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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