Red Blood Cell Disorders Flashcards

1
Q

What kind of stem cell differentiates and matures to form different blood cell lines

A

Hematopoietic stem cells

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2
Q

What is erythropoiesis

A

The process that produces RBCs

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3
Q

Describe the structure of Hb

A

4 proteins chains, 2 alpha and 2 beta, each chain has a heme group

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4
Q

What stimuli are there to increase erythropoiesis

A

Low level of O2
High altitude
Increase in exercise
Loss of lung tissue in emphysema

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5
Q

Describe the feedback mechanism of erythropoiesis

A

Negative:

  • reduced O2 levels in blood
  • kidney releases erythropoietin
  • erythropoietin stimulates red bone marrow
  • enhanced erythropoiesis increases RBC count
  • increases O2 carrying ability of blood and then yeh lol
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6
Q

What are the 2 types of red cell disorders

A
  • Anaemia

- Polycythaemia

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7
Q

Describe features anaemic red cell disorders

A
  • Defined as Hb below the normal for the age, gender and ethnic background
  • ↓ red blood cells, ↓ Hb level or defective Hb
  • ↓ oxygen carrying capacity of blood
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8
Q

Describe features polycythaemia red cell disorders

A
  • Over-production of red blood cells
  • ↑ Viscosity & BP, decrease flow rate of blood with ↓ O2 delivery
  • Lead to embolism, stroke or heart failure
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9
Q

How does primary polycythaemia occur

A

Due to cancer of erythropoietic cell line in the red bone marrow

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10
Q

How does secondary polycythaemia occur

A

From dehydration, emphysema, high altitude

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11
Q

What causes reduced erythrocyte and Hb production

A

Deficiency States -

  • Iron deficiency
  • Folic acid deficiency
  • Vitamin B12 deficiency
  • Coeliac/Crohn’s disease (malabsorption or iron folic acid and B12)
  • Bone marrow Aplasia
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12
Q

Name some etiological factors that cause anaemia

A
  • Increased RBC destrcution
  • Anaemia of chronic disease
  • Acute blood loss
  • Chronic renal failure associated anaemia
  • malignancy related, bone marrow infiltration associated anaemia
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13
Q

what can cause increased destruction of RBCs and lead to anaemia

A
  • Sickle cell anaemia
  • Thalassemia major/minor
  • Glucose-6-phosphate dehydrogenase deficiency
  • Hereditary spherocytosis
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14
Q

What does microcytic mean

A

Smaller RBCs

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15
Q

What are hypo chromic RBCs

A

RBCs with less colour under microscope due to less Hb

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16
Q

What does microcytic mean

A

Larger RBCs

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17
Q

What do MCH and MCV mean

A
MCV = measure of average size of your RBCs
MCH = measure of Hb and colour in RBCs
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18
Q

How are MCV and MCH affected with microcytic + hypochromic RBCs

A

MCV and MCH are lowered

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19
Q

How are MCV and MCH affected with macrocytic RBCs

A

MCV and MCH are raised

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20
Q

What is the most common anaemia and which population does it occur most often

A

Iron deficiency anaemia

Predominant in women of child bearing age

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21
Q

What are some of the causes of iron deficiency anaemia

A
Acute or chronic blood loss
Menorrhagia
GI bleeding
Chronic intake of aspirin and NSAIDs
Iron deficiency (vegans)
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22
Q

what arrest clinical features of iron deficiency anaemia

A
Early - none
Late - 
- Tiredness
- Dyspnoea
- Palpitations 
- Tachycardia
- Conjunctiva pallor
- Cold intolerance, tingling and numbness of extremities
- Koilonychia, Patterson-Brown-Kelly syndrome
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23
Q

What are the dental aspects of iron deficiency anaemia

A
  • Sore tongue
  • Atrophic glossitis
  • Cadidiasis
  • Angular stomatitis
  • Apthous-like ulcerations
  • Pallor of oral mucosa
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24
Q

What investigations and treatments are done for iron deficiency anaemia

A
Investigations = FBC and iron studies
Treatment = oral iron supplements, treat underlying cause
25
Q

Who commonly get B12 deficiency and what is the initial complaint they give

A
  • Common in older women

- Burning of the tongue with no obvious abnormality on examination (psychosomatic)

26
Q

What are the causes of B12 deficiency

A
  • Pernicious anaemia (autoantibody to gastric parietal cells)
  • Partial gastrectomy (low levels of intrinsic factors)
  • Crohn’s/Coeliac disease (malabsorption of B12)
  • Nitrous oxide abuse
27
Q

What are the dental aspects of B12 deficiency

A
  • Depapillated and beefy red tongue
  • Angular chelitis
  • Recurrent oral ulcers
  • Circumoral and peripheral tingling, numbness
  • LA is safe
  • Conscious sedation can be given if Hb slightly low
28
Q

What investigations and treatment are used for B12 deficiency

A
  • Serum Vitamin B12 levels
  • Intrinsic factor antibodies/ Gastric anti-parietal cell antibodies

Treatment - monthly IM injection of Vitamin B12

29
Q

List the aetiology of folate deficiency

A
  • Poor intake, poverty dietary ignorance, chronic alcoholism
  • Malabsorption
  • Drug induced
30
Q

What drugs can induce folate deficiency

A

Phenytoin, cytotoxic drugs, HIV/AIDs drugs

31
Q

Describe the clinical features of folate deficiency

A

Same as B12 deficiency without neurological symptoms

32
Q

What are the dental aspects of folate deficiency

A
  • Soreness of the tongue without depapillation/colour change
  • Atrophic glossitis
  • Angular stomatitis
33
Q

What investigations and treatment are used for folate deficiency

A

Investigations - Serum folate level

Treatment - daily oral intake of folic acid tablets

34
Q

What are the abnormalities found in haemolytic anaemia

A
  • Haemoglobin formation
  • Erythrocyte structure/function
  • RBC damage by external function (malaria, drugs)
35
Q

What abnormalities of haemoglobin formation can cause haemolytic anaemia

A

Sickle cell disease

Thalassaemia’s

36
Q

What abnormalities of erythrocyte structure/function can cause haemolytic anaemia

A
  • Spherocytosis

- Glucose-6-phosphate dehydrogenase deficiency

37
Q

What forms of Hb are present in people with SCA

A

HbS and HbC

38
Q

What populations have this most commonly

A

African and afro-carribeans

39
Q

What kind of inheritance gives SCA

A

Autosomal recessive

40
Q

How does SCA work

A

Reduced oxygen tension causes sickling resulting in significant red blood cells breakdown (haemolysis)

41
Q

What are the clinical features of SCA

A
  • Painful crises due to infarcts of bone, CNS, spleen and lungs
  • Haematological crises (aplastic)
  • Susceptible to infections (meningococci, pneumococci)
  • Chronic anaemia
  • Chronic hyperbilirubinaemia (jaundice, gall stones)
  • Sequestration syndrome (acute chest syndrome)
42
Q

What are some orofacial manifestations of SCA

A
  • Painful infarcts in jaw or osteomyelitis
  • Hypercementosis
  • Excessive overjet and overbite ~ enlarged haemopoietic maxilla
  • Hypomineralised teeth
43
Q

Besides orofacial manifestations what are the dental aspects of SCA

A
  • Prevent infections, hypoxia, acidosis or dehydration ~ precipitate crisis
  • Early management of infection
  • Patient at risk must be investigated prior to GA
  • Paracetamol and codeine ~ safe analgesics
44
Q

What is aplastic anaemia

A
  • Non-functioning bone marrow
  • Rare
  • Pancytopenia ~ leukopenia, thrombocytopenia and anaemia
45
Q

What are the causes aplastic anaemia

A
  • Idiopathic ~ exposure to benzene, irradiation, viral infections i.e hepatitis - Autoimmune disorders
  • Drugs ~ NSAIDs, allopurinol, anticonvulsants, cytotoxic drugs
46
Q

What are the clinical features

A
  • Purpura is often the 1st manifestation
  • Anaemia (normochromic, normocytic or macrocytic; fatigue, shortness of breath, pale skin)
  • Susceptibility to infection
  • Bleeding
47
Q

What treatment modifications need to made in aplastic anaemic patients

A
  • Anaemia (e.g. conscious sedation and GA should be avoided)
  • Haemorrhagic tendencies
  • Susceptibility to infections
48
Q

What are the oral manifestations you can see in patients with aplastic anaemia

A
  • Ulcerative lesions of oral mucosa, oral petechiae, spontaneous gingival bleeding etc.
  • Gingival swelling if ciclosporin is used
49
Q

What is thalassemia

A

Red cell disorder most common in patients of asian, mediterranean and middle east descent

50
Q

What is Glucose-6-phosphate dehydrogenase deficiency

A

RBC metabolic disorder - Hb denaturation, haemolysis

51
Q

What is hereditary spherocytosis

A

RBC disorder that causes spherical red blood cells - haemolysis

52
Q

What is polycythaemia vera

A

Over production of RBCs, thrombosis is a complication

53
Q

What are some of the key investigations for SCA

A
  • FBC (Hb, MCV, MCH and RBC distribution width
  • Blood film
  • Sickledex
  • Haemoglobin electrophoresis
  • Serum B12
  • Serum folate
  • Serum iron studies (iron, ferritin, total iron biding capacity)
54
Q

What are the symptoms of Microcytic anaemia + MCV<80fL

A
  • Iron deficiency
  • Thalassemia*
  • Sideroblastic anaemia*
55
Q

What are the symptoms of macrocytic anaemia + MCV>100fL

A
  • Vitamin B12 deficiency
  • Folate deficiency
  • Alcoholism*
  • reticulocytosis*
  • hypothyroidism*
  • Multiple myeloma*
  • Myelodysplasia*
  • Aplastic anaemia*
56
Q

What are the symptoms of normocytic anaemia + MCV 80-100 fL

A
  • chronic disease
  • haemolytic anaemia
  • acute blood loss*
    marrow infiltration*
57
Q

What can be done to manage anaemias

A
  • Eliminate the underlying disease
  • Replacement therapy
  • Blood transfusion (acute haemolysis/blood loss)
  • Erythropoietin (chronic renal failure and anaemia of chronic disease)
58
Q

What replacement therapies can be used for anaemia

A
  • Iron-ferrous sulphate, ferrous fumerate
  • Folate
  • IM B12