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Yr 2 CHHD > Haematology 1B - RBC disorders > Flashcards

Haematology 1B - RBC disorders Flashcards

1
Q

what do haematopoietic stem cells differentiate into

A

differentiate + mature to form different blood cell lines

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2
Q

what is erythropoiesis

A

process which produces RBCs

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3
Q

how is erythropoiesis controlled

A

negative feedback

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4
Q

what can be the stimulus for starting the negative feedback loop for erythropoiesis

A
  • ↓ O2 (hypoxia)
  • high altitude
  • ↑ exercise
  • lots lung tissue in emphysema
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5
Q

what are 3 reasons why hypoxia can be caused + therefore starts negative feedback loop for erythropoiesis

A

↓ RBC count,
↓ O2 available to blood ,
↑ tissue demands for O2

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6
Q

Describe the negative feedback loop for erythropoiesis

A
  • stimuli causes reduced O2 level in blood
  • kidney (+ liver) release erythropoietin hormone
  • erythropoietin stimulates red bone marrow
  • enhanced erythropoiesis ↑RBC count
  • ↑ O2 carrying ability of blood
  • normal blood O2 levels
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7
Q

how are erythrocytes recycled in the body

A

-RBC death + phagocytosis in spleen, liver or red bone marrow
- RBCs broken down into Globin + Heme group
- globin broken into AA – can be used for protein synthesis
- Heme broken into bilirubin – goes to liver and helps with bile for digestion
- bilirubin excreted in small intestine + part in urine
Heme group broken into ion and converted into ferritin in liver + if needed go to red bone marrow + used for new RBC

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8
Q

what are the 2 categories of red cell disorders

A

anaemia

polycythaemia

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9
Q

what is anaemia + why it happens

A

Hb below the normal bc of

  • ↓ RBC, ↓ Hb level or defective Hb
  • ↓ oxygen carrying capacity of blood
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10
Q

what is polycythaemia + why it happens + what can it lead to

A

over production of RBCs

  • ↑ blood viscosity + BP
  • ↓ flow rate of blood + ↓ O2 delivery
  • lead to embolism, stroke, heart failure
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11
Q

what is primary polycythaemia

A

cancer of erythropoietic cell line in red bone marrow

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12
Q

what is secondary polycythaemia

A

underlying conditions

from dehydration, emphysema, high latitude

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13
Q

what are main causes of anaemia

A
  • ↓ RBCs + Hb production
  • increased destruction of RBC
  • increased destruction of RBCs (haemolytic)
  • miscellaneous
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14
Q

what are the deficiency states that can cause anaemia

A

iron
folic acid
vitamin b12
coeliac/crohn’s disease (malabsorption of above)

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15
Q

what are main causes of ↓ RBCs + Hb production causing anaemia

A
    • deficiency states

- - bone marrow aplasia

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16
Q

what 4 diseases can cause haemolytic anaemia

A

sickle cell anaemia
thalassemia major/minor
glucose-6-phosphate dehydrogenase deficiency
hereditary spherocytosis

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17
Q

what is haemolytic anaemia

A

increased destruction of RBCs

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18
Q

what 4 type of miscellaneous things can cause anaemia

A

anaemia of chronic disease
acute blood loss
chronic renal failure associated anaemia
malignancy related

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19
Q

what are 3 categories anaemia is classified by

A

morphology (MCV) was -micro/macro cytic
Hb conc (colour) (MCH) hypo/hyper chromic
etiology

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20
Q

how do you classify microcytic, hypochromic anaemia

A

↓ MCV (<80) , ↓ MCH

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21
Q

how do you classify normocytic, normochromic anaemia

A

= MCV (80-100) , = MCH

22
Q

how do you classify macrocytic, hyperchromic anaemia

A

↑ MCV, (>100) ↑ MCH

23
Q

what are 4 ways anaemia can be classified by underlying etiology

A
  1. impaired erythropoiesis
  2. RBC depletion/hemolysis
  3. loss of RBCs (acute/chronic blood loss)
  4. impaired RBC distribution
24
Q

what can caused the impaired erythropoiesis when classifying anaemia

A

lack of vit B12 + folic acid
bone marrow diseases
disturbed Fe+ absorption

25
Q

what is iron deficiency anaemia + who is it common in

A

predominant in women of child bearing age
can be bc acute/chronic blood loss
menorrhagia, GI bleeding, vegans, ↑ aspirin intake, NSAIDs

26
Q

what are clinical features seen in those with iron deficiency anaemia

A

early - symptomless
late - tired, tachycardia, palpitations, cold intolerance, tingling + numbness extremities
Koilonychia, Patterson-Brown-Kelly syndrome

27
Q

what are dental aspects associated with iron deficiency anaemia

A
  • sore tongue
  • atrophic glossitis
  • candidiasis
  • angular stomatitis
  • apthous like ulcerations
  • pallor of oral mucosa
28
Q

how would you treat a patient with iron deficiency anaemia

A

oral iron supplements

treat underlying cause

29
Q

what is B12 deficiency + clinical features of it

A 
older women (55-60)
initial complaint - burning of tongue
patients thought to be psychosomatic + neurological symptoms
30
Q

what are 4 causes of B12 deficiency anaemia

A
  • pernicious anaemia (autoantibody to gastric parietal cells)
  • partial gastrectomy (↓ intrinsic factors)
  • crohn’s/coeliac disease (malabsorption of B12)
  • nitrous oxide abuse
31
Q

what are dental aspects associated with B12 deficiency anaemia

A
  • depapillated + beefy red tongue
  • angular chelitis
  • recurrent oral ulcers
  • circumoral + peripheral tingling, numbness
  • LA is safe
  • conscious sedation can be given if Hb slightly low
32
Q

what special investigations can be done if you suspect they have B12 deficiency

A
  • Serum Vit B12 levels

- IF ABs/ have high specificity (less likely to give you false positive)

33
Q

how would you treat someone with B12 deficiency

A

monthly IM injection of Vit B12

34
Q

what are main causes of folate deficiency

A

poor food intake - poverty, diet, chronic alcoholism
malabsorption
drug induced - phenytoxin, cytoxic drugs, HIV/AIDS

35
Q

what are clinical features of folate deficiency

A

-Initial complaint: ~burning of the tongue with no obvious abnormality on examination

36
Q

what are dental aspects associated with folate deficiency anaemia

A

soreness of tongue - without depapillation/colour change
atrophic glossitis
angular stomatitis

37
Q

how would you treat someone with folate deficien

A

daily oral intake of folic acid tablets

38
Q

what is sickle cell anaemia + how is it caused

A

autosomal recessive inheritance
- Abnormal Hb: HbS and HbC

-Different states:
• SS – sickle cell anaemia
• AS – sickle cell trait (carrier)
• SC (milder form of SS) – sickle-Hb disease
-
39
Q

what other anaemia can sickle cell disease cause + how

A

↓O2 tension causes sickling –> sig RBC breakdown (haemolysis ) –> haemolytic anaemia

40
Q

what are the 7 clinical features of sickle cell anaemia

A
  • small capillaries obstructed, restricted blood flow to organ
    o Ischaemia, necrosis, organ damage
  • Painful crises (due to infarcts of bone, CNS, spleen, lungs)
  • Haematological crises (aplastic); parvovirus

-Chronic anaemia (reticulocytosis, Hb levels as low as 5-9g/dl
o Body tries to compensate -> ↑ rbcs produced + stale immature reticulocytes

  • Chronic hyperbilirubinaemia; bc breakdown of haem -> bilirubin -> circulate around the body -> jaundice/gall stones

-Susceptible to infections (meningitis , pneumonia )
o Treated with Penicillin V (phenoxymethylpenicillin)

-Sequestration syndrome (lungs – chest syndrome, spleen – septicaemia)

41
Q

what are the 4 orofacial manifestation of sickle cell anaemia

A

o Painful infarcts in jaw or osteomyelitis
o Hypercementosis
o Excessive overjet & overbite (because of enlarged haematopoietic maxilla)
o Hypomineralised teeth

42
Q

what are other dental aspects associated with sickle cell anaemia other than orofacial manifestations

A
  • should Prevent infections, hypoxia, acidosis or dehydration
  • Early management of infection
  • Patient at risk must be investigated prior to GA
  • Safe analgesics to be used e.g. paracetamol and codeine
43
Q

what is aplastic anaemia + what can it cause

A

non-functioning bone marrow

cause pancytopenia, leukopenia, thrombocytopenia, + anaemia

44
Q

what are 3 main causes of aplastic anaemia

A
  1. idiopathic - exposure to benzene, viral infections(hepatitis)
  2. autoimmune disorders
  3. drugs ( NSAIDs, anticonvulsants, cytotoxic drugs, allopurinol)
45
Q

what are clinical features of aplastic anaemia

A
  1. purpura is often 1st manifestation
  2. anaemia features (tired, pale)
  3. Susceptibility to infection
  4. bleeding
46
Q

what treatment modifications should be considered when treating patient with aplastic anaemia

A

conscious sedation + GA avoided
haemorrhagic tendencies
Susceptibility to infections

47
Q

what oral manifestations can appear due to aplastic anaemia

A

ulcerative lesions of oral mucosa , spontaneous gingival bleeding
gingival swelling if cicloporin used

48
Q

what are 4 other red cell related conditions

A
  • Thalassaemia
  • G6P dehydrogenase deficiency: RBC metabolic disorder -> Hb denature -> haemolysis
    o X-linked recessive
  • Hereditary spherocytosis: Spherical RBCs -> haemolysis
    o Autosomal Dominant
  • Polycythaemia vera: ↑viscosity of blood ∴ ↑ risk of thrombosis
49
Q

what histories should be taken with anaemic patients

A
  • History of presenting complaint: severity of signs and symptoms of anaemia
  • Past medical history: any existing medical problems/prev surgery, could be part of the bowel that could affect absorption
  • Dietary history: are they vegans? Nutritional deficiency, iron deficiency anaemia
  • Family history: hereditary pattern? Type of anaemia present in family?
  • Social history: chronic alcoholics, folate deficiency, pregnancy, puberty
50
Q

what are key investigations that can be done if anaemic

A
  • FBC of Hb, MCV, MCH, red cell distribution width
  • Blood film (would give you an idea of what shape the rbc is)
  • Sickledex (solution turns cloudy if given)
  • Hb electrophoresis; measures different types of Hb
  • Serum B12 - Serum folate
  • Serum iron studies: iron, ferritin and TIBC
51
Q

how to manage anaemia

A

• Eliminate underlying disease

• Replacement therapy
o Iron; ferrous sulphate, ferrous fumerate
o Folate
o IM B12 monthly!

• Blood transfusion (acute haemolysis/blood loss)

• Erythropoietin (chronic renal failure & anaemia of chronic disease)
o Epo is a HORMONE that stimulates bone marrow to release blood cells

Yr 2 CHHD (4 decks)

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