A ghostly presence—G6PD deficiency Barbara J. Bain* Morphology update has already drawn attention to the importance of irregularly contracted cells and hemighosts in sug- gesting the diagnosis of hemolysis due to glucose-6-phosphate dehydrogenase (G6PD) deficiency [1]. The current patient, a man of African ancestry, suffered an acute fall of his hemoglobin concentration from normal to 65 g/l following exposure to rasburicase. The mean cell hemoglobin concentration (MCHC) was 362 g/l. Rasburicase had been administered prior to chemotherapy for Burkitt lymphoma. Examination of the blood film showed irregularly contracted cells and hemighosts, some containing visible Heinz bodies. In addition, there were considerable numbers of ghost cells—empty red cell mem- branes bereft of hemoglobin (images). Within some of the ghost cells, Heinz bodies were visible (black arrows, Heinz bodies in ghost cells; blue arrow, other ghost cells; red arrows, Heinz bodies in hemighost cells). The increased MCHC is attributable to the presence of irregularly contracted cells but is not specific, it is also a feature of spherocytic anemia. G6PD assay was performed and was normal. Subsequent assay, after resolution of the hemolytic episode, confirmed G6PD deficiency, A2 variant. An assay is particularly likely to be normal shortly after acute hemolysis in the A2 variant because of the normal level of the enzyme in reticulocytes. The history in this patient is strongly suggestive of G6PD deficiency and confirmatory evidence is provided by the blood film. Together they avoid the risk of misinterpretation of the initially normal assay. The presence of ghost cells indicates recent acute intravascular hemolysis. Reference 1. Bain B. Sudden onset of jaundice in a Sardinian man. Am J Hematol 2008; 83:810. Conflict of interest: Nothing to report. *Correspondence to: Barbara J. Bain, Department of Haematology, St. Mary’s Hospital Campus of Imperial College Faculty of Medicine, St. Mary’s Hospital, Praed Street, London W2 1NY, United Kingdom. E-mail: b.bain@ ic.ac.uk Received for publication 12 January 2010; Accepted 13 January 2010 Am. J. Hematol. 85:271, 2010. Published online 20 January 2010 in Wiley InterScience (www.interscience. wiley.com). DOI: 10.1002/ajh.21660 AJH Educational Material Morphology Update V V C 2010 Wiley-Liss, Inc. American Journal of Hematology 271 http://www3.interscience.wiley.com/cgi-bin/jhome/35105

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A ghostly presence—G6PD deficiency

Barbara J. Bain*

Morphology update has already drawn attention to the importance of irregularly contracted cells and hemighosts in sug-gesting the diagnosis of hemolysis due to glucose-6-phosphate dehydrogenase (G6PD) deficiency [1]. The current patient,a man of African ancestry, suffered an acute fall of his hemoglobin concentration from normal to 65 g/l following exposureto rasburicase. The mean cell hemoglobin concentration (MCHC) was 362 g/l. Rasburicase had been administered priorto chemotherapy for Burkitt lymphoma. Examination of the blood film showed irregularly contracted cells and hemighosts,some containing visible Heinz bodies. In addition, there were considerable numbers of ghost cells—empty red cell mem-branes bereft of hemoglobin (images). Within some of the ghost cells, Heinz bodies were visible (black arrows, Heinzbodies in ghost cells; blue arrow, other ghost cells; red arrows, Heinz bodies in hemighost cells). The increased MCHC isattributable to the presence of irregularly contracted cells but is not specific, it is also a feature of spherocytic anemia.G6PD assay was performed and was normal. Subsequent assay, after resolution of the hemolytic episode, confirmed

G6PD deficiency, A2 variant. An assay is particularly likely to be normal shortly after acute hemolysis in the A2 variantbecause of the normal level of the enzyme in reticulocytes.The history in this patient is strongly suggestive of G6PD deficiency and confirmatory evidence is provided by the blood

film. Together they avoid the risk of misinterpretation of the initially normal assay. The presence of ghost cells indicatesrecent acute intravascular hemolysis.

Reference1. Bain B. Sudden onset of jaundice in a Sardinian man. Am J Hematol 2008;

83:810.

Conflict of interest: Nothing to report.

*Correspondence to: Barbara J. Bain, Department of Haematology, St.Mary’s Hospital Campus of Imperial College Faculty of Medicine, St. Mary’sHospital, Praed Street, London W2 1NY, United Kingdom. E-mail: [email protected]

Received for publication 12 January 2010; Accepted 13 January 2010

Am. J. Hematol. 85:271, 2010.

Published online 20 January 2010 in Wiley InterScience (www.interscience.wiley.com).DOI: 10.1002/ajh.21660

AJH Educational Material Morphology Update

VVC 2010 Wiley-Liss, Inc.

American Journal of Hematology 271 http://www3.interscience.wiley.com/cgi-bin/jhome/35105