Haematology Flashcards
What are the causes of iron deficiency anaemia?
Dietary insufficiency, prolonged milk consumption
Iron loss - meckels diverticulum
Inadequate absorption - chrons
Where is iron absorbed?
Duodenum and jejunum
What type of anaemia is iron deficiency?
Microcytic
What is the presentation of iron deficiency anaemia?
Fatigue, lethargy, pallor, poor feeding, anorexia
What eating disorder can iron deficiency anaemia present with?
PICA
What is the management of iron deficiency anaemia?
Ferrous sulphate
Increase dietary iron - green veg, cereals, meat, egg yolk, increase vitamin C also
What is sickle cell disease?
AR condition resulting in abnormal beta chains causing sickle shaped RBC that have shorter life spans and get trapped in the microcirculation
What is the diagnosis of sickle cell anaemia?
Newborn blood spot test
Blood film
Hb electrophoresis
What is a vaso-occlusive crisis?
Sickled RBC clog capillaries causing distal ischaemia in hands and feet often triggered by cold weather, dehydration, infection
What is a splenic sequestration crisis?
RBC block blood flow in the spleen causing an acutely enlarged and painful spleen and susceptibility to infection from encapsulated bacteria
What does a splenectomy require?
Life-long penicillamine prophylaxis
What is an aplastic crisis?
Temporary absence of creation of new RBC triggered by parvovirus B19
What is acute chest syndrome?
Vessels supplying lungs become clogged with RBC causing chest pain, SOB, fever, cough
What can stimulate HBF?
Hydroxycarbamide
Name some long-term complications with sickle cell
Osteomyelitis from salmonella
Cardiomegaly and arrhythmias
Ischaemic colitis
Liver dysfunction trapping sickle cells
Pigment gallstones
Occlusive crisis
What is fanconi anaemia?
Rare genetic DNA repair disorder that is autosomal recessive and affects all 3 haemopoietic cell precursors
What is the presentation of fanconi anaemia?
Bruising, purpura, short stature, cafe au lait spots, absent thumb, horseshoe kidney, microcephaly, cryptochidism, deafness, low set ears
What is an FBC finding of fanconi anaemia?
Pancytopenia
What is raised in fanconi anaemia?
Alpha fetoprotein
What is VWD?
Defect in quantity or quality of VWF which links platelets to exposed underlying endothelium and binds and stabilises coagulation factor 8
What is the presentation of VWD?
easy bruising, excessive bleeding, large bruises
What is the diagnosis of VWD?
APTT raised
PT normal
Platelet count normal
VWF low
Factor 8 activity low
What is the management of VWD?
Desmopressin - increases factor 8 activity by releasing them from storage in weibel palade bodies in the endothelium
What is ITP?
Spontaneous low platelet count causing a purpuric rash caused by a type II hypersensitivity reaction 24-48 hours after viral illness (strep)
What is haemophilia?
Bleeding disorder that is X-linked recessive with A being more common and a deficiency of factor 8 and B of factor 9
what is the presentation of haemophilia?
Easy bleeding and bruising, haemorthioses, epistaxis
What is the diagnosis of haemophilia?
APTT raised, factor 8 low, PT and VWF normal
What is thalassaemia?
Genetic defect in haemoglobin protein chains - both types are autosomal recessive
What is the presentation of thalassemia?
Microcytic anaemia, fatigue, pallor, jaundice, splenomegaly, gallstones, frontal bossing, poor growth
Describe what you’d see on a slide if a child had iron deficiency anaemia.
Microcytic
Hypochromic
Low/ normal reticulocytes
What would you see on the blood results of a child with iron deficiency anaemia?
Low ferritin
Low serum iron
Increased TIBC
What are the side effects of ferrous sulphate?
Constipation and black stools
What are the Hb, Reticulocyte, WBC values in a person with Sickle Cell Disease?
Low Hb
Increased reticulocyte count
Elevated WBC
Describe the symptoms & blood film of beta-thalassaemia minor
Asymptomatic
Mild anaemia
Low MCV
Raised Hb
What signs + symptoms would be displayed by someone with beta-thalassaemia major?
Splenomegaly
Failure to thrive
Delayed puberty
Jaundice
Low MCV
Progressive severe anaemia
What is the most common form of immunologic thrombocytopenia?
ITP
What is meant by a ‘coagulopathy’?
Errors in the clotting cascade
What is haemolytic disease of the newborn?
Incompatibility between the rhesus antigens on the surface of RBCs of the mother and foetus = rhesus D antigen
What is the presentation of haemolytic disease of the newborn?
Jaundice, oedema, anaemia, splenomegaly
What is the treatment for haemolytic disease of the newborn?
Exchange transfusion, IV IG, Phototherapy and anti-D IG for mother
What is iron overload?
Result of the faulty creation of RBC, recurrent transfusions and increased absorption of iron in the gut in response to anaemia
