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IMAGES IN HEMATOLOGY

A rare case of autoimmune hemolytic anemia

A B

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c Orianne Wagner-Ballon and Marc Michel, APHP, Hôpitaux Universitaires Henri Mondor

A 47-year-old white woman, who was an active smoker, was referred to the emergency department for the
recent onset of exertional dyspnea with chest pain. She did not have a remarkable medical history or family
history of anemia and received no medications, although she took pain medication occasionally. Her clinical
examination revealed pallor and conjunctival jaundice with mild hepatosplenomegaly. She displayed isolated
macrocytic (mean corpuscular volume, 125 fL) anemia (hemoglobin, 4.2 g/dL). An elevated automated
reticulocyte count (603 3 109/L) was confirmed by supravital staining with brilliant cresyl blue (panel B; many
cells display a typical blue-stained reticulum, original magnification 31000). Hemolysis was confirmed (lactate
dehydrogenase, 2866 IU/L; bilirubin, 62 mmol/L; haptoglobin, ,0.1 g/L). The direct antiglobulin test (DAT)
was originally negative, however, the examination of the blood smear showed numerous spherocytes (panel A;
May-Grünwald–Giemsa stain shows sphere-shaped erythrocytes, original magnification 31000) that looked
consistent with autoimmune hemolytic anemia (AIHA) in the absence of a familial hemolysis history. Hence, a
new extensive DAT was performed and revealed the presence of erythrocyte-bound immunoglobulin A (IgA)
autoantibodies. Additional exploration revealed chronic nonactive hepatitis C. The severe AIHA was treated with
corticosteroids, rituximab, azathioprine, and eventually responded to splenectomy.
Such DAT-negative AIHAs remain rare (;3%-5% of all AHAIs). Warm IgA antibody AIHAs represent 1% to
2% of all warm AIHAs. This case highlights the relevance of careful blood-smear examination, which can
avoid costly investigation of unexplained hemolytic anemia.

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DOI 10.1182/blood-2017-05-783514 © 2017 by The American Society of Hematology

BLOOD, 27 JULY 2017 x VOLUME 130, NUMBER 4 559