haematological malignancies

Question 1

what is myelofibrosis?

Answer 1

excess bone marrow scarring due to abnormal megakaryocyte activity – leading to bone marrow failure

Question 2

what does polycythaemia Rubra vera (PRV) present with?

Answer 2

excess red blood cells in blood

Question 3

what does essential thrombocythaemia (ET) present with?

Answer 3

excess platelets in the blood

Question 4

what can both PRV and ET progress to?

Answer 4

myelofibrosis
or
acute leukaemia

Question 5

what gene problem causes both PRV and ET?

Answer 5

the janus kinase gene JAK2

Question 6

what are the 3 main symptoms of bone marrow failure?

Answer 6

• anaemia
• thrombocytopenia (low blood platelet count)
• neutropenia (low level of neutrophils)

Question 7

what is the treatment as soon as acute leukaemia is detected?

Answer 7

commence strong intravenous chemo immediately in short snap bursts
(kills 99% of what is in the bone marrow) apart from hopefully healthy dormant stem cells, hopefully these divide into a whole newline marrow (3 weeks) do more tests to see if it is healthy.
if not healthy do more chemo

Question 8

how do you treat acute lymphoblastic leukaemia (ALL)?

Answer 8

mix strong chemo and continuous tablets to prevent relapse

Question 9

what is myelodysplasia?

Answer 9

Myelodysplastic syndromes (MDS) are a group of cancers in which immature blood cells in the bone marrow do not mature and therefore do not become healthy blood cells.

Question 10

what causes the anaemia, low platelet and low WBC?

Answer 10

excess cell proliferation fills the bone marrow
squeezes normal bone marrow function out of the bone,
so patient now has bone marrow failure.

Question 11

what does thrombocytopenia cause?

Answer 11

means low platelet count so excess bruising and bleeding

Question 12

what do children with acute lymphoblastic leukaemia present with?

Answer 12

cytopenia (one or more types of blood cells are at low levels) and chest masses

Question 13

what investigations do you carry out if someone is presenting with symptoms that suggest leukaemia

Answer 13

blood transfusion, antibiotics and platelet transfusion

bone marrow tests to determine what type of leukaemia etc

Question 14

what happens in acute lymphoblastic leukaemia?

Answer 14

large numbers of white blood cells are released BEFORE THEY ARE READY. These are known as BLAST CELLS.
increase in blast cells= the no. of red blood cells + platelet cells decreases.
blast cells are less effective than mature white blood cells at fighting bacteria and viruses, making you more vulnerable to infection.

Question 15

Chronic Myeloid Leukaemia happens at what age usually?

Answer 15

most common in older adults around 60-65 years of age.

Question 16

what is the difference between CML and AML?

Answer 16

Acute leukemia involves the immature cells, called stem cells,
whereas chronic leukemia develops in mature cells.

Question 17

how does CML [chronic myeloid lymphoma] present with?

Answer 17

Priapism (painful erection)
Splenomegaly (enlarged spleen)
high WBC count

Question 18

what causes the Chronic Myeloid Leukaemia?

Answer 18

Philadelphia chromosome

Gene fusion produces BCR-ABL fusion protein

Question 19

what are some difference between CML and CLL?

Answer 19

CML also tends to affect younger individuals on average when compared with CLL

Question 20

what is the difference between acute and chronic?

Answer 20

chronic- is a long developing disease which worsens over a long period of time
acute- these are sudden and aggressive upon onset

Question 21

what is the difference between myeloid cells and lymphocytes?

Answer 21

myeloid cells are cells that become WBC (neutrophils, monocytes, macrophages) myeloid dendritic cells, RBC, platelet-making cells and mast cells.
whereas,
Lymphoid cells include T cells, B cells, and natural killer cells.

Question 22

what is the difference between AML (acute myeloid leukaemia) and ALL (acute lymphocytic leukaemia)?

Answer 22

AML- starts in the WBC

ALL- starts in the lymphocytes (T cells, B cells + natural killer cells)

Question 23

what cancer does the Philadelphia chromosome cause?

Answer 23

chronic myeloid leukaemia

Question 24

what are uterine fibroids / uterine leiomyomas?

Answer 24

Uterine fibroids, also known as uterine leiomyomas or fibroids, are benign smooth muscle tumors of the uterus.

Question 25

what is a tubular adenoma of the colon?

Answer 25

Tubular adenomas are the most common types of colon polyps doctors remove during a colonoscopy. This type of polyp can become cancerous, and that danger goes up the bigger the polyps get.

Question 26

what is a pituitary adenoma, is it benign?

Answer 26

Most pituitary adenomas are slow-growing and benign, which means they are not cancer and do not spread to other parts of the body.
not classed as brain tumours or CNS tumours