Myeloproliferative Disorders Flashcards
What is meant by myeloproliferative?
Clonal proliferation of haematopoietic myeloid stem cells in the bone marrow which retain the ability to differentiate into the RBCs, WBCs and platelets causing an excess.
What is raised RBC called?
What type of cancer is caused by raised WBC?
What is raised platelets called?
What are raised fibroblasts called?
Polycythemia vera
Chronic myeloid leukaemia
Essential thrombocythaemia
Myelofibrosis
Polycythaemia vera:
Can be asymptomatic.
There are types of emergency presentations. What are they?
Why could they get GI haemorrhaging?
Why do they get headaches?
Thrombosis (e.g. stroke, PE)
GI haemorrhage
Sometimes polycythemia vera prompts your body to make extra platelets. They normally help your blood clot, but the extra ones in polycythemia vera don’t always work well. They prevent your blood from clotting like it should. That can make you bleed too easily.
An increase in blood viscosity induces disturbed microcirculation, resulting in headaches with clinical symptoms.
Polycythaemia vera:
Why do they get an itch?
What may you notice on their face?
Why do they get burning in their fingers/toes?
Why do they get splenomegaly?
Due to the high turnover of red blood cells results in higher-than-normal uric acid production. What could this lead to?
Many things can cause itching. It can happen with polycythemia vera because the extra red blood cells prompt your immune system to release a chemical called histamine. This is the same chemical your body releases during an allergic reaction. Histamine makes your skin itch.
Redness
Also, the blood may be thick and gummy, causing it to clot in tiny blood vessels. If clotting occurs in the tiny blood vessels of the fingers and toes, a patient may experience numbness or burning.
The increased number of blood cells caused by polycythemia vera makes your spleen work harder than normal, which causes it to enlarge.
Gout
Polycythaemia vera:
Investigations:
FBC is obviously used to detect it. What can be used to look specifically at the conc of RBCs in the blood?
What does smoking do to the dissociation curve?
What else may cause PV which you may have to think about?
Management:
- What can be done to get rid of too much RBC?
- What drug can also be given to thin the blood?
- Hydroxycarbamide (hydroxyurea) can be used in severe disease. How does it work?
- CVD risk is also managed!
Pseudo-polycythemia is also seen which is due to a reduction in plasma volume. What 3 things could cause this?
What could this possibly develop into which would be more sinister?
Haematocrit
Cigarette smoking seems to cause a generalized upward shift of the hemoglobin distribution curve, which reduces the utility of hemoglobin level to detect anemia.
Increased number of erythrocytes and values of hematocrit in male smokers can be explained by the fact that tissue hypoxia caused by increased creation of carboxy hemoglobin leads to an increased secretion of erythropoietin, thus increasing erythropoiesis.
Venesection twice a wk and replaced with normal saline
Aspirin
Hydroxycarbamide is one of a group of chemotherapy drugs known as antimetabolites. These drugs stop cells making and repairing DNA. Cancer cells need to make and repair DNA in order to grow and multiply.
Myeloproliferative diseases can be classed as cancer
Dehydration
Alcohol
Diuretics
Risk of transformation to MF or acute leukaemia
Essential thrombocytosis (thrombocythaemia):
THIS IS ESSENTIALLY CLASSED AS CANCER!
What cells responsible for platelet production proliferate in the bone marrow?
S+S - may be asymptomatic:
- Where can they get bleeding? Why can they get bleeding as opposed to thrombosis ABOVE A CERTAIN LEVEL?
- Why do they get a headache and dizziness?
- Why do they get chest pain?
Megakaryocytes
GI bleeding
The extra platelets made don’t work as well which prevents your body from clotting properly. - UNDER THAT LEVEL, IT CAUSES THROMBOSIS!
Sometimes polycythemia vera prompts your body to make extra platelets. They normally help your blood clot, but the extra ones in polycythemia vera don’t always work well. They prevent your blood from clotting like it should. That can make you bleed too easily.
An increase in blood viscosity induces disturbed microcirculation, resulting in headaches with clinical symptoms.
Due to occlusion of coronary vessels - angina
Essential thrombocytosis (thrombocythaemia):
S+S:
- They get erythomelalgia in their fingers and toes. What is it?
They also get splenomegaly.
What is used to confirm this diagnosis?
Management:
- What drug is given to those who are young, asymptomatic and low risk?
- Hydroxycarbamide (hydroxyurea) can be used in severe disease. How does it work?
Vascular peripheral pain disorder in which blood vessels, usually in the lower extremities or hands, are episodically blocked (frequently on and off daily), then become painful (burning pain) and inflamed.
Also, the blood may be thick and gummy, causing it to clot in tiny blood vessels. If clotting occurs in the tiny blood vessels of the fingers and toes, a patient may experience numbness or burning.
Bone biopsy
Aspirin
Hydroxycarbamide is one of a group of chemotherapy drugs known as antimetabolites. These drugs stop cells making and repairing DNA. Cancer cells need to make and repair DNA in order to grow and multiply.
Myeloproliferative diseases can be classed as cancer
Myelofibrosis:
This is a type of cancer where you have marrow fibrosis leading to bone marrow failure. It is more severe than ET and PV.
What develops because of this? - 3
What symptoms would they get?
Why do they get massive hepatosplenomegaly?
They get hypermetabolic symptoms - give some examples?
What is there an increased risk of?
What type of cells are seen on blood film?
Management is usually supportive. What sort of support is given, knowing what myelofibrosis is?
Anaemia
Increased/decreased platelets
Increased/decreased WBC’s
Symptoms - tiredness, SOB etc.
Haemtopoeisis in the spleen and liver because they need to take over production
Night sweats
Fever
Weight loss
Abdo discomfort
Transformation to leukaemia
Epo injections or transfusion for anaemia
Poikilocytes (tear-shaped red blood cells)
Hydroxycarbamide for high platelets/WCC - remember, it can go either way for platelets or WBC’s
Neutrophillia:
Neutrophils can be raised due to infection/inflammation.
Why can malignancy cause neutrophilia?
What medication causes this?
What organ if dysfunctional, would lead to this?
Also caused by smoking, post-chemo etc.
What can done to confirm an infection/inflammation?
What can be done to look for another cause if the stimulus has passed and it is still high?
Due to release of cytokines
Steroids
Hyposplenism
Inflammatory markers - ESR/CRP
Blood film
