Leukaemia and myeloid diseases Flashcards
What is myelodysplastic syndrome?
A clonal blood disorder characterised by ineffective haemopoiesis
Pre-leukaemic
How is myelodysplastic syndrome differentiated from acute leukaemia in bone marrow biopsy?
> 20% of marrow cells blast cells, its leukaemia
How is MDS treated
Need SCT to cure. But since it mostly affects the elderly, normally supportive e.g. blood transfusion, drugs such as azacitidine chemo
What are myeloprofilerative disorders?
Clonal blood disorders characterised by effective haemopoiesis
Megakaryocytes (+WBCs)= myelofibrosis
Red cells = polycythaemia vera
Platelets = essential thrombocytopenia
How are ET and PRV treated?
Aspirin to reduce risk of thrombotic events
Cytoreduction e.g. venesection, hydroxycarbamide
Can cause AML and myelofibrosis
How does myelofibrosis present?
Splenomegaly (haematopoeisis occuring), fatigue, elderly, systemic weight loss/night sweats
High urate and LDH due to cell turnover
How is myelofibrosis treated?
SCT is only curable
JAK2 inhibitors: often mutations involved
What is ALL/
Malignancy of lymphoid blast cells. Usually occurs in children, with good prognosis (worse if older)
Genetics implicated (translocations)
What are Sx of ALL?
Bone marrow failure = SOB, fatigue (anaemia); infections (low WCC); bruising and bleeding (low platelets).
infiltration: hepatosplenomegaly, lymphadenopathy, bone pain
Can be CNS and testicular infiltration
What are predisposing factors in ALL?
Downs
Fanconi
ionising radiation in pregnancy
Klinefelter
What Ix for ALL/
FBC: will show decreased; WCC high with severe neutropenia
Blood smear: leukaemic cells
Bone marrow biopsy: blast cells > 20%.
Also can do cytogenetics, routine bloods
imaging for any lymphadenopathy
Treatment for ALL?
Chemo regimen. BMT if younger
CNS prophylaxis.
Supportive: blood and platelets, allopurinol, fluids
Complications of ALL/
Febrile neutropaenia Tumour lysis syndrome Chemo s/e Leukostasis Steroid s/e
What is CLL?
malignant proliferation of a clone of mature B cells in peripheral blood
most common leukaemia
What are Sx of CLL?
Mostly infections - lack of functioning B cells. Also lymphadenopathy, anaemia, splenomegaly
What Ix for CLL?
FBC: often found on here as incidental high lymphocytes, w low neutrophils, anaemia, low platelets
Can be AIHA, ITP
Blood smear: spherocytes and polychromasia
Flow cytometry
What is Rx for CLL?
Watch and wait if asymptomatic.
Treat if symptomatic or progressing (chemo) - SCT only curable but not good in older patients ,
What are complications of CLL/
Richter tranformation to NHL
MAHA
ITP
Hypogammaglobulinaemia
What is AML
Clonal expansion of myeloid blasts in bone marrow and peripheral blood
De novo or following MDS or certain cytotoxic drugs
What is APML?
Subtype of AML with distinct cytological features e.g. normal WCC, bilobed nuclei, Auer rods.. treated differently and more aggressive
What is Sx of AML?
Same as for CLL (infiltration and bone marrow failure). APML can cause DIC.
What ix in AML:?
FBC: high WCC low neutrophils, everything low.
(urea and LDH high)
Blood smear - Auer rods differentiate from ALL
bone marrow biopsy needed for diganosis
Cytogenetics and immunophenotyping for classifying AML and tailoring treatment
What is Rx for AML?
Aggresive chemo
same as for CLL
What is CML
Malignant clonal disorder of mature myeloid cells in bone marrow
Philadelphia chromosome
What are Sx of CMl
may be none.
Fatigue, weight loss, night sweats, abdominal pain due to splenomegaly
Chronic, accelerated, then blast phase
What Ix in CML?
FBC: raised WCC, anaemia, platelets can vary depending on the phase.
Blood smear: myeloid cells. Basophils > 20% = accelerated phsae
Bone marrow biopsy needed for phase of disesae
Cytogenetics/FISH/PCR for t(9,22)
What is Rx for CML?
Imatinib: TKI
Can add in 2/3rd generations
Chemo if not working
What are complications of CML?
Related to TKI mainly e.g. muscle cramps, QTc, myelosuppression, rash
