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MED4 ACP > Red cell disease > Flashcards

Red cell disease Flashcards

1
Q

What is the meaning of HCT, MCV, MCH, MCHC, RDW respectively?

A

HCT (haematocrit): proportion of red cells after centrifugation
MCV (mean corpuscular volume)
MCH (Mean corpuscular Hb)
MCHC (Mean corpuscular Hb conc.)
RDW (Red cell distribution width)

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2
Q

RR for MCV

A

80~100 fL

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3
Q

Which disease leads to elevated MCHC?

A

hereditary spherocytosis

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4
Q

DDx for nucleated RBCs

A

BM stress / damage

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5
Q

DDx for pencil cell

A

IDA

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6
Q

DDx for target cells (4)

A

(Increase membrane to cell volume ratio)
1. IDA
2. Thalassaemia
3. Sickle cell disease, HbC, HbE
4. Liver disease

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7
Q

DDx for spherocytes (2)

A
  1. Hereditary spherocytosis
  2. Warm AIHA
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8
Q

DDx for elliptocyte

A

Hereditary elliptocytosis

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9
Q

DDx for bite cells

A

G6PD deficiency

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10
Q

DDx for schistocytes

A

MAHA

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11
Q

DDx for golf ball cells

A

HbH disease

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12
Q

DDx for red cell agglutination

A

cold AIHA

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13
Q

DDx for rouleaux formation

A

multiple myeloma

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14
Q

DDx for basophilic stippling (3)

A

(Aggregates of ribosomes or fragments of ribosomal RNA)

  1. Lead poisoning
  2. Megaloblastic anaemia
  3. Thalassaemia
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15
Q

DDx for Howell-Jolly bodies (4)

A
  1. Megaloblastic anaemia
  2. Thalassaemia
  3. Sickle cell disease
  4. Splenectomy / Hyposplenism
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16
Q

Which red cell disease causes koilonychia?

A

IDA

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17
Q

Which red cell disease causes leg ulcers?

A

Sickle cell disease

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18
Q

Microcytic hypochromic anaemia DDx

A
  1. IDA
  2. Anaemia of chronic disease
  3. Sideroblastic anaemia
  4. Thalassaemia
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19
Q

Normochromic normocytic anaemia DDx (6)

A
  • ACD
  • Renal failure
  • Aplastic anaemia
  • Acute blood loss
  • Haemolytic anaemia
  • Mixed deficiencies
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20
Q

Congenital syndrome for pure red cell dysplasia

A

Diamond-blackfan syndrome

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21
Q

Microcytic hypochromic red cells on blood film

A

size < nucleus of lymphocytes
central pallor >1/2 of red cell diameter

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22
Q

Iron profile and ferritin for IDA, ACD, sideroblastic anaemia, and thalassaemia respectively

A

(Serum Fe, TIBC, TSAT, Ferritin)
IDA: ↓ , ↑ , ↓ , ↓
ACD: ↓ , ↓ / N, ↓ , ↑ / N
sideroblastic anaemia: ↑ , N , N / ↑ , N / ↑
thalassaemia: ↑ , N , ↑ , ↑

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23
Q

Effect of hepcidin

A

↓ ferroportin –> ↓ Fe absorption & release

24
Q

DDx of iron deficiency anaemia

A
  • chronic blood loss (GI, uterine)
  • ↑ demands (pregnancy, EPO therapy)
  • malabsorption
  • Plummer-Vinson syndrome
25
Q

DDx and pathogenesis of sideroblastic anaemia

A

Congenital (ALA-S mutation)
–> defective protoporphyrin –> ⨉ haem
Acquired (lead poisoning)
–> inhibit haem & globin synthesis

26
Q

Treatment for sideroblastic anaemia caused by ALA-S mutation

A

pyridoxine

27
Q

Pathogenesis of megaloblastic anaemia

A

defective DNA synthesis –> nuclear-cytoplasmic maturation asynchrony

28
Q

DDx of vitamin B12 deficiency

A

(1) Nutritional: strict vegetarian
(2) Malabsorption
a. Stomach: pernicious anaemia, gastrectomy
b. Pancreas
c. Small intestine: blind loops, Crohn’s disease, ileal resection / bypass
(3) Drugs: N2O anaethesia, metformin, PPI, H2 antagonists

29
Q

Diagnosis of pernicious anaemia

A

anti-IF Ab

30
Q

DDx for folate deficiency

A
  1. Nutritional
  2. Malabsorption: coliac disease
  3. Excess utilisation: pregnancy, haemolytic anaemia, CA
  4. Drugs: valproate, phenytoin, methotrexate
31
Q

Which neurological syndrome is irreversible after B12 deficiency?

A

Subacute combined degeneration of spinal cord

32
Q

Supplement for B12 and folate deficiency (caution)

A

B12: Hydroxocobalamin
(S/E: hypoK ; monitor K after treatment)
Folate: Folic acid
(not given alone unless r/o B12 deficiency)

33
Q

Pathogenesis of hereditary spherocytosis

A

defected protein –> loss of vertical interaction between cytoskeleton and lipid bilayer –> loss of biconcave shape ==> spherocytes –> ↑ fragility –> ↓ lifespan

34
Q

Diagnosis of hereditary spherocytes (2)

A
  1. Flow cytometry for EMA binding to RBC
  2. direct Coombs’ test
35
Q

Pathogenesis of G6PD deficiency

A

↓ reduction of NADP into NADPH –> ↓ GSH –> ↑ RBC susceptibility against oxidative stress

36
Q

Diagnosis of G6PD deficiency

A

G6PD assay

37
Q

Aetiology (4) and pathogenesis of warm AIHA

A

Aetiology:
- Idiopathic
- Autoimmune disease
- Lymphoproliferative diseases
- Drugs: methyldopa

[37 °C] IgG coats RBC –> recognised by macrophages –> RBC becomes spherical –> extravascular haemolysis

38
Q

Diagnosis and treatment (6) of warm AIHA

A

Dx: DCT anti-IgG +ve
Tx:
- treat underlying cause
- prednisolone
- rituximab
- splenectomy
- folate
- blood transfusion

39
Q

Aetiology (4) and pathogenesis of cold AIHA

A

Aetiology:
- idiopathic
- infection (Mycoplasma pneumoniae)
- lymphoproliferative diseases
- Paroxysmal cold haemoglobinuria

Pathogenesis: [4°C] IgM binds on I antigen on RBC –> fix complement quickly –> intravascular haemolysis

40
Q

Diagnosis (2) and treatment (4) of cold AIHA

A

Dx:
- red cell agglutination in cold
- DCT: anti-C3d +ve

Tx:
- keep warm
- treat underlying cause
- rituximab
- chemotherapy

41
Q

DDx of red cell fragmentation syndrome

A
  1. cardiac haemolysis
  2. AVM
  3. Microangiopathic haemolytic anaemia
42
Q

When does the main switch from HbF to HbA occur after birth?

A

3~6 months

43
Q

Molecular pathogenesis of α thalassaemia
1. which is MC in HK?
2. which produce elongated but unstable chains?
3. which are associated with mental retardation?

A
  1. SEA deletion
  2. Hb Constant Spring, Hb Quong Sze
  3. ATR-16, ATR-X
44
Q

Investigations for HbH disease

A

PBS: target cells, >1 golf ball cells in new methylene blue stain
Hb electrophoresis: HbH

45
Q

Pathogenesis of β thalassaemia

A

point mutation –> reduced production / inactivation of β chains –> precipitation of excess α chains in BM –> ineffective erythropoiesis

46
Q

Which disease shows hair-on-end appearance on X-ray?

A

β thalassaemia

47
Q

Investigations for β thalassaemia (3)

A

PBS: target cells, Howell-Jolly bodies, basophilic stippling
Hb electrophoresis: ⨉ HbA, major HbF
DNA analysis

48
Q

Management of β thalassaemia (4)

A

regular transfusion
allogeneic SCT
folic acid
vitamin D, bisphosphonates

49
Q

Pathogenesis of sickle cell disease

A

sickle gene: [position 6 of β chain] glutamic acid => valine

low oxygen level –> sickle Hb polymerise into long fibres –> RBCs sickle –> block microcirculations –> organ infarct

50
Q

3 types of crisis in sickle cell anaemia

A

vaso-occlusive crisis
aplastic crisis
haemolytic crisis

51
Q

Investigations of sickle cell disease (3)

A

PBS: sickle cells, target cells, Howell-Jolly bodies
Screening test for sickling (test with deoxygenation)
Hb electrophoresis: HbS peak, ↑ HbF, ⨉ HbA

52
Q

Management for sickle cell disease

A

!! Transfusion is contraindicated (↓ O2 —> sickling)

Prophylaxis
[crisis] rest, warmth, rehydration
Hydroxyurea (↑ HbF)

53
Q

Which haemoglobinopathy combined with β trait will give thalassaemia major?

A

HbE

54
Q

PBS findings of megaloblastic anaemia

A

oval macrocytes, Howell-Jolly bodies
hypersegmented neutrophils
basophilic stippling

55
Q

Diagnosis of β-thalassaemia trait

A

HbA2 > 3.5%

MED4 ACP (13 decks)

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