Haematology Flashcards

1
Q

What is fetal haemoglobin

A

2 alpha, 2 gamma subunits

Greater affinity for oxygen (bind more easily, harder to release)

Fetal haemoglobin production starts to decrease at 32-36 weeks (replaced by adult haemoglobin, all adult by 6 months)

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2
Q

What are the causes of anaemia in infants

A

Physiological anaemia of infancy (normal dip in Hb around 6-9 weeks)

Anaemia of prematurity (less time to get iron from mother, inadequate RBC production, reduced EPO levels, blood tests)

Blood loss

Haemolysis (haemolytic disease of the newborn, hereditary spherocytosis)

Twin-to-twin transfusion

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3
Q

What are the causes of anaemia in older children

A

Iron deficiency anaemia (dietary insufficiency)

Blood loss

Sickle cell anaemia

Thalassaemia

Leukaemia

Hereditary spherocytosis

Hereditary elliptocytosis

Sideroblastic anaemia

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4
Q

What are the causes of microcytic anaemia

A

TAILS

Thalassaemia

Anaemia of chronic disease

Iron deficiency anaemia

Lead poisoning

Sideroblastic anaemia

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5
Q

What are the causes of normocytic anaemia

A

3 As and 2 Hs

Acute blood loss

Anaemia of chronic disease

Aplastic anaemia

Haemolytic anaemia

Hypothyroidism

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6
Q

What are the causes of macrocytic anaemia

A

Impaired DNA synthesis

B12 deficiency

Folate deficiency

Alcohol

Reticulocytosis

Hypothyroidism

Liver disease

Drugs (azathioprine)

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7
Q

What are the symptoms of anaemia

A

Tiredness

Shortness of breath

Headaches

Dizziness

Palpitations

Worsening of other conditions

Pica and hair loss (with iron deficiency anaemia)

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8
Q

What are the generic signs of anaemia

A

Pale skin

Conjunctival pallor

Tachycardia

Raised respiratory rate

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9
Q

What are the signs of anaemia that are specific to iron deficiency

A

Koilonychia

Angular cheilitis

Atrophic glottis

Brittle hair and nails

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10
Q

What are the investigations for anaemia

A

Bloods (FBC, blood film, reticulocyte count, ferritin, B12, folate, bilirubin)

Direct Coombs test

Haemoglobin electrophoresis

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11
Q

What is the management for anaemia

A

Treat underlying cause

Consider blood transfusion

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12
Q

What are the causes of iron deficiency anaemia

A

Dietary insufficiency

Loss (heavy menstruation)

Inadequate absorption

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13
Q

Where is iron absorbed

A

Duodenum and jejunum

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14
Q

What medication interferes with iron absorption

A

PPIs

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15
Q

What is the management for iron deficiency anaemia

A

Treat underlying cause

Dietician input

Oral supplements

Consider blood transfusion

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16
Q

What is leukaemia

A

Cancer of particular line of stem cells in bone marrow

Unregulated production of blood cells (over production of one type, other types underproduced)

Get pancytopenia (low RBCs, low WBCs, low platelets)

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17
Q

What are the types of leukaemia that affect children

A

Acute lymphoblastic leukaemia (age 2-3)

Acute myeloid leukaemia (under 2s)

Chronic myeloid leukaemia

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18
Q

What are the risk factors for leukaemia

A

Radiation exposure (abdominal X-ray during pregnancy)

Conditions (Down’s, Klinefelter, Noonan)

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19
Q

How might a patient with leukaemia present

A

Persistent fatigue

Unexplained fever

Failure to thrive

Weight loss

Night sweats

Pallor

Petechiae and abnormal bruising

Unexplained bleeding

Abdominal pain

Generalised lymphadenopathy

Unexplained bone or joint pain

Hepatosplenomegaly

20
Q

What are the investigations for leukaemia

A

Refer immediately if have: unexplained petechiae, hepatomegaly

Very urgent FBC (48 hours)

Blood film (shows blast cells)

Bone marrow biopsy

Lymph node biopsy

Staging (chest X-ray, CT, lumbar puncture, genetic analysis)

21
Q

What is the management for leukaemia

A

Chemotherapy

Radiotherapy

Bone marrow transplant

Surgery

22
Q

What are the complications of chemotherapy

A

Failure to treat

Stunted growth and development

Immunodeficiency and infection

Neurotoxicity

Infertility

Secondary malignancy

Cardiotoxicity

23
Q

What is idiopathic thrombocytopenic purpura

A

Spontaneous low platelet count causing a non-blanching rash

A type II hypersensitivity reaction

Can be triggered by viral infections

Usually in under 10s

24
Q

How might a patient with idiopathic thrombocytopenic purpura present

A

Rash appears over 24-48 hours

Bleeding (gums, epistaxis, menorrhagia)

Bruising

25
What are the investigations for idiopathic thrombocytopenic purpura
Urgent FBC (look at platelet count)
26
What is the management for idiopathic thrombocytopenic purpura
Usually no treatment needed Monitor until platelets return to normal If active bleeding or severe: prednisolone, IV immunoglobulins, blood transfusion, platelet transfusion Education: avoid contact sports, avoid IM injections and lumbar punctures, avoid NSAIDs/aspirin/anticoagulants
27
What are the complications of idiopathic thrombocytopenic purpura
Chronic idiopathic thrombocytopenic purpura Anaemia Intracranial or subarachnoid haemorrhage GI bleeding
28
What is sickle cell anaemia
Crescent shaped red blood cells (more fragile, easily destroyed) A form of haemolytic anaemia Prone to sickle cell crisis Abnormal variant HbS Autosomal recessive Protective against malaria
29
What are the investigations for sickle cell anaemia
At risk mothers offered screening during pregnancy Newborn heel prick test
30
What is the management for sickle cell anaemia
Avoid dehydration Avoid triggers for crisis Ensure vaccines up to date Antibiotic prophylaxis (penicillin V) Hydroxycarbamide (protects against crisis, stimulates fetal Hb production) Bone marrow transplant Blood transfusion
31
What are the complications of sickle cell anaemia
Anaemia Increased risk of infection Stroke Avascular necrosis of large joints Pulmonary hypertension Priapism Chronic kidney disease Sickle cell crisis Acute chest syndrome
32
What is the management for sickle cell crisis
Low threshold for admission Treat any infection Keep warm Keep hydrated Simple analgesia Penile aspiration for priapism
33
What are the types of sickle cell crisis
Vaso-occlusive Splenic sequestration Aplastic Acute chest syndrome
34
What is thalassaemia
Genetic defect in the protein chains that make up haemoglobin Autosomal recessive Red blood cells more fragile Get splenomegaly Bone marrow expands (susceptibility to fractures, pronounced forehead, pronounced cheekbones)
35
How might a patient with thalassaemia present
Fatigue Pallor Jaundice Gallstones Splenomegaly Poor growth and development Pronounced forehead and cheekbones
36
What are the investigations for thalassaemia
FBC Haemoglobin electrophoresis DNA testing
37
What is iron overload
Due to: faulty creation of RBCs, recurrent transfusions, increased iron absorption Presentation: fatigue, cirrhosis, infertility, impotence, heart failure, osteoporosis, joint pain Management: limit transfusions, iron chelation
38
What is the management for thalassaemia
Monitor FBC Monitor for complications Blood transfusions Splenectomy Iron chelation Bone marrow transplant
39
What is hereditary spherocytosis
Sphere-shaped red blood cells Fragile, easily destroyed (when passing through spleen) Autosomal dominant
40
How might a patient with hereditary spherocytosis present
Jaundice Anaemia Gallstones Splenomegaly Episodes of haemolytic crisis Aplastic crisis
41
What are the investigations for hereditary spherocytosis
Blood film (spherocytes) FBC (high reticulocytes)
42
What is the management for hereditary spherocytosis
Folate supplements Splenectomy Blood transfusions
43
What is G6PD deficiency
X-linked recessive Crisis triggered by: infection, medications (primaquine, nitrofurantoin, trimethoprim) G6PD enzyme protects cells against reactive oxygen species damage Get haemolysis of red blood cells
44
How might a patient with G6PD deficiency present
Neonatal jaundice Anaemia Intermittent jaundice Gallstones Splenomegaly
45
What are the investigations for G6PD deficiency
Blood film (heinz bodies) G6PD enzyme assay
46
What is the management for G6PD deficiency
Avoid triggers