Blood cancer Flashcards
Myeloproliferative states
Group of blood conditions characterised by abnormal proliferation of myeloid cells.
- Commonly have JAK-STAT mutation (JAK2)
Types
- Polycythemia vera= RBCs
- Essential Thrombocythemia= thrombocytes
- Primary myelofibrosis state= megakaryocytes
- CML= WCCs
Polycythemia vera
- Definition
- Features
Definition
- Myeloproliferative state characterised by clinical proliferation of RBCs
- Highly associated with JAK2 V617F mutation
Features
- Hyperviscous blood= thrombosis, headaches, blurred vision
- Splenomegaly
- Hepatomegaly
- Gout (increased RBC breakdown= urea)
- Erythromelalgia
Diagnosis of polycytemia vera
- Raised RBC, Hb, Hct
- Raised WCC, Plts
- BM biopsy= hypercellular erythroid marrow
- Low EPO
Treatment of polycythemia vera
Thrombosis prophylaxis
- Aspirin
Venesection
High risk of thromobosis=
- hydroxycarbamide
- Ruxolitinib (JAK 2 inhibitor)
Essential thrombocythemia
- Definition
- Features
Myeloproliferative state
- Proliferation of circulating thrombocytes
Features
- Bleeding
- Thrombosis
- Splenomegaly
- Erythromelalgia
Essential thrombocythemia
- Treatment
Low risk do not need treatment
High risk (>60, thrombosis/ bleeding history) 1. Thrombosis prophylaxis= aspirin
- Cytoreductive drug= hydroxycarbamide
Primary myelofibrosis
- Defintion
- Features
Myeloproliferative state characterised by abnormal production of:
- RBC
- WBC
- Platelets
Causes marrow fibrosis and extra medullary haematopoeisis
Features
- Hallmarks: leukoerythroblasosis, splenomegaly
- Hepatomegaly
- BM failure= anaemia, infections, bleeding
Primary myelofibrosis treatment
Curative= allogenic STEM
Cytoreductive drugs
- If symptomatic
- Ruxolitinib/ hydroxycarbamide
Supportive
- Allopurinol
- Folic acid
NO thrombocytosis
- Thilaidomide/ INF alpha 2b
- Splenectomy/ splenic irradiation
CML
- Definition
- Features
Clonal disorder of haematopoeitic stem cells
- Myeloid hyperplasia in BM
- Philadelphia chromosome mutation t(9;22)= causes BCR-ABL fusion (tyrosine kinase)
- Causes abnormal expansion of myeloid cells in BM and periphery
- Can transform into AML
Features
- Splenomegaly
- Fatigue, dyspnoea
- Bleeding
CML treatment
Imantinib
- Inhibitor of BCR-ABL tyrosine kinase
Hydroxycarbamide
Curative
- Allogenic SCT (HLA-matched sibling/ unrelated donor)
- Chemo/ irradiation before transplant
AML
- definition
- Symptoms and signs
Clonal disorder of myeloid blast cells
- Pushed out into periphery
Causes
- Chromosomal abnromalites
(trisomy 21, t(15,17) )
- chemo/ radiothearapy
Symptoms and signs
- Marrow failure: anaemia symptoms, infection, bleeding, DIC
- Infiltration: Hepatomegaly, splenomegaly, gum hypertrophy
AML treatment
- Curative
- Supportive
- Infection prophylaxis and treatment
Curative
- Autogenic/ allogenic BMT
- Cyclophosphamide + total body irradiation before donor infusion
Supportive
- Allopurinol
- Blood products transfusion
Infection prophylaxis
- Ciprofloxacin
Infection treatment=
Tazocin/ gentamicin
Chemotherapy
- Induction then post-induction/ consolidation
AML classifications
M2 granulocyte maturation
M3 Acute promyelocytic leukaemia (t(15;17)
M4 Aute myelomonocytic leukaemia
M7 megakartoblastic leukaemia (in down’s)
Diagnosis of AML
- BM failure, cytopenia, blasts (>20%), Auer rods
- Bloods Hyperviscous, leucocytosis (esp blasts), anaemia, thrombocytopenia
- Flow cytometry
