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Year 4 > Haematology seminar 1 > Flashcards

Haematology seminar 1 Flashcards

1
Q

What are some examination findings of anaemia?

A

Pallor
Jaundice
Splenomegaly
Lymphadenopathy
Koilonychia

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2
Q

What is microcytic hypochromic anaemia?

A

When the red blood cells are small and pale in colour
Caused by iron deficiency and it’s a thalassaemia trait

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3
Q

How can anaemia arise?

A

Problems of synthesis
Over-consumption
Bleeding

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4
Q

What is thalassaemia?

A

When the body doesn’t make enough haemoglobin

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5
Q

What causes normocytic anaemia?

A

Its anaemia of chronic disease caused by bone marrow failure or bleeding

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6
Q

What causes macrocytic anaemia?

A

B12/folate deficiency
Myelodysplasia Haemolysis (reticulocytosis)
-Pregnancy
-Hypothyroidism
-Alcoholism

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7
Q

What is macrocytic anaemia?

A

The red blood cells are larger than normal

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8
Q

What is included in the haemolysis screen?

A
  • Reticulocytes
  • Direct anti-globulin test (DAT = Coomb’s test)
  • Bilirubin
  • LDH
  • Blood film
    Eg. Spherocytes, Sickle cells, RBC fragments
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9
Q

What are reticulocytes?

A

Immature red blood cells

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10
Q

What is Haemoglobinopathy?

A

Inherited disorders of globin, the protein component of haemoglobin

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11
Q

What are the different problems of synthesis causing anaemia?

A

Haematinic deficiency
- Iron deficiency (low ferritin)
- B12 (pernicious anaemia, gastrectomy or Crohn’s)
- Folate deficiency (diet, pregnancy or malabsorption e.g. coeliac)

Bone marrow failure
- Serum protein electrophoresis (myeloma?)
- Pancytopenia (bone marrow biopsy needed)
- Blasts (leukaemia)
- High MCV (myelodysplasia)

Renal failure
- Raised creatinine
- Low Epo levels

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12
Q

What is the treatment for iron deficiency?

A

Iron sulphate tablets 200mg TDS for 3 months

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13
Q

How is folate deficiency treated?

A

5mg oral OD for 3 months plus dietary advice

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14
Q

How is B12 deficiency treated?

A

Hydroxycobalamin 1mg IM injections for 5 alternating days then every 3 months

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15
Q

What is Hydroxycobalamin used for?

A

It’s a form of injectable B12

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16
Q

How does Erythropoeitin work?

A

It’s naturally produced by the peritubular cells of the kidney

It stimulates red blood cell production

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17
Q

What is hereditary spherocytosis?

A

Instead of being shaped like a disk, the cells are round like a sphere

These red blood cells (called spherocytes) are more fragile than disk-shaped RBCs

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18
Q

How does G6PD cause haemolytic disease?

A

Without Glucose-6-phosphate dehydrogenase to protect the RBC’s, they break apart

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19
Q

What is autoimmune haemolysis?

A

Premature destruction of healthy red blood cells

Warm (IgG-mediated): happens at room temperature
Cold (IgM): triggered by cold

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20
Q

What is valve haemolysis?

A

A complication of prosthetic heart valves

21
Q

What are the 2 types of bone marrow biopsy?

A

Aspirate (marrow smears – haematologists)
Trephine (tissue sections – pathologists)

22
Q

What is aplastic anaemia?

A

When the body doesn’t make enough RBC’s because the immune system attacking the stem cells in your bone marrow

23
Q

Name some haematological marrow diseases

A

Leukaemia
Lymphoma
Myeloma
Myelodysplasia
Myelofibrosis

24
Q

What is acute myeloid leukaemia?

A
  • Mostly older adults (>60 yrs)
    -25% of childhood cancers
  • Cure rate <50%, prognosis depends on cytogenetics and gene mutations
  • Treatment with intensive chemotherapy (DA 3+10 regime)
  • Allogeneic stem cell transplants is potentially curative
25
Q

What is acute lymphoid leukaemia?

A

Commonest childhood cancer (mostly <10yrs)

-Cure rate >90%
- Treatment with chemotherapy for 2 years

26
Q

What is chronic myeloid leukaemia?

A

High WCC (leucocytosis)

Massive splenomegaly

27
Q

What is the Philadelphia chromosome?

A

Abnormal fusion protein = BCR-ABL tyrosine kinase

Causes CML

28
Q

What is the treatment for CML?

A

Imatinib
It inhibits the ATP pocket of BCR-ABL

29
Q

What is Myelodysplasia?

A

A group of cancers in which immature blood cells in the bone marrow do not mature or become healthy blood cells

Causes anaemia, neutropenia, thrombocytopenia

It can transform into AML
Treated with Azacytidine (elderly) or curative allografting (young)

30
Q

What is Myelofibrosis?

A

Fibrosis of the bone marrow causing pancytopenia

  • Typically JAK2+ve
  • Transforms eventually into AML
    Treated with hydroxycarbamide or JAK inhibitors or allografting
31
Q

What is excess myelopoiesis in bone marrow?

A

Myelopoiesis: the production of myeloid cells by bone marrow hematopoietic stem and progenitor cells

Typically carry JAK2 V617F mutation

Excessive production causes a risk of thrombotic events

32
Q

What is Polycythemia rubra vera?

A

High Hb and haematocrit

Treat with venesection

33
Q

What is essential thrombocythemia?

A

High platelet count

Treated with hydroxycarbamide

34
Q

How does hydroxycarbamide work?

A

It works by increasing foetal haemoglobin

35
Q

What is myeloma?

A

Malignancy of plasma cells, it’s treatable but incurable

Treatment:
- Chemotherapy for 4-6 months (cyclophosphamide, Velcade/thalidomide, steroids)
- Autologous stem cell transplant if young and fit

36
Q

When should myeloma be concidered?

A

Hypercalcaemia
Renal failure
Unexplained anaemia
Bone pain or pathological fractures

37
Q

What is the CRAB acronym for end organ failure?

A

Hyperkalaemia
Renal failure
Anaemia
Bone disease

It most typical clinical manifestations of multiple myeloma

38
Q

What is the work up for myeloma?

A

Blood tests
- Haematology: anaemia, rouleaux, viscosity, ESR
– Biochemistry: U+Es, Ca, Albumin, total protein, LDH, CRP, β2-M

Serum protein electrophoresis
- Paraprotein (Monoclonal M band)
- Immunoglobulin levels low (immune paresis)

Serum free light chain assay
- Abnormal κ/λ light chain ratio
- Staging investigations

39
Q

What is ITP?

A

Immune thrombocytopenic purpura

A rare autoimmune disorder where the blood doesn’t clot properly because the immune system destroys the platelets

40
Q

What are the symptoms of Immune thrombocytopenic purpura?

A
  • Bruising
  • Nose bleeds
  • GI bleeds
  • Major haemorrhage

Usually self-limiting in children

41
Q

What is AIHA?

A

Autoimmune haemolytic anaemia

42
Q

What are the symptoms of autoimmune haemolytic anaemia?

A

Pallor
Jaundice
Dark urine

43
Q

How is autoimmune haemolytic anaemia treated?

A

Transfuse as required (but difficulty cross-matching)

– Warm AIHA – treat with steroids, splenectomy, Rituximab
– Cold CHAD – treat with immunosuppression

44
Q

What are the causes of splenomegaly?

A

Haematological causes
- Myeloproliferative disorders
- Lymphoproliferative disorders
- Haemolytic states

Infective causes
- Viral illness – EBV, CMV, HIV
- Tropical (malaria, schistosomiasis)

Portal hypertension due to liver disease
Rheumatoid arthritis (Felty’s syndrome)

45
Q

What is Haemophilia?

A

A = Factor VIII
B = Factor IX (Christmas disease)

Both cause prolonged APTT
X-linked recessive (males only affected)

Causes severe bleeding into joints and muscles

Treat with recombinant factor product (or DDAVP if mild)
Treat prophylatically if severe haemophilia (<1% factor levels)

46
Q

What is von Willebrand’s disease?

A

Deficiency of von Willebrand factor (vWF)

Low factor VIII and platelet aggregation causing mild bleeding into mucous membranes

Treat with DDAVP or Haemate P

47
Q

How does Warfarin work?

A

It prevents γ-carboxylation of factors II, VII, IX, X

Monitored by INR (based on prothrombin time) but watch drug interactions

Reversed by vitamin K or Octaplex (25-50 IU/kg) if major bleed

Teratogenic in pregnancy; safe in renal failure

48
Q

How does Heparin work?

A

It blocks thrombin to prevent clotting

LMWH
- Dosed by weight, no monitoring required
- Safe in pregnancy
- Unsafe in renal failure

Unfractionated Heparin
- Safe in renal failure
- Reversed by protamine sulphate

49
Q

What is anti-phospholipid syndrome?

A

An autoimmune disorder that causes abnormal blood clots to form

Treatment:
– Anticoagulation with warfarin
– LMW heparin and aspirin in pregnancy

Year 4 (100 decks)

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