Chapter 22 Aplastic anaemia and bone marrow failure

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22.1 Which ONE of the following commonly causes pancytopenia?

A Parvovirus infection

This virus infects red blood cells and does not affect the leucocyte or platelet lineage.

B Normal pregnancy

This typically causes thrombocytopenia, and anaemia can result if iron deficiency is present, but not
pancytopenia.

C Acute myeloid leukaemia

AML can present with leucocytosis, but often (especially in older patients) presents clinically with
suppression of all the blood cell lines.

D Anti-thyroid drugs

These can cause agranulocytosis.

22.2 Which ONE of the following is a recurrent cause of aplastic anaemia?

A Penicillin

No association.

B Sulphonamide

This is a rare but dangerous side-effect.

C Quinine

Associated with thrombocytopenia.

D Furosemide

This is a cause of agranulocytosis or thrombocytopenia.

22.3 Which ONE of the following is associated with ‘pure’ red cell aplasia?

A Haemophagocytic lymphohistiocytosis

A cause of pancytopenia.

B Methotrexate

A cause of pancytopenia.

C Thymoma
:
 Clear association with red cell aplasia.

D Ionising radiation

A cause of pancytopenia.

22.4 Which ONE of these is a feature of the blood count and bone marrow in patients with
severe acquired aplastic anaemia?

A Abnormal ‘blast’ cells on the blood film

This is seen in acute leukaemia.

B Hyperplastic bone marrow

By definition, the marrow is hypoplastic.

C Reduced number but normal appearance of neutrophils on the blood film

D Normal lymphocyte count

This is usually low in severe aplastic anaemia.

22.5 Which ONE of these statements is TRUE concerning the management of aplastic
anaemia?

A Corticosteroids are the most appropriate initial therapy

Steroids are not effective and should be avoided except to reduce adverse effects of antilymphocyte
globulin.

B Erythropoiesis-stimulating agents are useful agents in treatment

These are ineffective due to lack of erythroid precursors in the marrow.

C Antilymphocyte globulin is of benefit in almost all cases

This is about 50%.

D Eltrombopag can be indicated for treatment

This is approved by regulatory authorities for relapsed aplastic anaemia and may also be useful in front-line
therapy.

22.6 Which ONE of the following marrow failure syndromes also includes exocrine pancreatic
dysfunction? (P)

A Fanconi anaemia

B Shwachman–Diamond syndrome

C Blackfan–Diamond syndrome

D GATA2 deficiency

22.7 Which ONE of these statements is TRUE concerning congenital dyserythropoietic

anaemias? (P)
:
 A They may respond to pyridoxine

This is true for congenital sideroblastic anaemia.

B They show ineffective erythropoiesis and erythroblast multinuclearity

C They are often triggered by HIV infection

No association.

D The anaemia may cause hydrops fetalis

The anaemia is not severe enough in the fetus to cause this.

22.8 Which ONE of these statements is TRUE concerning Fanconi anaemia?

A It may be due to a mutation in the ATM gene

There are more than 16 different genetic variants, but these do not include the ATM gene.

B The diagnostic test is elevated chromosome breakage after incubation of peripheral blood lymphocytes
with a DNA cross-linking agent such as diepoxybutane (DEB test)

C It is associated with cardiac abnormalities

These are not an association.

D It most commonly has a sex-linked inheritance

Most forms have an autosomal recessive inheritance.

22.9 Which ONE of these abnormalities is a typical feature of Fanconi anaemia?

A Immune deficiency

This is not a feature; it is characteristic of some other inherited marrow failure syndromes such as GATA2
germline mutations.

B Fingernail and toenail abnormalities

This is a feature of dyskeratosis congenita, not Fanconi anaemia.

C Abnormal skin pigmentation

D Increased haemoglobin F expression

Characteristic of some patients with thalassaemia or juvenile leukaemias.

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