Blood Disorders

Anaemia

Symptoms
o Shortness of breath on exertion
o Palpitations
o Weakness and lethargy
o Headaches
o Hyperdynamic Circulation Tachycardia, increased circulatory volume, vasodilation
o Visual Disturbances
Signs
o Pallor of mucus membranes
o Congestive cardiac failure
o Retinal Haemorrhages
o Koilonychia

Microcytic Low MCV

Iron Deficiency
o Chronic blood loss
Gastrointestinal
Uterine
o Pregnancy, or increased growth
o Coeliac Disease Malabsorption
o Poor diet
o Results
Low Haem
Low RBC count
Low haematocrit
o Diagnosis
Check ferratin levels
Check tTGA (Tissue Transglutaminase Antibody) for coeliac
Faecal Calprotectin
Endoscopy
o Treatment
Oral iron Ferrous Sulphate 200mg, 3 times a day for 3 months
Parenteral iron anaphylaxis risk
Thalassaemia
Anaemia of Chronic Disease
o Chronic inflammation leads to increased IL-6 which increases Hepcidin levels
o Hepcidin inhibits ferroportin, reducing iron absorption and release from
macrophages
o Rheumatoid Arthritis
o NSAIDS
Macrocytic

Vitamin B12 Deficiency

o Pernicious Anaemia
Autoantibodies to intrinsic factor which is responsible for B12 uptake
Neuropathy, subacute degeneration of spinal chord
o Crohns Disease
o Intramuscular vitamin B12 1mg every 3 months forever
Folate Deficiency
o Coeliac Disease
o Pregnancy
o Low serum folate, low red cell folate
o No neuropathy
o Folic acid 5mg per day for 3 months
Alcohol Abuse
Macrocytosis in liver disease

Haemolytic Anaemia

Thalassaemia
o Reduced synthesis of globin chains
Hereditary Spherocytosis
o Sphere shaped red blood cells
o Symptoms
RUQ pain, gallstones, splenomegaly, jaundice, young
o Results
Low Hb, Low RBC count, Low Haematocrit
High reticulocyte count
o Diagnosis
Mothers history
Peripheral blood smear
o Treatment
Splenectomy
Remove gall bladder
Give folic acid

Sickle Cell Anaemia

o GAG substituted into GTG in beta globin chain
GD6P Deficiency
Autoimmune Haemolytic Anaemia
Shit Transplant
Malaria

Hypoxic Anaemia

Secondary polycytoxaemia
 o High Hb. Normal everything else
o Headaches
o COPD
More Hb made to bind more O2
Causes HBP and risk of stroke and stasis of blood flow
Treat COPD, quit smoking

White Cell Disorders

Neutrophil Leukocytosis

Neutrophilia Abnormally high neutrophils in blood

Caused by bacterial infection
Leads to inflammation and tissue necrosis
Acute haemorrhage can occur
Treated with steroids (immunosuppressant)
Can lead to myeloproliferative disease (excess cells produced in bone marrow)

Neutropenia

Lack of neutrophils
Usually acquired not congenital, because of drugs, immune disorder or infection
Pancytopenia (bone marrow failure) can cause this

Lymphocytosis

Increased lymphocytes in blood

Caused by infections, leukaemia and lymphomas

Infectious Mononucleosis

Glandular Fever Caused by Epson-Barr Virus

Symptoms Splenomegaly, Sore throat, cervical lymphadenopathy
Amoxicillin will cause a rash if given
Risk of Hep B, may increase risk of Hodgkins lymphoma, splenic rupture and haemolytic
anaemia
Monospot test to diagnose

Lymphopenia

Low lymphocyte levels

Congenital immune deficiencies
Acquired immune deficiency syndrome
Bone marrow failure
Steroids and immunosuppression
Hodgkins disease
Leukaemia

Clonal malignant disorder derived from a single cell

Causes an accumulation of abnormal cells in the bone marrow
These replace normal haematopoietic cells causing bone marrow failure
Causing a reduction in normal WBC, RBC and platelets produced
Acute Myeloid Leukaemia (AML)
o Blasts cannot differentiate, but still divide
o High myeloblasts and lymphoblasts in bone marrow
o Its a killer Im afraid
o Features
Anaemia Pallor, lethargy
Neutropenia mouth/throat infections
Skin and gut flora infections
No pus, no consolidation in lungs
Death within hours
Thrombocytopenia Spontaneous bruising, menorrhagia, bleeding gums,
nose bleeds
Bone pain
Lymphadenopathy
Hepatosplenomegaly
o Investigations
FBC, coagulation screen, Chest X-Ray
Bone marrow to confirm diagnosis and type
Morphology
Immunophenotyping
Chromosome analysis (Mandatory for diagnosis)
B or T cell derived check
o Treatment
Broad spectrum antibiotics for neutropenia
Platelet transfusion
Blood transfusion for anaemia
Chemotherapy
Bone marrow transplant
o Risk of HSV, CMV, VZV, Candida, Aspergillus, PCP
o Prophylaxis
Hand washing, isolation, diet restriction, antifungals and antivirals,
antibiotics
Chronic Myeloid Leukaemia (CML)
o Clonal malignant myeloproliferative disorder
o Leukaemia cells differentiate to mature cells
o 8% of leukamias
o Features
Sweating, fatigue, weight loss
Anaemia
Splenomegaly
Gout
Raised WBC count
 Cells present at all stages of development high blasts
High platelet count
Philadelphia Chromosome - Translocation of 9 and 22
o Treatment
Imantinib, nilotinib, dasatinib Tyrosine kinase inhibitors
Chronic Lymphocytic Leukaemia (CLL)
o 37% of leukaemia
o 43% >75 years old
o Lymphocytosis
o >14 year survival, may not need treatment
o Stage A
Hb >100
Platelets >100
<2 lymph node areas
o Stage B
HB > 100
Platelets >100
3-5 Lymph areas
o Stage C
Hb or Platelets <100
2.5yr survival
Acute Lymphocytic Leukaemia (ALL)
o Immature T and B cells

Sepsis Six

Blood Cultures What is the source of the infection?

Fluid for Blood Pressure
Catheterise for Kidney
Oxygen Hb is low
Check Lactate
Antibiotics Normally tazocin

Bleeding Disorders

Von Willebrand Disease (vWD)

o Type 1 Not enough produced (quantitative)
o Type 2 vWF does not work properly (qualitative)
o Type 3 None produced at all
o Symptoms
Mild mucosal bleeding
o Testing
Check VWF levels and activity
Check FIII levels
o Treatment
Tranexamic Acid Anti-fibrinolytic to stop bleeding
 Desmopressin Release VWF and FIII stores
Haemophilia
o X linked deficiency
o Haemophilia A FIII deficient More common (1/5000)
o Haemophilia B FIX deficient (1/30,000)
o Symptoms
Life threatening bleeding problems
Muscle or joint haematomas
Mild haemophilia can be asymptomatic until provoked (surgery)
o Testing
Prolonged ATPP
FIII and FIX assays
o Treatment
Type A treated with desmopressin
FIII and FIX concentrate as prophylaxis and treatment
o Complications
Intracranial haemorrhage
Long term joint damage
Immune Thrombocytopenia (ITP)
o Immune destruction of platelets
o Increased megakaryocytes
o Platelet transfusion doesnt work
o Corticosteroids and splenectomy
Disseminated Intravascular Coagulation (DIC)
o Coagulation constantly activated
o Generalised bleeding and thrombosis from stasis
o Causes
Infections malaria, meningitis
Adenocancers
Trauma
o Testing
Low platelets
Prolonged APTT
Prolonged PT
Low Fibrinogen
High D-Dimers
o Treatment
Treat cause
Treat bleeding with platelet transfusion, FFP and cryoprecipitate