutrophil count?
Viral infections Drugs – post chemo, carbimazole, sulphonamides Severe sepsis Neutrophil antibodies (rare) Hypersplenism Bone marrow failure
