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Haematology > FBE > Flashcards

FBE Flashcards

1
Q

Microcytic anaemia - causes

A

Common
- iron deficiency
- thalassaemia

Less common
- anaemia of chronic disease
- sideroblastic anaemia
- hyperthroidism
- heavy metal poisoning (lead)

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2
Q

Iron deficiency anaemia - causes

A

GI bleeding (ulcer, malignancy, diverticulitis)
Menstruation
Diet
Other (coeliac disease, gastrectomy, increased demand, polycycthemia rubra vera, bleeding disorders, pulmonary haemosiderosis)

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3
Q

IDA - film abnormalities and iron studies

A

microcytic, hypochromic red cells, pencil cells, increased platelets, increased RDW
Increased transferrin, reduced T-Sats, ferritin reduced

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4
Q

Anaemia of chronic disease
- prevalence
- causes
- blood film
- iron studies

A

2nd most common cause
- chronic inflammatory state, severe trauma, diabetes, elderly, obesity
- normocytic and normochromic but occasionally microcytic
- ferritin normal or increased and transferrin normal to low

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5
Q

Anaemia of chronic disease - pathophysiology

A

altered iron homeostasis, blunted EPO response, shortened red cell survival
- key players: IL-1b, IL-6, TNF-a cause increased hepcidin

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6
Q

Anaemia of chronic disease - management

A

treat underlying cause
EPO - variable response
Replace iron if deficient

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7
Q

Macrocytic anaemia
- causes

A

B12/folate deficiency
drugs: antifolate drugs (MTX, pentamadine, trimethoprim) DNA synthesis inhibitors (aza, hydroxyurea, phenytoin)
Rediculocytisis

Others:
BM pathology, liver disease, alcohol, copper deficiency, Down syndrome, factitious

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8
Q

Vitamin B12
- stores
- source
- absorption

A

2-4 years worth
absorbed from animal products
Bound to haptocorrin in presence of acid in stomach
In small intestine haptocorrin replaced by intrinsic factor
Absorbed in ileum by transcobaamin

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9
Q

What does a high methyl-malonic acid indicate?

A

B12 deficiency

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10
Q

Causes of B12 deficiency

A

pernicious anaemia - autoimmune destruction of parietal cells
Vegans
Ileal pathology (Crohns, ileal resection, tropical sprue, tapeworm),
Gastrectomy,
Congenital deficiency
Nitrous oxide poisoning

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11
Q

Pernicious anaemia serological testing

A

intrinsic factor antibodies - specific but not sensitive
parietal cell antibodies - sensitive but not specific

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12
Q

Blood film features of B12 deficiency
Bone marrow features
Other features

A

macrocytic anaemia
hypersegmented neutrophils
oval macrocytes
low reticulocyte count
pancytopaenia can occur

Megaloblastic on BM

Haemolysis can also be seen

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13
Q

Folate deficiency
- source
- stores
- absorption
- causes
- symptoms

A

diet
stores - months
small intestine
causes: reduced intake, increased requirements, reduced absorption
symptoms: fatigue, lethargy, sore tongue, headache, diarrhoea, irritability
Anaemia is seen with more severe deficiency

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14
Q

Folate deficiency investigations

A

red cell folate level (long term stores), serum folate (indicates recent intake)

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15
Q

Factors of haemolysis screen

A

reticulocyte count, LDH, haptoglobin, bilirubin (unconjugated)

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16
Q

What factors can cause low haptoglobin other than haemolysis

A

Liver failure**

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17
Q

Sites of haemolysis and their associated features

A

intravascular - very low haptoglobin, elevated urinary hemosiderin
extravascular - (liver, spleen, bone marrow), low-normal haptoglobin, negative urinary haemosiderin

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18
Q

Microangiopathic haemalytic anaemia (MAHA) causes: (8)

A
  • TTP
  • HUS
  • pre-eclampsia, HELLP, malignant hypertension, renal allograft rejection
  • Atypical HUS: inherited dysregulation of complement system (complement factor H) treated with eculizumab
  • DIC
  • Mechanical haemolytic anaemia (severe valvular disease or mechanical valve)
  • Vascular malformations
  • direct damage by heat, venom, toxins
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19
Q

TTP pentad
- how often are all 5 seen?

A

haemolysis with red cell fragmentation
thrombocytopaenia
fever
neurological changes
renal impairment
<10% have all 5

20
Q

TTP pathogenesis / causes (4)

A

increased high molecular weight von Willebrand Factor
ADAMTS13 deficiency (the protease that cleaves vWF)
Acquired antibodies against ADAMTS13 (most common)
Hereditary ADAMTS13 deficiency

21
Q

TTP diagnostic work up

A

clinical features + fragmentation
measure ADAMTS13 level (takes a long time)

22
Q

TTP management

A

Urgent plasma exchange
Avoid platelet transfusions
Immunosuppression (steroids or rituximab)
Exclude pregnancy or other causes
- if hereditary TTP can replace ADAMST13 with FFP infusions

23
Q

What is Caplacizumab?

A

targets vWF
Stops binding of platelets
Only a bandaid, treat underlying cause1

24
Q

What is Eculizumab?
- target
- uses
- risks

A

Anti-C5 humanised chimeric MAB
Targets terminal complement system
used for atypical HUS and paroxysmal nocturnal haemoglobulinuria
associated with increased risk of infection by encapsulated organisms

25
Q

Paroxysmal nocturnal haemoglobulinuria
- cause

A

somatic mutation in pig-A gene
anchors cell surface proteins
when absent makes cells vulnerable to complement mediated cell lysis

26
Q

Paroxysmal nocturnal haemoglobulinuria
- symptoms
- associations
- diagnosis

A

intravascular haemolysis
- macroscopy haematuria, iron deficiency, increased risk of thrombosis

associated with: aplastic anaemia, MDS
diagnosis by flow cytometry with CD55 and CD59 on neutrophils

27
Q

Paroxysmal nocturnal haemoglobulinuria
- managment

A

supportive care, stem cell transplantation
thrombosis management
eculizumab: fewer transfusions and less haemoglobulinuria

28
Q

Paroxysmal cold haemoglobinuria
- type of haemolysis
- causes
- Ab type
- pathophysiology

A
  • intravascular haemolysis after exposure to cold
  • causes: idiopathic, syphilis, viral infection
  • IgG against anti-P antibody
  • binds RBC at low temperatures and upon warming complement-mediated lysis occurs
29
Q

Autoimmune haemolytic anaemia
- types

A

warm or cold
primary or seondary

30
Q

Warm AIHA
- Ab type
- causes

A

IgG +/- Complement
idiopathic
SLE
lymphoproliferative disorders
Drugs: methyldopa, antibiotics
Evan’s syndrome- AIHA with ITP

31
Q

Warm AIHA
- film findings
- other bloods

A

anaemia, haemolysis, spherocytosis, splenomegaly
DAT - demonstrate autoantibodies

32
Q

Warm AIHA
- treatment

A

Prednisolone 1mg/kg then taper
Intragam
Folate supplementation
Consider splenectomy and vaccinations
Rituximab

33
Q

Cold AIHA
- antibody target and type
- causes
- management

A

Anti-C3d only (IgM)
almost always secondary; lymphoproliferative disorders, mycoplasma, EBV, autoimmune disease
avoid cold, rituximab
**does not respond to steroids or splenectomy
?C1s inhibitor sutimlimab (phase 3 trials)

34
Q

RBC membrane defect types

A

Hereditary spherocytosis
Hereditary elliptocytosis
South-East Asian ovalocytosis

35
Q

Hereditary spherocytosis
- investigation finding
- management

A

spherocytosis, DAT negative, flow cytometry - EMA binding
folate supplementation, splenectomy

36
Q

Red cell enzyme defects

A

glycolytic pathway (2,3-DPG, Pyruvate kinase)
Hexose-monophosphate pathway (G6PD deficiency)

37
Q

G6PD mode of inheritance

A

x-linked

38
Q

Haemoglobin types

A

HbA (alpha 2, beta 2) - adults
HbA2 (alpha 2, delta 2) - small amount in adults
Hb F (alpha 2, gamma 2) - neonates

39
Q

How many alpha genes and beta genes of globin are present usually

A

2 alpha genes (4 copies)
1 beta gene (2 copies)

40
Q

Alpha-thalassaemia
- trait
- HbH disease
- hydrops
- minor
- major

A

trait - 3 functioning copies
HbH disease - 1 functioning copy
Hydrops foetalis - no functioning copies

41
Q

Beta-thalassaemia
- trait

A

trait - 1 copy, reduced MCV, increased HbA2 and HbF
major - 0 copies, severely anaemic, absent HbA

42
Q

Sickle Cell Disease
- globin gene affected
- mutation

A

beta globin
valine substituted by glutamic acid

43
Q

Sickle Cell Disease
- management

A

Hydroxyurea
Transfusions
Monitor HbS level
Manage hyposplenism
Voxelotor (HbS polymerisation inhibitor) increases Hb and reduces haemolysis

44
Q

ITP
- demographic affected
- lab findings
- pathophysioogy
- disease associations

A

common in adults and children, F>M
thrombocytopaenia (isolated)
antibody mediated platelet destruction by liver and spleen
associated with AIHA, CLL, autoimmune diseases, H pylori, Hep C

45
Q

ITP
- management

A

IVIG (rapid)
Prednisolone
Splenectomy
Rituximab

46
Q

Heparin induced thrombocytopaenia
- Ab present
- scoring system used
- diagnosis
- management

A

IgG to heparin-PF4 complex
4-T score
Diagnosed by immuno-assay or functional assay
Cease heparin, anticoagulation with direct thrombin or Xa inhibitor

47
Q

What is acute chest syndrome

A

hypoxia and pain, new pulmonary infiltrates, usually post operative in context of sickle cell disease

Haematology (5 decks)

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