11. Haematology In Systemic Disease Flashcards
What are changes to the blood in systemic disease often caused by?
Underlying physiological or external cause - e.g. over expression of cytokines
Complications of the disease - e.g. ulcerative colitis
Treatment adverse effects - e.g. methotrexate
In anaemia of chronic disease what are the 3 main contributors caused by inflammatory cytokines?
Iron dysregulation: available iron not released for use in bone marrow
Marrow shows lack of response to erythropoietin
Reduced lifespan of red cells
What is anaemia of chronic disease?
Functional iron deficiency
Sufficient iron in the body but not available to the developing erythroid cells
What is hepcidin regulated by?
HFE
Transferring receptor
Inflammatory cytokines
What is the role of hepcidin and how does it work?
Prevents iron absorption from gut and prevents iron release form macrophages
Works by degrading ferroportin the protein involved in moving iron out of cells
Describe anaemia of chronic kidney disease
Reduced erythropoietin production due to damage to kidneys
Underlying cause often associated with raised cytokines (makes more hepcidin)
Reduced clearance of hepcidin
Reduced lifespan of RBC as direct effect of uraemia
What negative effect can dialysis have?
Damage to red blood cells
What can uraemia cause?
Reduced lifespan of RBC
Inhibits megakaryocytes leading to low platelet counts
What are the treatments for anaemia of chronic disease?
If associated with renal failure, recombinant human erythropoietin
Ensure vit B12 folate and iron stores are adequate
Transfuse red cells, only if all else fails and patient is symptomatic
What are the possible haematological abnormalities in kidney disease related to red cells?
Anaemia - CKD, blood loss, dietary causes
Secondary polycythaemia - renal transplant, renal tumour, polycystic kidneys
What are the possible haematological abnormalities in kidney disease relating to neutrophils?
Neutropenia - immunosuppression (post renal transplant), autoimmune kidney disease
Neutrophilia - inflammation, infection, drugs (steroids)
What are the possible haematological abnormalities in kidney disease relating to platelets?
Thrombocytopenia - uraemia, many drugs, haemolytic uraemia syndrome
High - inflammation, bleeding, iron deficiency
What is rheumatoid arthritis treated with?
Pain relief - often NSAIDs
Disease modifying agents - corticosteroids, chemotherapy, biological agents
How is the haematology affected in rheumatoid arthritis?
High platelets and neutrophils when disease is active (CRP also up)
Low platelets and neutrophils may occur due to treatment, autoimmune reactions or to hypersplenism
Felty’s syndrome
What is Felty’s syndrome?
Rheumatoid arthritis, splenomegaly and neutropenia
Neutropenia thought to be due to splenomegaly contributing to peripheral destruction of eutrophic and failure of bone marrow to produce neutrophils as insensitivity of myeloid cells to GCSF
What can chronic liver disease cause?
Portal hypertension which causes splenomegaly which leads to splenic sequestration of cells and overactive removal of cells
Low blood counts
What are the haematological features of liver disease?
Portal hypertension leads to oesophageal and gastric varies
Blood loss contributed to by deficiencies of coagulation factors, endothelial dysfunction, thrombocytopenia, defective platelet function
Why does thrombocytopenia occur n patients with liver disease?
Impaired production as thrombopoietin is made in the liver
Splenic pooling
Increased destruction
Platelets made often have reduced function which contributes to bleeding
What cells are often seen in liver disease?
Target cells, due to increased cholesterol:phospholipid ratio, membrane not as tight
What is the effect of alcohol excess?
Directly toxic to bone marrow cells - can contribute to pancytopenia
Secondary malnutrition common (esp. folic acid deficiency) - leading to megaloblastic anaemia
What is the effect of viral hepatitis?
Bone marrow failure can develop after an episode of hepatitis
What is he effect of autoimmune liver disease?
Immune mediated anaemia
Thrombocytopenia
Neutropenia
What post operative reactive changes can cause problem associated with red blood cells?
Anaemia - blood loss pre-op (trauma), blood loss during op
Temporary relative polycythaemia - dehydration (rare)
What post operative reactive changes can occur relating to neutrophils?
Neutropenia - sever sepsis
Neutrophilia - post-op reactive, infection, severe bleeding
What post operative reactive changes can occur relating to platelets?
Thromocytopenia - drugs, sepsis, DIC
Thrombocytosis - post-op reactive, infection, bleeding
How can haematological changes with infection affect red blood cells?
Chronic infection can cause anaemia of chronic disease
Infection with malaria can cause haemolytic anaemia
How can haematological changes with infection affect white blood cells?
Bacterial infection often associated with neutrophilia
Severe bacterial infection/sepsis can cause neutropenia
Parasitic infections associated with eosinophilia
Viral infections can cause lymphocytes is and neutropenia
How can haematological changes with infection affect platelets?
Infection can cause reactive thrombocytosis
Severe infection can cause thrombocytopenia
Thrombocytopenia may be associated with DIC in severe sepsis
What is disseminated intravascular coagulation (DIC)?
Pathological activation of coagulation
Numerous microthrombi are formed in circulation
Consumption of clotting factors and platelets and a consequent microangiopathic haemolytic anaemia
Long clotting times, low fibrinogen, raised D-dimers or fibrin degradation
Risk of bleeding and thrombosis
What does a blood film show of DIC?
Fragmented RBC
Low platelets
Immature RBC
What are the haematological changes in cancer relating to red blood cells?
Anaemia - bleeding, iron deficiency, ACD, treatments (chemotherapy)
Polycythaemia - EPO producing tumours
What are the haematological changes in cancer relating to neutrophils?
Neutropenia - chemotherapy, marrow infiltrated by cancer cells
Neutrophilia - inflammation, infection
What are the haematological changes in cancer relating to platelets?
Thrombocytopenia - chemotherapy, sepsis, DIC, marrow infiltrated
Thrombocytosis - inflammation, infection, bleeding, iron deficiency
What is a leucoerythroblastic film?
Granulocyte precursors and nucleated RBC seen on blood film
Spilling out form marrow into blood when marrow is under stress
When is a leucoerythroblastic film seen?
Sepsis/shock One marrow infiltration by carcinoma or haematological malignancy Severe megaloblastic anaemia Primary myelofibrosis (tear drop RBCs) Leukaemia Storage disorders
