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Cryohydrocytosis(CHC)

MedGen UID:
396137
Concept ID:
C1861453
Disease or Syndrome
Synonyms: CRYOHYDROCYTOSIS DUE TO BAND 3 BLACKBURN; CRYOHYDROCYTOSIS DUE TO BAND 3 HEMEL; CRYOHYDROCYTOSIS DUE TO BAND 3 HURSTPIERPOINT; Hereditary cryohydrocytosis with normal stomatin; Pseudohyperkalemia Cardiff; Stomatocytosis, cold-sensitive
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): SLC4A1 (17q21.31)
 
Monarch Initiative: MONDO:0008494
OMIM®: 185020
Orphanet: ORPHA398088

Definition

Cryohydrocytosis (CHC) is an exceedingly rare condition characterized by a mild stomatocytic hemolytic state with hyperbilirubinemia. A hallmark of this condition is that red blood cells (RBCs) lyse on storage at 4 degrees centigrade. RBC cation permeability is increased at 37 degrees centigrade, and the cells also accumulate sodium in the cold (summary by Coles et al., 1999). Patients present with fatigue, mild anemia, and pseudohyperkalemia due to a potassium leak from the RBCs (summary by Bogdanova et al., 2010). For a discussion of clinical and genetic heterogeneity of the hereditary stomatocytoses, see 194380. [from OMIM]

Clinical features

From HPO
Hemolytic anemia
MedGen UID:
1916
Concept ID:
C0002878
Disease or Syndrome
A type of anemia caused by premature destruction of red blood cells (hemolysis).
Reticulocytosis
MedGen UID:
60089
Concept ID:
C0206160
Finding
An elevation in the number of reticulocytes (immature erythrocytes) in the peripheral blood circulation.
Stomatocytosis
MedGen UID:
760280
Concept ID:
C0677598
Laboratory or Test Result
The presence of erythrocytes with a mouth-shaped (stoma) area of central pallor on peripheral blood smear.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Abnormal increased size of the spleen.
Pseudohyperkalemia
MedGen UID:
905233
Concept ID:
C0948309
Finding
Pseudohyperkalemia describes a falsely elevated potassium level upon measurement of the serum electrolyte concentration due to disruption of cells during the collection or processing of the sample.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCryohydrocytosis

Recent clinical studies

Etiology

Furia A, Muccioli L, Santucci M, Licchetta L, Bisulli F
Epileptic Disord 2023 Jun;25(3):410-415. Epub 2023 Apr 27 doi: 10.1002/epd2.20039. PMID: 36938875
Iolascon A, Andolfo I, Russo R
Br J Haematol 2019 Oct;187(1):13-24. Epub 2019 Jul 31 doi: 10.1111/bjh.16126. PMID: 31364155
Guizouarn H, Borgese F, Gabillat N, Harrison P, Goede JS, McMahon C, Stewart GW, Bruce LJ
Br J Haematol 2011 Mar;152(5):655-64. Epub 2011 Jan 23 doi: 10.1111/j.1365-2141.2010.08454.x. PMID: 21255002
Bruce LJ
Blood Cells Mol Dis 2009 May-Jun;42(3):216-22. Epub 2009 Mar 3 doi: 10.1016/j.bcmd.2009.01.014. PMID: 19261491
Jarvis HG, Gore DM, Briggs C, Chetty MC, Stewart GW
Br J Haematol 2003 Sep;122(5):859-68. doi: 10.1046/j.1365-2141.2003.04487.x. PMID: 12930402

Diagnosis

Furia A, Muccioli L, Santucci M, Licchetta L, Bisulli F
Epileptic Disord 2023 Jun;25(3):410-415. Epub 2023 Apr 27 doi: 10.1002/epd2.20039. PMID: 36938875
Iolascon A, Andolfo I, Russo R
Br J Haematol 2019 Oct;187(1):13-24. Epub 2019 Jul 31 doi: 10.1111/bjh.16126. PMID: 31364155
Klepper J
Handb Clin Neurol 2013;113:1689-94. doi: 10.1016/B978-0-444-59565-2.00036-8. PMID: 23622389
Delaunay J
Semin Hematol 2004 Apr;41(2):165-72. doi: 10.1053/j.seminhematol.2004.02.005. PMID: 15071792
Haines PG, Jarvis HG, King S, Noormohamed FH, Chetty MC, Fisher J, Hill P, Nicolaou A, Stewart GW
Br J Haematol 2001 Jun;113(4):932-7. doi: 10.1046/j.1365-2141.2001.02792.x. PMID: 11442486

Therapy

Bruce LJ
Blood Cells Mol Dis 2009 May-Jun;42(3):216-22. Epub 2009 Mar 3 doi: 10.1016/j.bcmd.2009.01.014. PMID: 19261491

Prognosis

Mahesh B, Besser M, Ravaglioli A, Pepke-Zaba J, Martinez G, Klein A, Ng C, Tsui S, Dunning J, Jenkins DP
Eur J Cardiothorac Surg 2016 Sep;50(3):537-41. Epub 2016 Mar 17 doi: 10.1093/ejcts/ezw062. PMID: 26987879
King MJ, Behrens J, Rogers C, Flynn C, Greenwood D, Chambers K
Br J Haematol 2000 Dec;111(3):924-33. PMID: 11122157

Clinical prediction guides

Bogdanova A, Goede JS, Weiss E, Bogdanov N, Bennekou P, Bernhardt I, Lutz HU
Haematologica 2010 Feb;95(2):189-98. Epub 2009 Dec 16 doi: 10.3324/haematol.2009.010215. PMID: 20015879Free PMC Article
Iolascon A, De Falco L, Borgese F, Esposito MR, Avvisati RA, Izzo P, Piscopo C, Guizouarn H, Biondani A, Pantaleo A, De Franceschi L
Haematologica 2009 Aug;94(8):1049-59. doi: 10.3324/haematol.2008.002873. PMID: 19644137Free PMC Article
King MJ, Behrens J, Rogers C, Flynn C, Greenwood D, Chambers K
Br J Haematol 2000 Dec;111(3):924-33. PMID: 11122157

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