Myeloproliferative Neoplasms Flashcards
Give some clinical features of Polycythemia.
Thrombosis Haemorrhage (skin GI) Plethora Pruritis Erythomelalgia Splenomegaly Gout Arthritis
Outline treatment for PV.
What is the goal of treatment.
Phlebotomy - HC<45%
Aspirin
Hydroxycarbimide - cytoreduction
Why is hydroxycarbimide considered?
Splenomegaly Venesection intolerance Progressing symptoms (malaise and night sweats)
Give 2 broad reasons for increase EPO.
Hypoxia and increased EPO production
Name 3 ways in which thrombocytosis can occur.
Primary - essential thrombocythaemia - myeloproliferative neoplasm
Secondary - infection inflammation
Redistribution - splenic pool -> blood
What is the difference between primary and secondary myelofibrosis?
Primary - myelofibrosis alone
Secondary - due to PV or essential thrombocythaemia
Why do you get hepatosplenomegaly in primary myelofibrosis?
Mobilisation of mutated haemopoietic cells that colonise the spleen and liver
Originally from bone marrow
Name some symptoms of primary myelofibrosis.
Bruising Hepatosplenomegaly Bruising Fatigue Increased sweating Portal hypertension Fever Weight loss
What treatment is given in advanced disease?
Blood transfusions and splenectomy
Name a JAK2 inhibitor and why is it used to treat primary myelofibrosis.
Ruxolitinib
Reduce spleen volume and improve symptoms as a result.
What is the cause of CML?
Philadelphia chromosome
Reciprocal translocation between Chr 9 and 22.
Oncoprotein BCL-ABL results
What is the main cause of immune thrombocytopenic purpura in children?
Acute inflammation
What some causes of immune thrombocytopenic purpura in adults?
SLE
HIV
RA
Lymphoid cancers
Although 80% cases idiopathic
What are the treatment options for immune thrombocytopenic purpura?
Corticosteroids
IV pooled humans Ig’s
Immunosurpression
TPO receptor agonists
What is a common complication of immune thrombocytopenic purpura in adults?
Intracranial haemorrhage.
