6.1 - Haematology In Systemic Disease Flashcards

1
Q

How can reduced erythropoiesis occur and by what Mechanism?

A

Lack of response to the haemostasis loop e.g in chronic kidney disease the kidney stops making EPO

1) empty bone marrow unable to respond to stimulus from EPO e.g after chemotherapy or toxic insult such as parvovirus infection or in aplastic anaemia
2) marrow infiltrated by cancer cells or fibrous tissue (myelofibrosis) = normal haemopoietic cells are reduced.

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2
Q

How can you get dyserythropoesis?

A

Dyserythropoesis = lack of RBC

1) iron disregulation: iron not released for use in bone marrow
2) marrow shows lack of response to EPO
3) reduced lifespan of RBC

All as a result of inflammatory cytokines

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3
Q

What diseases can you get dyserythropoesis with?

A
  • Renal disease

- Inflammatory conditions e.g rheumatoid arthritis, SLE, inflammatory bowel disease, chronic infections, etc.

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4
Q

What is a functional iron deficiency?

A

Sufficient iron in body but not available to developing erythroid cells

  • macrophages eat old senescent RBC and recycle iron
  • recycled iron is the main source of iron for new RBC
  • small amount of iron absorbed from the gut
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5
Q

What does hepcidin do?

A

A negative regulator of iron absorption

Decreases ferroportin, a protein involved in moving iron out of cells
So
- prevents iron absorption from gut
- prevents iron release from macrophages

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6
Q

What is hepcidin regulated by?

A

HFE
Transferrin receptor
Inflammatory cytokines

NB: its made in the liver

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7
Q

What would you look for to detect anaemia of chronic renal failure?

A
  • normocytic monochromic or microcytic anaemia
  • Normal/high ferritin
  • normal/high reticulocyte haemoglobin content
  • CRP often elevated
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8
Q

What are the consequences of anaemia of chronic renal failure?

A

Reduced EPO due to damaged kidneys
Raised cytokines
Reduced lifespan of RBC
Reduced clearance of hepcidin = increased hepcidin production due to inflammatory cytokines.

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9
Q

What’s the treatment for anaemia of chronic disease/inflammation?

A

Treat underlying condition
If associated renal failure, recombinant human EPO
Ensure b12, iron and folate stores and adequate
Transfuse if all else fails and patient is symptomatic

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10
Q

What is the management of anaemia of chronic renal failure?

A

iron given as IV as absorption is impaired

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11
Q

What is the treatment of rheumatoid arthritis?

A

Analgesis, often NSAIDs
Corticosteroids
Chemotherapy e.g methotrexate
Biological agents

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12
Q

Why would blood be hypochromic in rheumatoid arthritis?

A

Low

  • ACD
  • blood loss e.g due to NSAIDs/corticosteroids
  • haematinic
  • immune
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13
Q

Why would a blood film for someone with RA show neutrophils?

A

High

  • associated inflammatory infection
  • drug reactions
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14
Q

What’s feltys syndrome?

A

Rheumatoid arthritis + splenomegaly + neutropenia

Neutropenia is secondary to splenomegaly, peripheral destruction of neutrophils and failure of bone marrow to produce neutrophils

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15
Q

What does portal hypertension cause and What does this lead to?

A

Oesophageal and gastric varices, but mainly causes splenomegaly
This leads to
- splenic sequestration of cells
- overactive removal of cells

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16
Q

What are the haematological features of liver disease?

A
  • blood loss: response to anaemia may be impaired due development of iron deficiency
  • deficiencies of coagulation factors
  • thrombocytopenia
  • defective platelet function
  • alcohol excess = malurtrition secondary (folate) and directly toxic to bone marrow
  • hepatitis can cause bone marrow failure
  • immune mediated anaemia
17
Q

What is zieves syndrome?

A

Acute haemolytic anaemia

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

18
Q

Why do you get thrombocytopenia in liver disease?

A

Impaired production as thrombopoietin is made in the liver
Splenic pooling
Increased destruction

Functional problems =platelets don’t work properly

19
Q

Why would you get a low platelet and neutrophil count in liver disease?

A
  • impaired production
  • splenic pooling
  • increased destruction
20
Q

Low RBC count in liver disease as…

A
  • impaired production
  • splenic pooling
  • increased destruction
21
Q

How does neutrophil count change with sepsis?

A

Starts off high then the more severe it gets the lower it is

22
Q

What haematological changes occour with infection?

A
  • Chronic infection can cause anaemia of chronic disease
  • infection with malaria causes haemolysis
  • bacteria infection = neutrophilia
  • sepsis = neutropenia
  • parasite infection = eosinophilia
  • viral infection = lymphocytosis
  • infections = thrombocytosis
  • severe infection = thrombocytopenia
23
Q

What’s disseminated intravascular coagulation?

A
  • pathological activation of coagulation
  • numerous microthrombi are formed in the circulation
  • this leads to consumption of clotting factors and platelets and a haemolytic anaemia
  • risk of bleeding and thrombosis
  • tests show low fibrinogen and raised D dimers (fibrin degradation products)
24
Q

What causes a low neutrophil and platelet count in cancer?

A

Chemotherapy
Sepsis
Bone marrow infiltration

25
Q

What causes a low and high red cell count in cancer?

A

Low

  • bleeding
  • iron deficiency
  • chemotherapy

High
- EPO secreting tumour

26
Q

What causes high neutrophils and high platelets in cancer?

A

Neutrophils

  • infection
  • inflammation

Platelets

  • infection
  • inflammation
  • bleeding
  • iron deficiency
27
Q

What’s a leucoerythroblastic film?

A

Granulocyte precursors and nucleated RBC on blood film

28
Q

Why would you get a leucoerythroblastic film?

A
  • sepsis/shock
  • bone marrow infiltration by carcinoma or haematological malignancy
  • severe megaloblastic anaemia
  • primary myelofibrosis
  • AML/MDS
  • storage disease