Haem MRCPCH

Question 1

Acanthocytes - Name four causes for presence of acanthocytes on peripheral film

Answer 1

1. Abetalipoproteinaemia
2. Severe liver disease
3. Vitamin E deficiency in premature neonates
4. hereditary acanthocytosis

Question 2

BASOPHILIA - Name four causes of basophilia

Answer 2

1. Myeloproliferative disorders
2. chronic myeloid leukaemia (CML)
3. basophilic leukaemia
4. Reactive disorders, e.g. ulcerative colitis, infection

Question 3

BASOPHILIC STIPPLING - name four causes

Answer 3

1. Ineffective erythropoiesis
2. Haemoglobinopathies
3. Recovering bone marrow
4. Lead poisoning

Question 4

ECHINOCYTES (BURR CELLS) - give 5 causes

Answer 4

1. Liver disease
2. Renal failure
3. HUS
4. Burns
5. pyruvate kinase deficiency

Question 5

ELLIPTOCYTES - give a cause

Answer 5

Hereditary elliptocytosis

Question 6

EOSINOPHILIA - give four causes

Answer 6

1. Parasitic infections
2. allergic states, e.g. asthma, eczema
3. Drugs
4. Polyarteritis

Question 7

SCHISTOCYTES (fragmented red blood cells)

Answer 7

1. Microangiopathic anaemia
2. Mechanical haemolytic anaemias
3. DIC
4. HUS
5. Renal failure

Question 8

Heinz bodies (intracellular Hb precipitate) - give 5 causes

Answer 8

1. G6PD deficiency
2. haemoglobinopathies
3. post-splenectomy
4. hyposplenism
5. Heinz body haemolysis

Question 9

Howell– Jolly bodies (intracellular DNA fragments) - 4 causes

Answer 9

1. Normal neonatal blood picture
2. hyposplenia
3. post-splenectomy
4. megaloblastic anaemia

Question 10

Lymphocytopenia (lymphopenia) - causes

Answer 10

1. Infection, mainly viral
2. malignancy
3. stress
4. vomiting
5. burns
6. anorexia
7. drugs
8. SLE
9. Crohn’s disease
10. immunodeficiency states (SCID, diGeorge syndrome, acquired, e.g. HIV)
11. marrow failure
12. aplastic anaemia
13. leukaemia

Question 11

LYMPHOCYTOSIS

Answer 11

1. Infection - pertussis, mycoplasma, malaria, viruses
2. leukaemia
3. Atypical lymphocytosis (EBV, CMV, adenovirus)
4. stress
5. exercise
6. status epilepticus

Question 12

MACROCYTIC RBCs - 3 causes

Answer 12

1. Vitamin B12 or folate deficiency
2. aplastic anaemia
3. normal neonatal blood picture

Question 13

MICROCYTIC RBCs - causes

Answer 13

1. Iron deficiency
2. thalassaemia
3. anaemia of chronic disease

Question 14

MONOCYTOPAENIA - causes

Answer 14

1. Autoimmune disorders, e.g. SLE
2. Drugs, e.g. corticosteroids, chemotherapy

Question 15

MONOCYTOSIS

Answer 15

1. Chronic bacterial infection
2. Malaria
3. Typhoid
4. TB
5. Infective endocarditis
6. Post-chemotherapy
7. JMML, CML

Question 16

NEUTROPHILIA - Causes

Answer 16

1. Infection
2. inflammation
3. chronic bleeding
4. post-splenectomy
5. drugs, e.g. corticosteroids

Question 17

Reticulocytosis/ polychromatic RBCs - causes

Answer 17

1. Haemolysis
2. bleeding
3. response to haemotinics (e.g. iron)
4. marrow infiltration
5. co-existent megaloblastic and iron deficiency anaemia?

Question 18

SPHEROCYTES - causes

Answer 18

1. Normal neonatal blood picture
2. hereditary spherocytosis
3. immune mediated haemolytic disease
4. post-splenectomy

Question 19

TARGET CELLS - Causes

Answer 19

1. Severe iron deficiency
2. sickle cell disease
3. Thalassaemia
4. Liver disease
5. Post-splenectomy, asplenia

Question 20

Immune haemolytic anaemia (OHP)

Answer 20

Immune haemolytic anaemia

In this group of disorders, RBCs react with autoantibody +/ − complement, which leads to their destruction by the reticuloendothelial system. Many drugs can induce antibody-mediated haemolysis, e.g. penicillins, cephalosporins, ibuprofen, anti-malarials, rifampicin, antihistamines. Mechanisms are variable. Immune haemolytic anaemia can be divided into isoimmune and autoimmune forms.

Isoimmune

Sensitization induces maternal red cell antibodies that cross placenta and haemolyse foetal and neonatal red cells. Usually, direct Coombs test + ve.

• Rhesus haemolytic disease.
• ABO incompatability.
• Other blood group incompatibilities, e.g. Kell, Duffy, blood groups

Autoimmune

Warm antibody type— mostly IgG

• Rare.
• Majority are idiopathic.
• Other causes: drugs (e.g. penicillin), lymphoid malignancies, autoimmune diseases (e.g. SLE, IBD).
• Variable haemolytic anaemia, mild jaundice, splenomegaly, DCT + ve.
• Warm autoantibodies— often non-specific.
• Treatments include IVIG, steroids, other immunosuppressants, splenectomy, rituximab

Cold antibody type— mostly IgM

• Very rare in children except PCH (see Paroxysmal cold haemoglobinuria).
• RBC antibody reacts most actively < 32 ° C to cause intravascular RBC haemolysis.
• Idiopathic or secondary to EBV or Mycoplasma infection.
• Acrocyanosis in cold, splenomegaly.
• Chronic haemolytic anaemia, DCT − ve for IgG, + ve for C3.
• IgM autoantibodies react best at 4 ° C.
• Treatment: Treatment rarely needed. Warmth, immunosuppression, plasma exchange, and splenectomy may help. Usually, the condition is self-limiting if there is an infectious cause.

Paroxysmal cold haemoglobinuria (PCH)

• 2 ° to infections (varicella, measles, syphilis) and vaccinations.
• Acute onset of intravascular haemolysis, after fever and chills.
• Due to a biphasic antibody, called Donath Landsteiner antibody.
• Antibody fixes on the cells in the cold peripheries and lyses in the central warmth of the body— protect from cold.
• Transfuse as required. Condition is self limiting.