Haematology In Systemic Disease Flashcards

1
Q

What is anaemia of chronic disease/inflamamtion

A

Dyserythropoiesis
1. Iron dysregulation: iron not released for use in bone marrow
2. the marrow shows a lack of response to erythropoietin
3. reduced lifespan of red cells
Renal disease, inflammatory conditions such as Rheumatoid arthritis, SLE, Inflammatory bowel disease (Ulcerative Colitis or Crohn’s), chronic infections etc.

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2
Q

What is functional iron deficiency

A

Sufficient iron in the body but not available to the
developing erythroid cells
Plenty of iron in the macrophages but they cant give it to the red cells that need it

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3
Q

What is the main source of iron for new rbcs?

A

 Macrophages ‘eat’ old senescent RBCs and recycle iron
 Iron recycling is the main source of iron for new rbc
 Small amount of iron absorbed from gut

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4
Q

What is hepcidin regulated by?

A

Regulated by:
HFE
Transferrin receptor
Inflammatory cytokines

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5
Q

What is the role of hepcidin

A

Degrades ferroportin a protein involved in moving iron out of cells - prevents iron release from macrophages and prevents iron absorption from gut

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6
Q

What do inflammatory cytokines do in anaemia of chronic disease ?

A

 Iron dysregulation: iron not released for use in bone marrow
Due to inflammatory cytokines
 the marrow shows a lack of response to erythropoietin
Due to inflammatory cytokines
 reduced lifespan of red cells
Due to inflammatory cytokines

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7
Q

What is anaemia of chronic renal failure?

A

Reduced erythropoietin production due to damage to kidneys
Often associated with raised cytokines
Reduced clearance of hepcidin
Increased hepcidin production due to inflammatory cytokines
Dialysis-damage to rbc and loss due to bleeding
Reduced lifespan of RBC as a direct effect of uraemia - Also inhibits megakaryocytes leading to low platelet counts

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8
Q

What are investigations into acd?

A

 Often normocytic normochromic or microcytic anaemia
 Normal or high ferritin
 Normal or high Reticulocyte Haemoglobin content (CHr)
 CRP often elevated
In most ppl, ferritin is best test of iron stores - ferritin produced by liver -
in some patients ferritin levels high (liver disease) - liver pushing it out - in these patients it is not. A good measure -
chr low is iron deficient . Chr will be normal/high if enough /a lot of iron stores -
You may read about: Low serum iron and normal/high % transferrrin saturation Not very reliable

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9
Q

What is the treatment for ACD?

A

Treat the underlying condition
If associated renal failure
Recombinant human Erythropoietin
Must have all building blocks e.g Iron and Vit B12/folate
Ensure Vit B12 folate and iron stores are adequate
Transfuse, if all else fails and patient is symptomatic - transfuse sparingly and only i patient is clinically symptomatic

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10
Q

What is the management of anaemia of chronic renal failure

A

Use Reticulocyte Haemoglobin Content (CHr) (or % hypochromic cells) to assess for functional iron deficiency - Iron given intravenously for patients with anaemia of renal failure
Give iron if ferritin <200 mg/L (normal range 15-400mg/L ) or CHr low then give iron
Iron given as IV as absorption is impaired (….Hepcidin)

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11
Q

What are abnormalities in RBCs in renal disease?

A

Low • ARF/ACD • Blood loss • Haematinic causes

High • Post renal transplant • Renal tumour

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12
Q

What are abnormalities in WBCs in renal disease?

A

Low: • immunosuppression
post renal transplant
drugs; • marrow infiltration eg in
myeloma

High • inflammation • connective tissue
disease • Infection • drugs: steroids - Steroids to damp down immune cause of renal failure

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13
Q

What are abnormalities in platelets in renal disease?

A

Low • direct effect of uraemia on platelet production • drugs • Haemolytic uraemic syndrome

High • reactive • bleeding • iron deficiency

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14
Q

How is rheumatoid arthritis treated

A
Chronic immune mediated inflammatory condition
 Treated with
◦ analgesis often NSAIDs
◦ Corticosteroids
◦ chemotherapy eg methotrexate
◦ biological agents
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15
Q

What are abnormalities in RBCs in rheumatoid arthritis?

A

Low • ACD • blood loss eg

due to NSAIDs/corticost eroids • Haematinic • Immune

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16
Q

What are abnormalities in WBCs in rheumatoid arthritis?

A

High •Associated inflammation •infection •drug reactions…..

Low •drugs eg methotrexate •immune

17
Q

What are abnormalities with platelets in rheumatoid arthritis?

A

High •reactive •bleeding •iron deficiency

Low •drugs autoimmune splenomegaly (Felty’s)……

18
Q

What is feltys syndrome

A

Triad of RA, splenomegaly, neutropenia
Neutropenia
◦ secondary to splenomegaly, peripheral destruction of neutrophils, and failure of bone marrow to produce neutrophils
◦ high level of G-CSF, insensitivity of myeloid cells to cytokines ◦

19
Q

What does portal hypertension cause?

A

Portal Hypertension causesvsplenomegaly which leads to:

• Splenic sequestration of cells • Overactive removal of cells

20
Q

What are haematological features of liver disease?

A

 Blood loss
Response to anaemia may be impaired due to the development of iron deficiency
 deficiencies of coagulation factors
 endothelial dysfunction
 thrombocytopenia
 defective platelet function
Portal hypertension Increased back up of blood - veins swell
clotting factors made in the liver
thrombocytopenia maybe due to splaenomaegly
defective platelet function - other chemical changes
prone to bleeding
chronic blood loss - may become iron deficien
FACT: Many clotting factors are made by the liver
Response to anaemia may be impaired due to the development of iron deficiency

21
Q

What is zieves syndrome?

A

Zieve’s syndrome- acute haemolytic anaemia

Lipid abnormalities affect RBC membrane leading to macrocytosis, target cells and can lead to haemolysis

22
Q

Describr thrombocytopenia in liver disease

A

Thrombocytopenia in 75% patients with liver disease •Impaired production as thrombopoietin is made in the liver •Splenic pooling •Increased destruction
Functional problems-platelets don’t work properly
Liver disease bc of immune causes eg primary biliary cirrhosis

23
Q

What are some haematological features of liver disease?

A

 Alcohol excess
◦ Directly toxic to bone marrow cells
◦ Secondary malnutrition (folic acid deficiency)
 Hepatitis
◦ Bone marrow failure can develop after an episode of hepatitis
 Immune
◦ Immune mediated anaemia, thrombocytopenia or neutropenia

24
Q

Describe th eRBC count in liver disease

A

Low • Impaired production (iron or folate deficiency, alcohol toxicity) • Splenic pooling • Increased destruction (bleeding/haemolysis etc)

High • alcohol

25
Q

Describe the wbc count in liver disease

A

Low • Impaired production • Splenic pooling • Increased destruction
High • Steroids

26
Q

Describe the platelet count in liver disease

A

Low • Impaired production • Splenic pooling • Increased destruction
High • Bleeding

27
Q

What are the post operative reactive changes to rbcs?

A

Low • Bleeding

High • Dehydration

28
Q

What are the post operative reactive changes to wbc count

A

Low • Severe sepsis

High • Infection/Sepsi s

29
Q

What are the post operative reactive changes to platelet count

A

Low •Medication (heparin/antibiotics) •Spesis +/- DIC

High •Bleeding •Infection •‘reactive’

30
Q

What are the changes to rbcs with infection

A

Chronic infection can cause anaemia of chronic disease/inflammation Infection with malaria causes haemolysis

31
Q

What are the changes to WBCs with infection

A

Bacterial infection is often associated with a neutrophilia
Severe infection/sepsis can cause a neutropenia
Parasitic infections are associated with an eosinophilia
Viral infections can cause a lymphocytosis

32
Q

What are the changes too platelets with infection

A

Infection can cause a reactive thrombocytosis

Severe infection can cause thrombocytopenia Thrombocytopenia may be associates with DIC in severe sepsis

33
Q

How can sepsis lead to clotting abnormalities

A

Disseminated intravascular coagulation
 Pathological activation of coagulation
 Numerous microthrombi are formed in the circulation
 This leads to consumption of clotting factors and
platelets, and a haemolytic anaemia
 Clotting tests are affected - usually raised PT/INR,
raised APTT, low fibrinogen and raised D
dimers/fibrin degradation products
 Risk of bleeding and thrombosis

34
Q

What are changes to rbcs with cancer?

A

Low
• Bleeding eg Ca bowel
• Iron deficiency • ACD
• Chemotherapy

High
• EPO secreting
tumours

35
Q

What are Changes to WBCs with cancer

A

Low • Chemotherapy • Sepsis • Bone marrow
infiltration

High • Infection • Inflammation

36
Q

What are changes to platelets with cancer

A

Low • Chemotherapy • Sepsis • Bone marrow infiltration • DIC

High • Infection • Inflammation • Bleeding • Iron deficiency

37
Q

What is a leucoerythroblastic film

A
Granulocyte precursors and nucleated RBC on blood film
 Sepsis/shock
 Bone marrow infiltration by carcinoma or haematological
malignancy
 Severe megaloblastic anaemia
 Primary Myelofibrosis
 AML/MDS
 Storage diseases