By Angela Gascoigne Haematology CNS Chesterfield Royal Hospital

Transcription

1 By Angela Gascoigne Haematology CNS Chesterfield Royal Hospital

2 Myeloproliferative Neoplasms Essential Thrombocythaemia Polycythaemia Vera Myelofibrosis

3 Essential Thrombocythaemia (ET) Chronic condition characterised by too many platelets 2 to 3 cases per 100/000 per annum More common in women More prevalent over the age of 60

4 Symptoms of ET pruritus Enlarged spleen Fatigue Headache ET Easy bruising Epistaxis Night sweats Fever Weight loss Dizziness light headedness

5 Investigations FBC, ESR, CRP, Auto antibodies, JAK 2 CXR Abdominal USS ET Bone marrow if genetic mutation studies negative Ferritin and iron studies Genetic mutation studies MPL, CALR, BCR-ABL

6 Risk stratification Low risk Age under 40 years Platelet count less than 1500 No history of ischaemia, thrombosis or embolic features Absence of haemorrhagia considered related to ET Absence of diabetes Absence of hypertension Intermediate risk Age Platelet count less than 1500 No history of ischaemia, thrombosis or embolic events Absence of haemorrhagia considered related to EY Absence of diabetes Absence of hypertension High Risk Age 60 years or above Platelet count more than 1500 History of ischaemia, thrombosis or embolic features Haemmorhagia considered related to ET Presence of diabetes Presence of hypertension

7 Treatment options Low/Intermediate risk disease Asprin only Manage vascular risk factors Cytoreductive therapy only if symptomatic ; splenomegaly severe microvascular symptoms if not improving with asprin uncontrolled bleeding associated with high platelets High risk disease 1 st line Hydroxycarbamide (interferon in patients less than 40) 2 nd line anagrelide Other agents to consider ; Busulphan, P32 Consider relaxing platelet target to in patients intolerant/resistant to Hydroxycarbamide

8 Polycythaemia Vera(PV) chronic condition characterised by too many red cells and sometimes platelets and white cells 2 cases per 1000/000 per annum more common in men more prevalent over the age of 60

9 Symptoms of Polycythaemia Vera Reddish or purple skin Enlarged spleen Fatigue Headache Pruritus PV Easy bruising/ Epistaxis Night sweats Fever Weight loss Dizziness Light headedness

10 Investigations FBC, EPO Oxygen saturations, CXR Gene mutation studies JAK2,EXON 12, BCR-ABL PV Abdominal USS Bone marrow if genetic mutations negative

11 Management options Address risk factors Venesection: maintain haematocrit to less than 0.45 Asprin 75mg daily unless contraindicated Consider Cytoreduction therapy

12 Cytoreduction therapy Less than 40 1 st line Interferon 2 nd line Hydroxycarbamide or Anagrelide st line Hydroxycarbamide 2 nd line Interferon or Anagrelide Over 70 1 st line Hydroxycarbamide 2 nd line low dose Busulphan or 32P

13 Potential side effects of cytoreductive therapy Hydroxycarbamide Cytopaenias Mouth/skin ulcers Nausea/loss of appetite Diarrhoea Hair thinning Anagrelide Anaemia Headache Palpitations Tachycardia Nausea/loss of appetite Diarrhoea Busulphan Increased risk of other neoplasms particularly leukaemia Cytopaenias particularly thrombocytopaenia Nausea and vomiting Renal impairment

14 Potential side effects of venesection Vasovagal episodes Phlebitis Anaemia Iron deficiency

15 Myelofibrosis primary or secondary bone marrow replaced by fibrous(scar) tissue 1-2 cases per 100,000 per annum affects men and women equally most common between 60 and 70 years of age

16 Symptoms Fatigue Enlarge d spleen Sweats (predominant ly night sweats) Arthralgia (bone pain) Myalgia(muscle pain) MF Weight loss/feve r Pruritus

17 Investigations Abdomina l USS Bone marrow biopsy FBC Blood film MF Gene mutation studies: JAK 2, CALR, MPL, BCR-ABL

18 Prognostic Factors DIPSS score Variable Over 65 Constitutional symptoms Haemoglobin less than 100g/l White cell count more than 25 Circulating blasts DIPSS 1 point 1 point 2 points 1 point 1 point Risk group Predictors Median survival (years) Low 0 Not reached Intermediate 1 1 or Intermediate 2 3 or 4 4 High 5 or 6 1.5

19 Management options Anaemia Erythropoetin injections if reduced Epo levels Thalidomide/prednisolone Low risk Watch and wait Interferon if symptomatic Hydroxycarbamide for cytoreduction Intermediate 2 or High risk Ruxolitinib (if platelet count more than 50) Consider allogeneic transplant if aged less than 70

20 Thank you