Front. Physiol. Frontiers in Physiology Front. Physiol. 1664-042X Frontiers Media S.A. 10.3389/fphys.2019.01331 Physiology Correction Corrigendum: The Spectrum of SPTA1-Associated Hereditary Spherocytosis Chonat Satheesh 1 2 Risinger Mary 3 Sakthivel Haripriya 4 Niss Omar 4 5 Rothman Jennifer A. 6 Hsieh Loan 7 Chou Stella T. 8 9 Kwiatkowski Janet L. 8 9 Khandros Eugene 8 9 Gorman Matthew F. 10 Wells Donald T. 11 Maghathe Tamara 4 Dagaonkar Neha 12 Seu Katie G. 4 Zhang Kejian 13 Zhang Wenying 5 14 Kalfa Theodosia A. 4 5 * 1Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, United States 2Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Atlanta, GA, United States 3College of Nursing, University of Cincinnati, Cincinnati, OH, United States 4Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States 5Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States 6Duke University Medical Center, Durham, NC, United States 7Division of Hematology, CHOC Children's Hospital and UC Irvine Medical Center, Orange, CA, United States 8Division of Hematology, Children's Hospital of Philadelphia, Philadelphia, PA, United States 9Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States 10Kaiser Permanente Santa Clara Medical Center, Santa Clara, CA, United States 11Dell Children's Medical Center, Austin, TX, United States 12Genomics Analysis Facility, Institute for Genomic Medicine, Columbia University, New York, NY, United States 13Coyote Bioscience Co., Ltd., San Jose, CA, United States 14Laboratory of Genetics and Genomics, Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States

Edited by: Paola Bianchi, IRCCS Ca 'Granda Foundation Maggiore Policlinico Hospital, Italy

Reviewed by: Elisa Fermo, IRCCS Ca 'Granda Foundation Maggiore Policlinico Hospital, Italy

 *Correspondence: Theodosia A. Kalfa theodosia.kalfa@cchmc.org

This article was submitted to Red Blood Cell Physiology, a section of the journal Frontiers in Physiology

 18 10 2019 2019 10 1331 03 09 2019 04 10 2019 Copyright © 2019 Chonat, Risinger, Sakthivel, Niss, Rothman, Hsieh, Chou, Kwiatkowski, Khandros, Gorman, Wells, Maghathe, Dagaonkar, Seu, Zhang, Zhang and Kalfa. 2019 Chonat, Risinger, Sakthivel, Niss, Rothman, Hsieh, Chou, Kwiatkowski, Khandros, Gorman, Wells, Maghathe, Dagaonkar, Seu, Zhang, Zhang and Kalfa

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 A Corrigendum on The Spectrum of SPTA1-Associated Hereditary Spherocytosis by Chonat, S., Risinger, M., Sakthivel, H., Niss, O., Rothman, J. A., Hsieh, L., et al. (2019). Front. Physiol. 10:815. doi: 10.3389/fphys.2019.00815 SPTA1 α-spectrin αLEPRA hereditary spherocytosis next generation sequencing hemolytic anemia hydrops fetalis

In the original article, there was a mistake in Table 1 as published. The SPTA1 mutation of Allele 2 in Patient 1, is stated as “c.4294T>A (p.L1432*).” The correct mutation should read “c.4295del (p.L1432*).” The corrected Table 1 appears below.

Genetic mutations and associated phenotype in HS due to SPTA1 mutations.

Phenotype Patient Allele 1 Allele 2 Age at time of report and comments Ektacytometry α-spectrin in RBC ghosts (% of control)
GROUP I (patients 1–4) Severe, recessive HS (transfusion-dependent, responding to splenectomy) 1 c.4339-99C > T c.4295del (p.L1432*) 11 year-old, chronic transfusion requirement with partial response to partial splenectomy, resolved after total splenectomy 54%
2 c.4339-99C > T c.5102A > T (p.L1701*) 7 year-old, chronic transfusion requirement, improved with partial splenectomy 64%
3 c.4339-99C > T c.3267A > T (p.Y1089*) 11 year-old, not splenectomized due to family preference, continues to require frequent transfusions Not evaluable in a transfused sample
4 Mutation not identified Gross deletion of SPTA1 3.5 year-old, RT-PCR demonstrated significantly decreased α-spectrin expression; hemoglobin has normalized after recent splenectomy Not evaluable in a transfused sample
GROUP II (patients 5–8) Severe to moderately severe, recessive HS 5 c.4339-99C > T c.1120C > T (p.R374*) 4 year-old, chronic transfusion requirement for first three years with improved pattern since. Sample not provided after age 3, when transfusion-independent
6 c.4339-99C > T c.1351-1G > T 7 year-old, occasional transfusion requirement, resolved after splenectomy at 5 years of age 59%
7 c.4339-99C>T c.2671C > T (p.R891*) 4 year-old, has not been transfused so far, Hgb 7.1-8.9 g/dL, ARC 420-572 x 103/μl. 61%
8 c.4339-99C > T c.3257delT 8 year-old, transfused once as neonate, Hgb 10.6–11.8 g/dL, ARC 354–535 x 103/μl; now Hgb 15–16 g/dL with normal ARC after splenectomy at 6 years of age (splenectomy performed because of chronic abdominal pain due to co-morbidities) Not performed.
GROUP III (patients 9-11) Life-threatening anemia in utero leading to fatal hydrops fetalis if untreated (transfusion-dependent, not responding to splenectomy) 9 c.4206delG (fs) c.4180delT (fs) in haplotype with c.6631C > T (p.R2211C) Died at birth. Post-mortem diagnosis from parental studies and DNA extracted from liver tissue saved in paraffin block N/A
10 c.6788+11C > T c.6788+11C > T 11 year-old, born prematurely at EGA of 33 weeks with hydrops fetalis, remained transfusion-dependent even after splenectomy; now doing well after matched sibling transplant Not evaluable in a transfused sample (required chronic transfusions up until bone marrow transplant) 26% (performed in CD71+ cells)
11 c.6154del (p.Ala2052fs) c.6154del (p.Ala2052fs) 2 year-old, severe in-utero anemia requiring five in-utero transfusions. Born with severe neonatal hyperbilirubinemia requiring exchange transfusion. Remains transfusion-dependent Not evaluable in a transfused sample

Of note, all the SPTA1 variants reported here except c.4339-99C > T (αLEPRA) and c.2671C > T; p.R891

(Bogardus et al., 2014) have not been previously described.

The authors apologize for this error and state that this does not change the scientific conclusions of the article in any way. The original article has been updated.

References Bogardus H. Schulz V. P. Maksimova Y. Miller B. A. Li P. Forget B. G. . (2014). Severe nondominant hereditary spherocytosis due to uniparental isodisomy at the SPTA1 locus. Haematologica 99, e168–e170. 10.3324/haematol.2014.11031224895341