U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Elliptocytosis 1(EL1)

MedGen UID:
394841
Concept ID:
C2678497
Disease or Syndrome
Synonyms: 4.1- TRAIT; 4.1-MINUS TRAIT; EL1; ELLIPTOCYTOSIS, RHESUS-LINKED TYPE; PROTEIN 4.1 OF ERYTHROCYTE MEMBRANE, DEFECT OF
 
Gene (location): EPB41 (1p35.3)
 
Monarch Initiative: MONDO:0012731
OMIM®: 611804

Definition

Elliptocytosis is a hematologic disorder characterized by elliptically shaped erythrocytes and a variable degree of hemolytic anemia. Usually inherited as an autosomal dominant trait, elliptocytosis results from mutation in any one of several genes encoding proteins of the red cell membrane skeleton (summary by McGuire et al., 1988). Genetic Heterogeneity of Elliptocytosis Elliptocytosis-2 (130600) is caused by mutation in the SPTA1 gene (182860). Elliptocytosis-3 (617948) is caused by mutation in the SPTB gene (182870). Elliptocytosis-4 (166900), also known as Southeast Asian ovalocytosis, is caused by mutation in the SLC4A1 gene (109270). Also see pyropoikilocytosis (266140). See Delaunay (2007) for a discussion of the molecular basis of hereditary red cell membrane disorders. [from OMIM]

Clinical features

From HPO
Jaundice
MedGen UID:
43987
Concept ID:
C0022346
Sign or Symptom
Yellow pigmentation of the skin due to bilirubin, which in turn is the result of increased bilirubin concentration in the bloodstream.
Hemolytic anemia
MedGen UID:
1916
Concept ID:
C0002878
Disease or Syndrome
A type of anemia caused by premature destruction of red blood cells (hemolysis).
Elliptocytosis
MedGen UID:
98107
Concept ID:
C0427480
Finding
The presence of elliptical, cigar-shaped erythrocytes on peripheral blood smear.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Abnormal increased size of the spleen.
Pallor
MedGen UID:
69133
Concept ID:
C0241137
Finding
Abnormally pale skin.

Professional guidelines

PubMed

Risoluti R , Caprari P , Gullifa G , Sorrentino F , Maffei L , Massimi S , Carcassi E , Materazzi S
Chem Commun (Camb) 2020 Jul 14;56(55):7557-7560. Epub 2020 Jun 18 doi: 10.1039/d0cc02948c. PMID: 32555869
Niss O, Chonat S, Dagaonkar N, Almansoori MO, Kerr K, Rogers ZR, McGann PT, Quarmyne MO, Risinger M, Zhang K, Kalfa TA
Blood Cells Mol Dis 2016 Oct;61:4-9. Epub 2016 Jul 17 doi: 10.1016/j.bcmd.2016.07.003. PMID: 27667160Free PMC Article
Christensen RD, Agarwal AM, Nussenzveig RH, Heikal N, Liew MA, Yaish HM
J Perinatol 2015 May;35(5):357-61. Epub 2014 Nov 6 doi: 10.1038/jp.2014.202. PMID: 25357094

Recent clinical studies

Etiology

Fitzgerald PG, Langer JC, Cameron BH, Park AE, Marcaccio MJ, Walton JM, Skinner MA
Surg Endosc 1996 Aug;10(8):859-61. doi: 10.1007/BF00189553. PMID: 8694957

Diagnosis

Amann C, Geipel A, Müller A, Heep A, Ritgen J, Stressig R, Kozlowski P, Gembruch U, Berg C
Ultraschall Med 2011 Dec;32 Suppl 2:E134-40. Epub 2011 Dec 9 doi: 10.1055/s-0031-1281756. PMID: 22161617

Therapy

Fitzgerald PG, Langer JC, Cameron BH, Park AE, Marcaccio MJ, Walton JM, Skinner MA
Surg Endosc 1996 Aug;10(8):859-61. doi: 10.1007/BF00189553. PMID: 8694957

Prognosis

Amann C, Geipel A, Müller A, Heep A, Ritgen J, Stressig R, Kozlowski P, Gembruch U, Berg C
Ultraschall Med 2011 Dec;32 Suppl 2:E134-40. Epub 2011 Dec 9 doi: 10.1055/s-0031-1281756. PMID: 22161617

Clinical prediction guides

Amann C, Geipel A, Müller A, Heep A, Ritgen J, Stressig R, Kozlowski P, Gembruch U, Berg C
Ultraschall Med 2011 Dec;32 Suppl 2:E134-40. Epub 2011 Dec 9 doi: 10.1055/s-0031-1281756. PMID: 22161617
Avraham S, London R, Tulloch GA, Ellis M, Fu Y, Jiang S, White RA, Painter C, Steinberger AA, Avraham H
Gene 1997 Dec 19;204(1-2):5-16. doi: 10.1016/s0378-1119(97)00420-4. PMID: 9434160

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...