Session 6 -Haematology in systemic disease

Question 1

what is the physiological life cycle of blood cells

Answer 1

from the bone marrow to peripheral blood cells to the reticulo-endothelial system

Question 2

what things contribute to the changes to blood in systemic disease

Answer 2

IL-6 - underlying physiological or external cause

complications of disease

treatmemt- adverse effects

Question 3

what contributes to anaemia of chronic disease

Answer 3

iron dysregulation- iron isnt released for use in bone marrow
lack of response to erythropoeietin
reduced life span of red cells

Question 4

what dieases can lead to anaemia of chronic disease

Answer 4

rheumatoid diseases
inflammatory bowel disease
chronic infections- TB

Question 5

why is anemia of chronic disease a functional iron deficiency

Answer 5

sufficient iron in the body but not available to the developing erythroid cells

Question 6

what is the main exporter of iron out of a macrophage

Answer 6

ferroportin

Question 7

what regulates hepcidin

Answer 7

HFE
Transferrin receptor
inflammatory cytokines

Question 8

what does Hepcidin do

Answer 8

prevents iron release from macrophages

by degrading ferroportin the protein involved in moving iron out of cells

prevents iron absorption from the gut

Question 9

how does an inflammatory condition cause anaemia

Answer 9

cytokines- IL-6- are released by immune cells

increased production of Hepcidin by liver

inhibition of ferroportin

decreased iron release and absorption

plasma iron reduced

inhibition of erythropoiesis in bone marrow

Question 10

what factors lead to anaemia of chronic kidney disease

Answer 10

raised cytokines

reduced clearance of hepcidin and increased hepcidin production due to inflammatory cytokines

dialysis- damage to RBC

reduced lifespan of RBC as a direct effect of uraemia

reduced erythropoietin production due to damage to kidneys

Question 11

what effects can uraemia have

Answer 11

inhibits megakaryocytes leading to low platelet counts

reduced lifespan of RBC

Question 12

what is needed for EPO therapy to work

Answer 12

iron vit B12 and folate

Question 13

how can you treat anaemia of chronic disease

Answer 13

treat the underlying condition
-recombinant human erythropoietin

if fails- transfuse red cells

Question 14

How is rheumatoid arthritis usually treated

Answer 14

Pain relief- NSAIDs

Disease modifying agents- DMARDS

• corticosteroids
• chemotherapy
• biological agents

Question 15

What is feltys syndrome

Answer 15

Triad of syndromes

• Rheumatoid arthritis
• splenomegaly
• neutropenia

Question 16

Why is neutropenia a part of feltys syndrome

Answer 16

• due to splenomegaly contributing to peripheral destruction of neutropenia
• failure of bone marrow to produce neutrophils as there is insensitivity of the myeloid cells to the stimulator GCSF

Question 17

In liver disease why can gastric varices develop

Answer 17

Portal hypertension leads to oesophageal and gastric varices because the dilated vessels prone to bleeding due to hig than normal pressure

Question 18

What are clotting factors dependant on

Answer 18

Vitamin K

Question 19

Why is thrombocytopenia seen in liver disease

Answer 19

Impaired production of thrombopoietin- made in liver

Splenic pooling

Increased destruction

Question 20

What type of red blood cells are seen liver disease

Answer 20

Target cells

Question 21

What is disseminated intravascular coagulation

Answer 21

Pathological activation of coagulation

Numerous microthromi are formed in the circulator

Consumption of clotting factors and platelets and a consequent micrangiopathic haemolytic anaemia

Long clotting times

Low fibrinogen

Raised D-Dimers or fibrin degradation products

Risk of bleeding and thrombosis

Question 22

What is leucoerythroblastic film

Answer 22

Spilling out from the marrow into the blood when the marrow is under stress

Granulocyte precursors and uncleared RBCs seen on blood film

Question 23

What can cause leucoerythroblastic film

Answer 23

Sepsis/shock

Bone marrow infiltration by carcinoma or haematological malignancy

Severe megaloblastic anaemia

Primary myeofibrosis (with tear drop RBCs)

Leukaemia

Storage disorders