|LETTER TO THE EDITOR
|Year : 2017 | Volume
| Issue : 3 | Page : 284
Early-onset evans syndrome in a 4-month-old infant: A case report and review of literature
Mahmood D Al-Mendalawi
Department of Paediatrics, Al-Kindy College of Medicine, University of Baghdad, Baghdad, Iraq
|Date of Web Publication||21-Aug-2017|
Mahmood D Al-Mendalawi
Department of Paediatrics, Al-Kindy College of Medicine, University of Baghdad, Baghdad, P.O. Box 55302, Baghdad Post Office, Baghdad
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Al-Mendalawi MD. Early-onset evans syndrome in a 4-month-old infant: A case report and review of literature. Saudi J Med Med Sci 2017;5:284
|How to cite this URL:|
Al-Mendalawi MD. Early-onset evans syndrome in a 4-month-old infant: A case report and review of literature. Saudi J Med Med Sci [serial online] 2017 [cited 2022 Dec 4];5:284. Available from: https://www.sjmms.net/text.asp?2017/5/3/284/213308
I have two comments on the interesting case report by Mohamed et al. titled “Early-onset Evans syndrome in a 4-month-old infant.”
First, the authors mentioned that autoimmune hemolytic anemia and thrombocytopenia in association with a positive direct Coombs test (DCT) confirmed the diagnosis of Evans syndrome (ES) in the studied infant. In the diagnosis of ES, it should be noted that although DCT is almost invariably positive (often weakly) for immunoglobulin G (IgG), complement, or both, negative DCT has also been reported., In these cases, there are primarily three reasons for negative DCT. First, in some commercial antiglobulin reagents, IgG sensitization is below the detection threshold. Second, if preparatory washes are not conducted at 4°C or at low ionic strength, it may result in low-affinity IgG being removed. Third, sensitization of red cell with an IgA alone or, in rare cases, with a low molecular weight (monomeric) IgM not accompanied by complement fixation may result in negative DCT because many commercial antiglobulin reagents comprise only anti-IgG and anti-C3. Accordingly, I presume that absolute reliance on positive DCT as a critical cornerstone in the diagnostic algorithm for ES may result in ES being undiagnosed in a sizeable number of patients.
Second, the case report further supports the observation of early-onset ES in young infants, as ES has also been reported in neonates in pediatric literature.
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Conflicts of interest
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| References|| |
Mohamed KK, Al-Qurashi FO, Al-Qahtani MH, Osman YS. Early-onset Evans syndrome in a 4-month-old infant: A case report and review of literature. Saudi J Med Med Sci 2017;5:177-80. [Full text]
Ganly PS, Laffan MA, Owen I, Hows JM. Auto-anti-Jka in Evans' syndrome with negative direct antiglobulin test. Br J Haematol 1988;69:537-9.
Maheshwari VD, Kumar R, Singh S. Coomb's negative autoimmune hemolytic anemia with thrombocytopenia (Evan's syndrome). J Assoc Physicians India 2002;50:457-8.
Segel GB, Lichtman MA. Direct antiglobulin (”Coombs”) test-negative autoimmune hemolytic anemia: A review. Blood Cells Mol Dis 2014;52:152-60.
Gamboa-Marrufo JD, Hernández-Caldera S, Bello-González A. Evans syndrome. Presentation of a case in a newborn infant. Bol Med Hosp Infant Mex 1984;41:682-4.