ANAEMIA

Question 1

Name 6 Normocytic Haemolytic anaemias

Answer 1

Sickle Cell disease

Autoimmune Haemolytic Anaemia (Warm IgG, Cold IgM)

Microangiopathic Haemolytic Anaemia
- TTP, DIC, HUS

G6PD Deficiency

Falciparum Malaria

Hereditary Spherocytosis

Question 2

Thrombotic Thrombocytopenic purpura (TTP)

• type of anaemia
• pathophysiology
• presentations

Answer 2

TTP = Normocytic Haemolytic

ADAMTS13

• Fever
• NEURO SnS
• Splenomegaly
• Haemolytic anemia
• Thrombocytopenia - easy bruising, purpura, petechia
• acute renal failure

Question 3

Pt presents with 1wk of fever, numbness and difficulty speaking.

Splenomegaly is noted, and purpura on extremities.

Bloods show normocytic anaemia, decreased plts, high creatinine

Diagnosis?

Answer 3

Thrombotic Thrombocytopenic Purpura (TTP)

Pathophys: ADAMTS13 deficiency

• fever
• Neuro SnS
• Splenomegaly
• thrombocytopenia
• renal failure (creatinine)

Question 4

Immune Thrombocytopenia

• pathophysiology
• presentations

Answer 4

Pathophysiology - immune destruction of platelets

• purpura
• gingival bleeding

(NO fever/ neuro/ splenomegaly)

Question 5

Haemolytic-Uraemic syndrome (HUS)

• Pathophysiology
• Presentations

Answer 5

Pathophysiology: Children, following STEC (shigella-toxin E coli)

1. thrombocytopenia
2. haemolytic anaemia
3. acute renal failure

(NO Neuro)

Question 6

Microangiopathic Haemolytic anaemia blood film

Answer 6

Schistocytes - Intravascular haemolysis

Question 7

Autoimmune Haemolytic anaemia

Types? Investigations? Management?

Answer 7

Warm - IgG (SLE!!)
Cold - IgM

Investigations: DIRECT COOMBS TEST

PBS - Spherocytes

Mx: Corticosteroids

Question 8

Sickle Cell disease

presentations
Investigations
Management

Answer 8

1. bone pain
2. dactylitis
3. thrombotic event
4. (Malaria - endemic)

Ix:

1. PBS - Sickle RBC, Howell-Jolly, Nucleated RBC
2. Hb electrophoresis - HbS

Mx: Hydroxycarbamide + frequent transfusions

Question 9

Howell-Jolly bodies suggest…

Answer 9

Sickle cell disease

Question 10

Hereditary Spherocytosis

Presentations? RF? Pathophysiology? Investigations? Management?

Answer 10

1. Neonatal/childhood
2. Jaundice
3. Anaemia
4. splenomegaly

RF: NORTHERN-EUROPE

Pathophys: RBC Spectrin defect > spherical RBC

Ix:
1. PBS: spherocytes, ^LDH, low haptoglobin

2. EMA BINDING TEST
   - reduced EMA fluorescence

Mx:

• Folic acid
• transfusions
• Splenectomy before 5 is curative!!