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Harderoporphyria(HARPO)

MedGen UID:
137981
Concept ID:
C0342859
Disease or Syndrome
Synonym: HARPO
SNOMED CT: Homozygous hereditary coproporphyria (238056003); Harderoporphyria (238056003)
 
Gene (location): CPOX (3q11.2)
 
Monarch Initiative: MONDO:0030048
OMIM®: 618892

Definition

Harderoporphyria (HARPO) is a rare erythropoietic variant form of hereditary coproporphyria (HCP; 121300) characterized by neonatal hemolytic anemia, sometimes accompanied by skin lesions, and massive excretion of harderoporphyrin in feces. During childhood and adulthood, a mild residual anemia is chronically observed (review by Schmitt et al., 2005). [from OMIM]

Clinical features

From HPO
Red urine
MedGen UID:
910906
Concept ID:
C0858862
Finding
An abnormal red color of the urine.
Increased urinary porphobilinogen
MedGen UID:
868601
Concept ID:
C4023000
Finding
The concentration of porphobilinogen in the urine, normalized for urine concentration, is above the upper limit of normal.
Increased urine harderoporphyrin level
MedGen UID:
1783842
Concept ID:
C5539729
Finding
Concentration of harderoporphyrin in the urine, normalized for urine concentration, is above the upper limit of normal.
Hepatomegaly
MedGen UID:
42428
Concept ID:
C0019209
Finding
Abnormally increased size of the liver.
Vomiting
MedGen UID:
12124
Concept ID:
C0042963
Sign or Symptom
Forceful ejection of the contents of the stomach through the mouth by means of a series of involuntary spasmic contractions.
Prolonged neonatal jaundice
MedGen UID:
347108
Concept ID:
C1859236
Finding
Neonatal jaundice refers to a yellowing of the skin and other tissues of a newborn infant as a result of increased concentrations of bilirubin in the blood. Neonatal jaundice affects over half of all newborns to some extent in the first week of life. Prolonged neonatal jaundice is said to be present if the jaundice persists for longer than 14 days in term infants and 21 days in preterm infants.
Increased fecal harderoporphyrin
MedGen UID:
1841832
Concept ID:
C5826418
Finding
Abnormally high concentration of harderoporphyrin in feces.
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.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Abnormal increased size of the spleen.
Increased circulating ferritin concentration
MedGen UID:
69130
Concept ID:
C0241013
Finding
Increased concentration of ferritin in the blood circulation.
Neonatal hyperbilirubinemia
MedGen UID:
208991
Concept ID:
C0857007
Disease or Syndrome
A type of hyperbilirubinemia with neonatal onset.
Hyperpigmentation of the skin
MedGen UID:
57992
Concept ID:
C0162834
Pathologic Function
A darkening of the skin related to an increase in melanin production and deposition.
Cutaneous photosensitivity
MedGen UID:
87601
Concept ID:
C0349506
Pathologic Function
An increased sensitivity of the skin to light. Photosensitivity may result in a rash upon exposure to the sun (which is known as photodermatosis). Photosensitivity can be diagnosed by phototests in which light is shone on small areas of skin.

Professional guidelines

PubMed

Lamoril J, Puy H, Whatley SD, Martin C, Woolf JR, Da Silva V, Deybach JC, Elder GH
Am J Hum Genet 2001 May;68(5):1130-8. Epub 2001 Apr 16 doi: 10.1086/320118. PMID: 11309681Free PMC Article

Recent clinical studies

Etiology

Lamoril J, Puy H, Gouya L, Rosipal R, Da Silva V, Grandchamp B, Foint T, Bader-Meunier B, Dommergues JP, Deybach JC, Nordmann Y
Blood 1998 Feb 15;91(4):1453-7. PMID: 9454777

Diagnosis

Hasanoglu A, Balwani M, Kasapkara CS, Ezgü FS, Okur I, Tümer L, Cakmak A, Nazarenko I, Yu C, Clavero S, Bishop DF, Desnick RJ
J Inherit Metab Dis 2011 Feb;34(1):225-31. Epub 2010 Nov 20 doi: 10.1007/s10545-010-9237-9. PMID: 21103937Free PMC Article
Takeuchi H, Kondo M, Daimon M, Susa S, Ueoka K, Uemura O, Togari H
Blood 2001 Dec 15;98(13):3871-3. doi: 10.1182/blood.v98.13.3871. PMID: 11739202
Lamoril J, Martasek P, Deybach JC, Da Silva V, Grandchamp B, Nordmann Y
Hum Mol Genet 1995 Feb;4(2):275-8. doi: 10.1093/hmg/4.2.275. PMID: 7757079
Nordmann Y, Grandchamp B, de Verneuil H, Phung L, Cartigny B, Fontaine G
J Clin Invest 1983 Sep;72(3):1139-49. doi: 10.1172/JCI111039. PMID: 6886003Free PMC Article

Prognosis

Hasanoglu A, Balwani M, Kasapkara CS, Ezgü FS, Okur I, Tümer L, Cakmak A, Nazarenko I, Yu C, Clavero S, Bishop DF, Desnick RJ
J Inherit Metab Dis 2011 Feb;34(1):225-31. Epub 2010 Nov 20 doi: 10.1007/s10545-010-9237-9. PMID: 21103937Free PMC Article
Schmitt C, Gouya L, Malonova E, Lamoril J, Camadro JM, Flamme M, Rose C, Lyoumi S, Da Silva V, Boileau C, Grandchamp B, Beaumont C, Deybach JC, Puy H
Hum Mol Genet 2005 Oct 15;14(20):3089-98. Epub 2005 Sep 13 doi: 10.1093/hmg/ddi342. PMID: 16159891
Lamoril J, Puy H, Whatley SD, Martin C, Woolf JR, Da Silva V, Deybach JC, Elder GH
Am J Hum Genet 2001 May;68(5):1130-8. Epub 2001 Apr 16 doi: 10.1086/320118. PMID: 11309681Free PMC Article

Clinical prediction guides

Hasanoglu A, Balwani M, Kasapkara CS, Ezgü FS, Okur I, Tümer L, Cakmak A, Nazarenko I, Yu C, Clavero S, Bishop DF, Desnick RJ
J Inherit Metab Dis 2011 Feb;34(1):225-31. Epub 2010 Nov 20 doi: 10.1007/s10545-010-9237-9. PMID: 21103937Free PMC Article
Lamoril J, Puy H, Whatley SD, Martin C, Woolf JR, Da Silva V, Deybach JC, Elder GH
Am J Hum Genet 2001 May;68(5):1130-8. Epub 2001 Apr 16 doi: 10.1086/320118. PMID: 11309681Free PMC Article
Lamoril J, Puy H, Gouya L, Rosipal R, Da Silva V, Grandchamp B, Foint T, Bader-Meunier B, Dommergues JP, Deybach JC, Nordmann Y
Blood 1998 Feb 15;91(4):1453-7. PMID: 9454777
Lamoril J, Martasek P, Deybach JC, Da Silva V, Grandchamp B, Nordmann Y
Hum Mol Genet 1995 Feb;4(2):275-8. doi: 10.1093/hmg/4.2.275. PMID: 7757079
Nordmann Y, Grandchamp B, de Verneuil H, Phung L, Cartigny B, Fontaine G
J Clin Invest 1983 Sep;72(3):1139-49. doi: 10.1172/JCI111039. PMID: 6886003Free PMC Article

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