myeloid cancers Flashcards
How can polycythemia and myeloproliferative disorders be classified?
Polycythemia can be relative (lack of plasma) or true (due to excess rbc)
Myeloproliferative neoplasms can be philadelphia positive or negative
If there is polycythemia aka raised haemoglobin count or raised haematocrit, what do you need to think?
45-48 percent should normally be red blood cell
Is there pseudopolycythemia aka a relative deficiency of plasma
Dilution studies or Fick principle seeks to understand the ratio of albumin (plasma) to rbc ratio by radiolabelling
What might cause pseudopolycythmia?
Alcohol
Obesity
Diuretics
If we have established that there is polcythemia, how do we subclassify it?
Secondary - raised EPO
Primary - low EPO
EPO can be raised appropriately or inappropriately. What are causes of appropriate high EPO?
High altitude (low oxygen causing chronically high EPO)
Hypoxic lung disease
Cyanotic heart disease (left to right shunt)
High affinity haemoglobin (which fails to release oxygen)
EPO can be raised appropriately or inappropriately. What are causes of appropriately high EPO?
High altitude (low oxygen causing chronically high EPO)
Hypoxic lung disease
Cyanotic heart disease (left to right shunt)
High affinity haemoglobin (which fails to release oxygen)
EPO can be raised appropriately or inappropriately. What are causes of inappropriate high EPO?
Renal disease - cysts, tumours e.g. medulloblastomas (renal cancers) or inflammation
Uterine myoma
Other tumorus
What can haematopoetic stem cells
Common haematopoetic stem cell can become granulocyte macrophage precursor, megakarocyte precursor and also red blood cell precursors
Polycythemia vera is associated with which mutaiton?
JAK2 - myelofibrosis
Others include calcitrieun??????? and mpl????????
diagnosis of
Note:organomegaly is the key clnicla
Hepatosplenomegaly
FBC
BM biopsy
EPO
??
Symptoms of PCV
Headaches
Light headedness
TIA
storke
Visual disturbances
Fatigue
Dysnpoea
Increased histamine released
Pepetic ulceration
Aqua pruritis (after a hot bath)
Principles of treatment in PCV
reduce HCT through venesection or cytoreductive therapy - hydroxycarbamide
reduce risk of thrombosis
Principles of treatment in PCV (myeloproliferative disorder of erythrocytes)
reduce HCT through venesection or cytoreductive therapy - hydroxycarbamide
reduce risk of thrombosis
Essential thromobcythemia (myeloproliferative disorder of platelets) epidemiology
Peaks in 55 or 30 years of age
M=F
e.g. around 400/500 platelets for no reason - picked up on normal scan
ET symptoms
Hypercoagulability
Strokes
Gangrenes
TIAs
Bleeding due to defects - mucous membrane and cutaneous bleeding
Modest enlargement of spleen
ET treatment
Give aspirin to prevent thrombosis
Hydroxycarbamide to bring platelet count down
Anagrelide - specific inhibition of platelets
What is the prognosis of ET
5 percent chance of acquiring new mutations (leukemic transformation)
prognosis good
Phil negative myeloproliferative disorder - PRIMARY MYELOFIBROSIS
Abnormal myeloproliferation associated with fibroblast growth stimulating factor, causing reactive bone marrow fibrosis
Phil negative myeloproliferative disorder - PRIMARY MYELOFIBROSIS
Abnormal myeloproliferation associated with fibroblast growth stimulating factor, causing reactive bone marrow fibrosis
m=f
main clinical symptom of primary myelofibrosis and why
MASSIVE SPLENOMEGALY
- budd chiari syndrome
as the BM starts to have collage deposition, so haemopoesis starts to occur in other organs where it can happen - e.g. liver and spleen
e.g. as fetuses we made blood cells in the liver as kids
Dry bone tap
Primary myelofibrosis treatments
Not great
Supportive treatment - RBCs, platelet transfusion ineffctive due to splenomegaly
Philadelphia POSITIVE myeloproliferative disorders
CML
- radiation is a major risk factor
CML presentation
Tiredness
Hypermetabolism
Thrombotic events
Monocular blindness
Bleeding upon bruising
Examination of CML
Massive splenomegaly
Hepatomegaly
