Myeloproliferative disorders Flashcards
Types of myeloproliferative disorders
Polycythaemia - high RBC
Thrombocytosis - high platelets
Chronic myeloid leukaemia - high WCC
Myelofibrosis
Complications of thrombocytosis
Thrombosis - stroke/MI/DVT/PE
Haemorrhage - increased platelets bind and use up VWF
Splenomegaly and splenic rupture
Transformation to myelofibrosis
Transformation to AML
Treatment for thrombocytosis
Cytoreduction in high-risk patients using low dose chemotherapy - hydroxycarbamide
Pegasys - Pegalated interferon (immunotherapy) causes myelosuppression
Wait and see
Aspirin - dependent on platelet count
Pathophysiology of polycythaemia vera
Uncontrolled production of RBCs in bone marrow despite erythropoietin production being low
Commonly caused by JAK2 mutation
Presentation of polycythaemia vera
Thrombosis - stroke/MI/DVT/PE
Viscosity symptoms - headaches, visual disturbances, drowsiness
Aquagenic pruritis -especially after a warm bath
Plethroic skin complexion
Complications of polycythaemia vera
Transformation to AML
Myelofibrosis
Secondary causes of thrombocytosis
Bleeding - trauma
Iron deficiency
Inflammation
Infection
Post surgery
Splenectomy
Secondary causes of polycythaemia vera
Chronic hypoxia - altitude
Smoking
Obstructive sleep apnoea
Renal or hepatic tumours - produce more EPO
Congenital cyanotic heart disease
Anabolic steroids
Chronic lung conditions such as COPD
Investigations for myeloproliferative disorders
Bloods - FBC
Blood PCR - JAK 2 Kinase
Bone marrow biopsy
USS abdomen - spleen
Hydroxycarbamide mechanism of action
Myelosuppression
Side effects of hydroxycarbamide
Anaemia
Thrombocytopenia - bleeding
Neutropoenia - recurrent infections
Rash
Dry, peeling skin
Leg ulcers
Constipation or diarrhoea
GI discomfort
Hallucinations
Confusion
Secondary skin cancer
Treatment for polycythaemia vera
Cytoreduction in high-risk patients using low dose chemotherapy - hydroxycarbamide
Pegasys - Pegalated interferon (immunotherapy) causes myelosuppression
Venesection - used as adjunct or acutely
Stem cell transplant
Myelofibrosis features
Initial proliferative stage - high cell lines
Then RBC decreased with increased other cell lines
Then decreased platelets
How to treat nose bleeds in thrombocytosis
Tranexamic acid - antifibrinolytic
