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Haematology RBC Disorders Flashcards

1. Illustrate the haematopoietic system 2. Knowledge of red cell disorders 3. Categorise anaemia 4. Knowledge of signs and symptoms of RBC disorders 5. Interpret haematological data for RBC disorders 6. Knowledge of indices used to analyse RBC disorders 7. Elucidate dental aspects of common RBC disorders

1
Q

What is a haematopioetic stem cell

A

Cell that differentiates and matures to form different blood cell lines

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2
Q

What is erythropoiesis

A

Production of RBCs in bone marrow

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3
Q

What can reticulocyte count be used for

A

As an estimation of erythropoiesis rate

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4
Q

What are the requirements of erythropoiesis

A
  1. Healthy bone marrow to produce RBCs
  2. Healthy kidneys to produce erythropoietin
  3. Healthy liver for iron and B12 storage
  4. Healthy small intestine for vitamin absorption
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5
Q

What are the different Hb types in adults

A
  • HbA; 2 alpha and 2 beta chains
  • HbA2; 2 alpha 2 delta chains
  • HbF; 2 alpha and 2 gamma chains
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6
Q

What increases erythropoiesis

A

It is controlled by negative feedback so EPO production increases when;

  • altitude increases
  • hypoxia
  • exercise
  • cardiac/pulmonary disease
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7
Q

How long do RBCs circulate in the blood

A

120 days before kupffer cells (macrophages) engulf them

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8
Q

What is anaemia

A

When the Hb count is below the normal for a given age, gender and ethnicity

There are fewer RBCs which makes Hb low and so the blood carries less oxygen

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9
Q

Outline the four aetiological factors for anaemia

A
  1. Deficiency states; iron, folic acid, B12, coeliac/chrons (malabsorption)
  2. Bone marrow aplasia
  3. Increased destruction (haemolytic anaemia)
    - sickle cell = inherited
    - thalassemia = inherited
    - G6P dehydrogenase deficiency = inherited
    - hereditary spherocytosis = inherited
  4. Miscellaneous; chronic diseases, acute blood loss, real failure, malignancy thus bone marrow infiltration
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10
Q

What is polycythaemia

A

The opposite of anaemia; RBC overproduction

This causes increased viscosity and blood pressure and so reduces the blood flow rate - this decreases oxygen delivery = Hyper-viscosity syndrome which increases thrombosis risk

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11
Q

Outline the aetiological factors for polycythaemia

A

Primary = cancer of erythropoietic cell line

Secondary = dehydration, emphysema, altitude

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12
Q

What does polycythaemia lead to

A
  • embolism
  • heart failure
  • stroke
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13
Q

What are the morphological classifications of anaemia

A
  1. Microcytic
  2. Normocytic
  3. Macrocytic
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14
Q

What are the Hb concentration (colour) classifications for anaemia

A
  1. Hypochromic
  2. Normochromic
  3. Hyperchromic
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15
Q

What are the aetiological classifications for anaemia

A
  1. Impaired erythropoiesis
  2. RBC depletion/haemolysis
  3. Loss of RBCs due to chronic/acute bleeding
  4. Impaired RBC distribution causing hypersplenism
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16
Q

What are the causes of iron deficiency anaemia

A

This is the most common, and typically in women of childbearing age

  • acute/chronic blood loss
  • menorrhagia, GIT bleeding
  • aspirin and NSAIDS
  • vegans
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17
Q

Describe the clinical presentations of iron deficiency anaemia

A

Early stage = symptomless
Late = tiredness, dyspnoea (laboured breath)
- cold intolerance, tingling and numbness
- koilonychia (nail deformity)
- Patterson-Brown-Kelly syndrome making it hard to swallow

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18
Q

What is the relevance of iron deficiency anaemia for dentistry (SPACAA)

A
  1. Sore tongue
  2. Atrophic glossitis (dorsally); there are no filiform/fungiform papillae
  3. Angular stomatitis; maceration of skin by angle of mouth
  4. Candidiasis; creamy white lesions
  5. Aphthous ulcerations (round)
  6. Pallor of oral mucosa
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19
Q

What are the causes of B12 deficiency anaemia

A

Common in 55-60 year olds

  • pernicious anaemia = antibody to parietal cells (gastric)
  • partial gastrectomy = low levels of IF
  • crohn’s/coeliac disease = B12 malabsorption
  • nitrous oxide abuse
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20
Q

Describe the clinical presentations of B12 deficiency anaemia (same for folate deficiency)

A

Burning of the tongue

Patients are psychosomatic and can have neurological symptoms (pins + needles at extremities and visual disturbances)

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21
Q

What tests are used to diagnose B12 deficiency anaemia

A
  1. Schillings test; IM B12 given and urinary excretion is tested (no longer used)
  2. Serum B12 test
  3. Intrinsic factor antibodies = highly specific so there is rarely false positives
  4. Gastric anti-parietal cell antibody = low specificity
22
Q

How is B12 deficiency anaemia treated

A

Monthly IM B12 injections

23
Q

What is the relevance of B12 deficiency anaemia in dentistry (TUNAB)

A

Presents depapillated and beefy red tongue

Angular chelitis (inflamed corners of mouth)

Circumoral and peripheral tingling numbness

Recurrent oral ulcers

Neurological symptoms

LA is safe and conscious sedation can be given if Hb is slightly low

24
Q

What is folate needed for

A

DNA/RNA synthesis; the body cannot store folate

25
What does folate deficiency cause during pregnancy
Cleft palate in the child
26
What are the causes of folate deficiency
Poor intake, chronic alcoholism, malabsorption and it can be drug induced (phenytoin, cytotoxic drugs, HIV/AIDS drugs)
27
What treatments are given for folate deficiency anaemia
Daily oral intake of folic acid tablets
28
What causes haemolytic anaemia
Abnormalities in... 1. Hb formation - sickle cell disease where 2 abnormal HbS copies inherited (most common presentation) - thalassemia; no alpha/beta globulin chain 2. Erythrocyte structure (presenting as sickle cell anaemia) - spherocytosis = broken down - elliptocytosis = oval shaped - G6P dehydrogenase deficiency causes premature RBC metabolism - RBC damage by malaria and drugs
29
What causes sickle cell anaemia
Abnormal HbS + HbC which is common in African + Afro-caribbeans This reduces oxygen tension causing sickling; this leads to haemolysis and so haemolytic anaemia
30
SS
Sickle cell anaemia
31
AS
Sickle cell trait = carrier
32
SC
Sickle Hb disease (milder SS)
33
Describe the clinical presentation of sickle cell anaemia
1. Obstruction of small capillaries so restricted BF to organs causing ischaemia, necrosis and damage 2. Painful crises due to infracts of bone, CNS, spleen + lungs as small vessels are occluded 3. Haematological crisis (aplastic); paravirus - cessation of erythropoiesis causes severe anaemia 4. Chronic anaemia (reticulocytosis where the body compensated and overproduces stale immature RBC reticulocytes) 5. Chronic hyperbilirubinaemia; due to heam breakdown giving bilirubin which circulates in the body causing jaundice and gall stones 6. Increased susceptibility to infections; meningitis and pneumonia = treated with penicillin 7. Sequestration syndrome; chest and septicaemia (spleen)
34
What is the relevance of sickle cell anaemia in dentistry
Orofacial manifestations present: - painful infarcts in jaw/osteomyelitis - hypercementosis - XS overjet/overbite due to enlarged haematopoietic maxilla - hypo mineralised teeth Patients with SS should always be investigated before GA and given early infection management 1. Prevent infections, hypoxia, acidosis and dehydration and these precipitate crisis 2. Manage infection early
35
Give examples of safe analgesics to use for someone with sickle cell anaemia
- paracetamol | - codeine
36
What is aplastic anaemia
A rare condition where the bone marrow is non-functional; Pancytopenia (leukopenia, thrombocytopenia and anaemia)
37
What causes aplastic anaemia
Pancytopenia; leukopenia, thrombocytopenia + anaemia Ideopathic causes; benzene, irradiation, hepatitis Autoimmune disorders Drugs = NSAIDs, allopurinol, anticonvulsants, cytotoxic drugs
38
Describe the clinical presentation of aplastic anaemia
Anaemia, bleeding, increases susceptibility to infection, purpura is the first manifestation
39
How is aplastic anaemia treated
Similar treatment to that of leukaemia
40
Who is more at risk of thalassaemia and what is this
Asians, meditaranians and middle easterns - this is a disorder in globing chain reduction = autosomal recessive
41
What does G6P dehydrogenase deficiency cause
RBC metabolic disorder causing Hb to denature by haemolysis and this is X-linked recessive
42
What does hereditary spherocytosis cause
Spherical RBCs which undergo haemolysis; this is autosomal dominant
43
What is polycythaemia vera
Where blood viscosity is increased which increases risk of thrombosis
44
What key investigations are taken for patients queried anaemia
1. FBC pf Hb, MCV, MCH, red cell distribution width 2. Blood film which shows RBC shape 3. Sickledex 4. Hb electrophoresis which measures different Hb types 5. Serum B12/folate/iron studies
45
Outline management for RBC disorders
- eliminate underlying disease - replacement therapy; folate, IM B12, ferrous sulphate/ fumerate - blood transfusion - erythropoietin
46
What is erythropoietin
EPO hormone; stimulates bone marrow to release RBCs
47
How is erythropoiesis increased
Reduced O2 is detected in the kidneys (and liver) to release erythropoietin which stimulates red bone marrow to increase the RBC count
48
What are the special investigations and treatments for iron deficiency anaemia
- FBC - Iron studies Oral iron supplements and treatment of the underlying cause
49
List the dental aspects of folate deficiency
1. Soreness of the tongue without depapilation/colour change 2. Atrophic glossitis 3. Angular stomatitis
50
Describe oral manifestations of aplastic anaemia (PUBS)
Similar to leukaemia : - ulcerative lesions of oral mucosa - oral petechiae - spontaneous gingival bleeding - gingival swelling if cyclosporin given

BDS2; CHHD (32 decks)

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