3 Haematology Flashcards
what are the main sites or haemopoiesis at different stages of life?
Foetus 0-2 months: yolk sac 2-7 months: liver, spleen 5-9 months - infant: all bone marrow Adults: bone marrow of central skeleton (mainly) (vertebrae, ribs, sternum, skull, sacrum, pelvis, ends of femurs)
Outline the general pathway of haemopoiesis
haematopoietic stem cell
->
myeloid stem cell / lymphoid stem cell
->
“blast” cells (myeloblasts)
->
mature WBCs and RBCs
where are haemopoietic stem cells found?
- bone marrow
- peripheral blood after treatment with G-CSF (granulocyte Colony Stimulating Factor)
- Umbilical Cord Blood
Name a few conditions affecting bone marrow function and briefly summarise each
Hereditary:
- Thalassaemia
- Sickle Cell Anaemia
- Fanconi Anaemia
Acquired:
- Aplastic anaemia
- Leukaemia
- Myelodysplasia
- Chemotherapy
- Infections (TB + HIV)
what is thalassaemia?
Inherited blood disorder characterised by abnormal haemoglobin production.
Signs:
Anaemia (tiredness and palor)
Sometimes bone problems, splenomegaly, jaundice, and dark urine
Aplastic anaemia?
Aplastic anaemia is a rare disease in which the bone marrow and the hematopoietic stem cells that reside there are damaged. This causes a deficiency of all three blood cell types (pancytopenia): red blood cells (anemia), white blood cells (leukopenia), and platelets (thrombocytopenia).
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. Early on, there are typically no symptoms. Later symptoms may include feeling tired, shortness of breath, easy bleeding, or frequent infections.
What are myeloproliferative disorders? (and an example)
clonal disorders of haemopoiesis leading to increased numbers of one or more mature blood progeny
(polycythaemia rubra vera
Essential Thrombocytosis,
Myelofibrosis)
What is the haematocrit?
Haematocrit is a measure of the number of red blood cells in the blood. The value is expressed as a percentage or fraction of cells in blood
Essential Thrombocytosis?
increase in number and size of circulating platelets.
Polychthaemia rubra vera
↑ RBC
Myelofibrosis
Scarring + fibrous tissue in marrow
Fanconi Anaemia
impaired response to DNA damage. Bone marrow failure.
- Microphthalmia (eyes)
- GU and GU malformations
- mental retardation
- Hearing loss
What the flip is Hodgkin’s lymphoma?
Hodgkin’s lymphoma (HL), also referred to as Hodgkin’s disease, is an uncommon haematological malignancy arising from mature B cells. It is characterised by the presence of Hodgkin’s cells and Reed-Sternberg cells (to differentiate from Non-Hodgkins Lymphoma- only difference)
Most commonly presents with painless cervical and/or supraclavicular lymphadenopathy in a young adult.
B symptoms (fevers, night sweats, weight loss) occur in approximately 30% of patients; more common in advanced disease.
What does G-CSF do?
Makes stem cells leave the bone marrow so that they can be collected from the blood.
leukaemia vs lymphoma vs myeloma
Leukaemia:
Lymphoma: cancer in lymphocytes (lymph nodes, spleen, thymus, bone marrow)
Myeloma: cancer from plasma cells of bone marrow
what all is needed for erythropoiesis?
EPO (from kidney) [drive]
Genes [recipe]
Iron, B12, folate, minerals [ingredients]
Functioning bone marrow
Anaemia?
not enough blood
what is polycythaemia?
too much blood
what is the normal total body content of iron?
4g
what is the effect of hepcidin?
low iron hormone- reduces the levels of iron in plasma
(hereditary haemochromotosis= less of hepcidin)
-binds ferroprtin and degrades it - reducing iron absorption (enterocyte) and decreasing iron release from the RES.
