haematological malignancies Flashcards
what is myelofibrosis?
excess bone marrow scarring due to abnormal megakaryocyte activity – leading to bone marrow failure
what does polycythaemia Rubra vera (PRV) present with?
excess red blood cells in blood
what does essential thrombocythaemia (ET) present with?
excess platelets in the blood
what can both PRV and ET progress to?
myelofibrosis
or
acute leukaemia
what gene problem causes both PRV and ET?
the janus kinase gene JAK2
what are the 3 main symptoms of bone marrow failure?
- anaemia
- thrombocytopenia (low blood platelet count)
- neutropenia (low level of neutrophils)
what is the treatment as soon as acute leukaemia is detected?
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
how do you treat acute lymphoblastic leukaemia (ALL)?
mix strong chemo and continuous tablets to prevent relapse
what is myelodysplasia?
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.
what causes the anaemia, low platelet and low WBC?
excess cell proliferation fills the bone marrow
squeezes normal bone marrow function out of the bone,
so patient now has bone marrow failure.
what does thrombocytopenia cause?
means low platelet count so excess bruising and bleeding
what do children with acute lymphoblastic leukaemia present with?
cytopenia (one or more types of blood cells are at low levels) and chest masses
what investigations do you carry out if someone is presenting with symptoms that suggest leukaemia
blood transfusion, antibiotics and platelet transfusion
bone marrow tests to determine what type of leukaemia etc
what happens in acute lymphoblastic leukaemia?
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.
Chronic Myeloid Leukaemia happens at what age usually?
most common in older adults around 60-65 years of age.
what is the difference between CML and AML?
Acute leukemia involves the immature cells, called stem cells,
whereas chronic leukemia develops in mature cells.
how does CML [chronic myeloid lymphoma] present with?
Priapism (painful erection)
Splenomegaly (enlarged spleen)
high WBC count
what causes the Chronic Myeloid Leukaemia?
Philadelphia chromosome
Gene fusion produces BCR-ABL fusion protein
what are some difference between CML and CLL?
CML also tends to affect younger individuals on average when compared with CLL
what is the difference between acute and chronic?
chronic- is a long developing disease which worsens over a long period of time
acute- these are sudden and aggressive upon onset
what is the difference between myeloid cells and lymphocytes?
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.
what is the difference between AML (acute myeloid leukaemia) and ALL (acute lymphocytic leukaemia)?
AML- starts in the WBC
ALL- starts in the lymphocytes (T cells, B cells + natural killer cells)
what cancer does the Philadelphia chromosome cause?
chronic myeloid leukaemia
what are uterine fibroids / uterine leiomyomas?
Uterine fibroids, also known as uterine leiomyomas or fibroids, are benign smooth muscle tumors of the uterus.
