Haematology Flashcards
Define anaemia
A low level of haemoglobin in the blood.
Examples of microcytic anaemia
TAILS
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
Examples of normocytic anaemia
3 As and 2 Hs
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
Examples of macrocytic anaemia
Megaloblastic - B12, folate deficiency
Normoblastic - alcohol, reticulocytosis, hypothyroidism, liver disease, azathioprine.
History of anaemia
Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
Worsening of other conditions
Iron deficient
Pica - crave abnormal things like dirt
Hair loss
Clinical signs of anaemia
Pallor
Tachycardia
Raised respiratory rate
Koilonychia - iron
Angular cheilitis - iron
Atrophic glossitis - iron
Brittle hair - iron
Jaundice - haemolytic
Bone deformities - thalassaemia
Oedema HTN and excoriations - CKD
Investigations in anaemai
Bloods - MCV, B12, folate, ferritin, film
OGD + colonoscopy - cancer?
Bone marrow biopsy if cause unclear
Causes of iron deficiency anaemia
Insufficient dietary iron
Increased requirements - pregnancy
Iron being lost - slow bleed, colon cancer
Inadequate iron absorption
Most common cause - menorrhagia
Management of iron deficiency anaemia
Investigate cause - ?cancer
Blood transfusion
Iron infusion
Oral iron supplement
Define pernicious anaemia
An autoimmune condition attacking the parietal cells or intrinsic factor. This prevents the absorption of vitamin B12 and they become deficient.
History of B12 deficiency
Peripheral neuropathy
Loss of vibration sense or proprioception
Visual changes
Mood or cognitive change
Management of pernicious anaemia
If dietary deficient - cyanocobalamin
IM hydroxocobalamin - B12 replacement
Treat B12 before folate -spinal cord degeneration
Define haemolytic anaemia
Anaemia caused by the destruction of red blood cells. Caused often by inherited condition .
Inherited haemolytic anaemias
Hereditary spherocytosis
Hereditary elliptocytosis
Thalassaemia
Sickle cell anaemia
G6PD dificiencty
Acquired haemolytic anaemia
Auto immune
Alloimmune - transfusion reactions, haemolytic disease of newborn
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related
Clinical signs of haemolytic anaemia
Splenomegaly
Jaundice
Normocytic anaemia
Investigations in haemolytic anaemia
FBC - normocytic anaemia
Film - schistocytes
Direct coombs test - positive in autoimmune haemolytic anaemia
Define hereditary spherocytosis
Most common inherited haemolytic anaemia - causing sphere shaped red blood cells that are fragile and easily broken down when passing through the spleen
History of hereditary spherocytosis
Jaundice
Gall stones
Splenomegaly
Aplastic crisis - parvovirus
Management of hereditary spherocytosis
Folate supplementation
Splenectomy
Cholecystectomy - stones
Define G6PD deficiency
Defect in the red blood cell enzyme G6PD
X linked recessive condition causing crises that are triggered by infection medications or fava beans.
Classic presentation of G6PD deficieny
Mediterranean or African patient turns jaundiced and anaemic after eating broad beans, an infection or treatment with antimalarials.
What is seen on blood fil in G6PD deficiency
Heinz bodies - individual blobs seen inside rbcs - denatured globin.
Define target cells
Central pigmented area surrounded by a pale arrea, surrounded by a ring of thicker cytoplasm on the outside - bull’ eye
What conditions would you see target cells in
Iron deficiency anaemia
Post splenectomy
Define Howell-Jolly bodies
Individual blobs of DNA material seen inside RBCs,
What conditions would you see Howell-Jolly bodies in
Post splenectomy
Severe anaemia
Define reticulocytes
Immature RBCs - still have RNA in, appears in a mesh like (reticular) pattern
What conditions would you see reticulocytes in
Normal to have 1% or RBCs as reticulocytes
Goes up if increased turnover
Haemolytic anaemia
Define schistocytes
Fragments of red blood cells - indicate physical damage by trauma
What conditions would you see schistocytes in
Networks of small clots
Haemolytic uremic syndromes
DIC
TTP
Metallic heart valves
Haemolytic anaemia
Define sideroblasts
Immature RBCs that contain blobs of iron
What conditions would you see sideroblasts in
Myelodysplasic syndrome
Define smudge cells
Ruptured white blood cells occur during the process of preparing the blood film due to aged or fragile cells
What conditions would you see smudge cells in
Chronic lymphotic leukaemia
Define spherocytes
Spherical red blood cells
What conditions would you see spherocytes in
Autoimmune haemolytic anaemia
Hereditary spherocytosis
Define thalassaemia
A autosomal recessive genetic defect that changes the protein chains that make up haemoglobin.
Define thalassaemia
A autosomal recessive genetic defect that changes the protein chains that make up haemoglobin.
Define thalassaemia
A autosomal recessive genetic defect that changes the protein chains that make up haemoglobin.
Clinical signs of thalasaemia
Microcytic anaemia
Fatigue
Pallor
Jaundice
Gallstones
Splenomegaly
Poor growth and development
Pronounced forehead and malar eminences
Investigations in thalassaemia
FBC
Haemoglobin electrophoresis
DNA testing
Screening in pregnancy
Features of iron overload
Fatigue
Liver cirrhosis
Infertility
Heart failure
Arthritis
Diabetes
Osteoporosis and joint pain
Define alpha-thalassaemia
Thalassaemia caused by defects in the alpha globin chain - issues with chromosome 16
Management of alpha thalassaemia
Monitoring FBC
Monitoring complication
Blood transfusions
Splenectomy
Bone marrow transplant - can be currative
Define beta thalassaemia
Defects in the beta globin chains - defect on chromosome 11
Define beta thalassaemia minor
Carriers of abnormal gene - one abnormal and one normal.
Causes mild microcytic anaemia - often no active treatment
Define beta thalassaemia intermedia
Two abnormal copies of the beta globin gene - either two defective or one defective and one deletion
Define beta thalassaemia major
Patients who are homozygous for the deletion genes - no functioning beta-globin gene
Severe microcytic anaemia, splenomegaly, bone deformities
Define sickle Cell anaemia
A autosomal recessive condition causing production of sickle shaped RBCs. Defect on chromosome 11 on the beta globin gene. Makes RBCs more fragile leading to haemolytic anaemia.
What affect does sickle cell disease have on malaria
Having one copy of the sickle-cell trait reduces the severity of malaria
Investigations in sickle cell anaemia
Newborn screening heel prick
Film
Complications of sickle cell anaemia
Anaemia
Increased risk of infection
Stroke
Avascular necrosis
Pulmonary hypertension
Priapism
CKD
Sickle cell crises
Acute chest syndrome
Management of sickle cell anaemia
Avoid dehydration
Ensure vaccines up to date
Antibiotic prophylaxis
Hydroxycarbamide - stimulate HbF production
Blood transfusion - in severe anaemia
Bone marrow transplant - can be curative
Define sickle cell crises
Ubrella term for a spectrum of acute crises related to the condition
Range from mild to life threatening, can be spontaneous or triggered by infection, dehydration, cold or significant events