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Paeds > Haematology > Flashcards

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
Q

What are the investigations for idiopathic thrombocytopenic purpura

A

Urgent FBC (look at platelet count)

26
Q

What is the management for idiopathic thrombocytopenic purpura

A

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
Q

What are the complications of idiopathic thrombocytopenic purpura

A

Chronic idiopathic thrombocytopenic purpura

Anaemia

Intracranial or subarachnoid haemorrhage

GI bleeding

28
Q

What is sickle cell anaemia

A

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
Q

What are the investigations for sickle cell anaemia

A

At risk mothers offered screening during pregnancy

Newborn heel prick test

30
Q

What is the management for sickle cell anaemia

A

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
Q

What are the complications of sickle cell anaemia

A

Anaemia

Increased risk of infection

Stroke

Avascular necrosis of large joints

Pulmonary hypertension

Priapism

Chronic kidney disease

Sickle cell crisis

Acute chest syndrome

32
Q

What is the management for sickle cell crisis

A

Low threshold for admission

Treat any infection

Keep warm

Keep hydrated

Simple analgesia

Penile aspiration for priapism

33
Q

What are the types of sickle cell crisis

A

Vaso-occlusive

Splenic sequestration

Aplastic

Acute chest syndrome

34
Q

What is thalassaemia

A

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
Q

How might a patient with thalassaemia present

A

Fatigue

Pallor

Jaundice

Gallstones

Splenomegaly

Poor growth and development

Pronounced forehead and cheekbones

36
Q

What are the investigations for thalassaemia

A

FBC

Haemoglobin electrophoresis

DNA testing

37
Q

What is iron overload

A

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
Q

What is the management for thalassaemia

A

Monitor FBC

Monitor for complications

Blood transfusions

Splenectomy

Iron chelation

Bone marrow transplant

39
Q

What is hereditary spherocytosis

A

Sphere-shaped red blood cells

Fragile, easily destroyed (when passing through spleen)

Autosomal dominant

40
Q

How might a patient with hereditary spherocytosis present

A

Jaundice

Anaemia

Gallstones

Splenomegaly

Episodes of haemolytic crisis

Aplastic crisis

41
Q

What are the investigations for hereditary spherocytosis

A

Blood film (spherocytes)

FBC (high reticulocytes)

42
Q

What is the management for hereditary spherocytosis

A

Folate supplements

Splenectomy

Blood transfusions

43
Q

What is G6PD deficiency

A

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
Q

How might a patient with G6PD deficiency present

A

Neonatal jaundice

Anaemia

Intermittent jaundice

Gallstones

Splenomegaly

45
Q

What are the investigations for G6PD deficiency

A

Blood film (heinz bodies)

G6PD enzyme assay

46
Q

What is the management for G6PD deficiency

A

Avoid triggers

Paeds (17 decks)

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