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Pathology · Haematology: sickle cell disease
Sample itemA 9-year-old boy with sickle cell disease has pallor, tiredness and fever after a viral illness with a slapped-cheek rash in a sibling. Haemoglobin has fallen from 8 to 4 g/dL and the reticulocyte count is 0.1%. What is the most likely complication?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Aplastic crisis due to parvovirus B19 infection of erythroid precursors
Parvovirus B19 infects erythroid progenitors and halts red cell production. In a patient with shortened red cell survival this causes an abrupt fall in haemoglobin with reticulocytopenia. Sequestration and hyperhaemolysis show raised reticulocyte counts and, in sequestration, a large spleen.
Option by option
A. Aplastic crisis due to parvovirus B19 infection of erythroid precursors (correct)
Correct. A very low reticulocyte count with a sharp haemoglobin fall points to aplastic crisis.
B. Vaso-occlusive crisis
Vaso-occlusive crisis causes pain and does not suppress reticulocytes.
C. Hyperhaemolytic crisis
Hyperhaemolysis raises the reticulocyte count, whereas here it is suppressed.
D. Splenic sequestration crisis
Sequestration produces acute splenomegaly with a high reticulocyte count.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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