MEH haemotology

Question 1

cause of pernicious anaemia?

Answer 1

Auto-antibodies interfering with the production or function of intrinsic factor
> essential for the absorption of vitamin B12

“Pernicious” means “deadly”

Question 2

role of ferroportin

Answer 2

ferroportin transports it out of the erythrocytes across the basolateral membrane of the enterocytes and into the bloodstream.

Question 3

role of hepcidin

Answer 3

inhibits iron transport by binding to ferroportin

> prevents iron from being exported into the bloodstream thereby reducing dietary iron absorption.

Question 4

what facilitates uptake of ferrous iron (Fe2+) from the intestinal lumen into enterocytes?

Answer 4

Divalent metal transporter 1 (DMT1)

Question 5

intracellular protein-iron complexes that are used to store iron?

Answer 5

Ferritin and haemosiderin

Question 6

treatment option for a patient with severe hereditary haemochromatosis?

Answer 6

Therapeutic phlebotomy to remove excess iron

Question 7

what is haemoglobin H disease?

Answer 7

type of alpha- thalassemia

reduced formation of alpha globin chains.
imbalance of global chains needed to form haemoglobin

• see skeletal abnormalities as RBCs are created in unusual places e.g. bone cortex and impair growth

Question 8

effect of chronic haemolysis?

Answer 8

XS bilirubin >gall stone formation

Question 9

Microangiopathic haemolytic anaemia?

Answer 9

type of anaemia that can result from red blood cells becoming damaged by the shear stress produced by a defective heart valve

Question 10

role of janus kinase 2 receptor?

Answer 10

stimulate signalling pathways leading to erythrocyte production in response to the hormone erythropoietin.

Question 11

megakaryocytes?

Answer 11

the cells that platelets (thrombocytes) ‘bud off’ from

Question 12

what is primary myelofibrosis?

Answer 12

myeloproliferative neoplasm- proliferation of mutated hematopoietic stem cells results in reactive bone marrow fibrosis eventually leading to the replacement of marrow with scar tissue

> extra medullary haemopoesis occurs (haemopoesis outside of bone marrow e.g liver > enlarged liver

Question 13

what is the name given to small red spots under the skin from broken capillaries?

Answer 13

Petechiae.
- seen in thrombocytopenia

(less than 3mm)- purpra over 3mm

Question 14

what would cause an inc in hepcidin hormone?

Answer 14

cytokines- ACD/ inflammation

Question 15

cause or normocytic anaemia?

Answer 15

chronic kidney disease

Question 16

effect of urea on erythropoiesis?

Answer 16

increase in urea concentration in blood:
inhibit erythropoiesis
reduces the lifespan of existing red blood cells
inhibits platelet function
> can cause chronic bleeding from GI

Question 17

what is Felty syndrome?

Answer 17

triad of symptoms

• Rheumatoid arthritis
• splenomegaly
• neutropenia (low)

Question 18

what is a Leucoerythroblastic film

Answer 18

Blood film where Granulocyte precursors and nucleated RBCs are present

Question 19

effects of XS iron?

Answer 19

cirrhosis, 
cardiomyopathy, 
diabetes mellitus, 
skin pigmentation, 
polyarthropathy
hypogonadotrophic hypogonadism.

Question 20

Which vitamin is involved in the synthesis of clotting factors?

Answer 20

Vit K

Question 21

which condition show a red blood cell mean cell volume (MCV) lower than the normal range?

Answer 21

Iron deficiency anaemia

Question 22

which conditions show a red blood cell mean cell volume (MCV) greater than the normal range?

Answer 22

Alcoholism
Liver disease
Haemolytic anaemia
Megaloblastic anaemia

Question 23

term meaning increased variation in the size of red blood cells?

Answer 23

Anisocytosis

Question 24

Define :
Poikilocytosis
Eliptocytosis
Spherocytosis
Anisocytosis
Hypochromic

Answer 24

Poikilocytosis-abnormally shaped RBCs

Eliptocytosis- elliptical RBCs

Spherocytosis-spherical RBCs

Anisocytosis-increased variation in the size of RBCs

Hypochromic- RBCs that are paler than normal (due to less haemoglobin)

Question 25

what is haematocrit?

Answer 25

volume percentage of red blood cells in blood

divide this (haematocrit) by the concentration of RBCs to get the mean corpuscular volume

Question 26

which thalassemia is a cause of severe anaemia?

Answer 26

‘β-thalassaemia intermedia’