eMedicine Specialties > Hematology > Heme Synthesis and Disorders

Porphyria, Hereditary Coproporphyria: Treatment & Medication

Author: Thomas G DeLoughery, MD, Professor of Medicine and Pathology, Divisions of Hematology/Oncology and Laboratory Medicine, Associate Director, Department of Transfusion Medicine, Division of Clinical Pathology, Oregon Health Sciences University
Contributor Information and Disclosures

Updated: Nov 4, 2009

Treatment

Medical Care

The goals in managing an acute attack of porphyria are to decrease heme synthesis and to reduce the production of porphyrin precursors.6

  • Glucose
    • High doses of glucose (400 g/d) can inhibit heme synthesis and are useful for the treatment of mild attacks.
    • Treat severe attacks, especially those with severe neurologic symptoms, with hematin at a dose of 4 mg/kg/d for 4 days.
  • Pain control: This is best achieved with narcotics. Administer laxatives and stool softeners with the narcotics to avert exacerbating the patient's constipation.
  • Seizure control: Treat with Neurontin; most of the classic antiseizure medications are contraindicated in acute attacks of porphyria.
  • Other: Unlike porphyria cutanea tarda, the skin disease in coproporphyria does not respond to phlebotomy or antimalarial drugs.

Diet

Patients should receive a high-carbohydrate diet during the attack. Administer intravenous glucose if patients cannot eat. Between attacks, patients should eat a constant balanced diet rather than one that is extremely rich in glucose.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Blood products

The key treatment of porphyria is stopping heme synthesis. Hematin provides negative feedback to the heme synthetic pathway and shuts down productions of porphyrins and porphyrin precursors.


Panhematin (Hemin)

DOC for severe porphyria attacks. Enzyme inhibitor derived from processed red blood cells and an iron-containing metalloporphyrin. Was previously known as hematin, a term used to describe the chemical reaction product of hemin and sodium carbonate solution.

Adult

4 mg/kg/d IV for 4 d; for severe attacks can administer 4 mg/kg q12h until symptoms abate; 1-4 mg/kg/d IV over 10-15 min for 3-14 d, based on clinical signs; in severe cases, may repeat no earlier than q12h; not to exceed 6 mg/kg/24h

Pediatric

Not established

May further increase effect of anticoagulants

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Attacks of porphyria may progress to irreversible neuronal damage; may prevent an attack from causing neuronal degeneration; not effective in repairing neuronal damage; asymptomatic and reversible renal shutdown, oliguria, and increased nitrogen retention have occurred; no worsening of renal function has been observed with recommended dosages

More on Porphyria, Hereditary Coproporphyria

Overview: Porphyria, Hereditary Coproporphyria
Differential Diagnoses & Workup: Porphyria, Hereditary Coproporphyria
Treatment & Medication: Porphyria, Hereditary Coproporphyria
Follow-up: Porphyria, Hereditary Coproporphyria
References
Further Reading
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References

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  2. Ventura P, Cappellini MD, Rocchi E. The acute porphyrias: a diagnostic and therapeutic challenge in internal and emergency medicine. Intern Emerg Med. Aug 2009;4(4):297-308. [Medline].

  3. Billoo AG, Lone SW. A family with acute intermittent porphyria. J Coll Physicians Surg Pak. May 2008;18(5):316-8. [Medline].

  4. Corrigall AV, Campbell JA, Siziba K, Kirsch RE, Meissner PN. The expression of protoporphyrinogen oxidase in human tissues. Cell Mol Biol (Noisy-le-grand). Jul 1 2009;55(2):89-95. [Medline].

  5. Roshal M, Turgeon J, Rainey PM. Rapid quantitative method using spin columns to measure porphobilinogen in urine. Clin Chem. Feb 2008;54(2):429-31. [Medline].

  6. Harper P, Wahlin S. Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria. Curr Treat Options Gastroenterol. Dec 2007;10(6):444-55. [Medline].

  7. Anderson KE. The Porphyrias. In: Zakim D, Boyer TD, eds. Hepatology: A Textbook of Liver Disease. Philadelphia, Pa:. WB Saunders;1996:417-463.

  8. Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med. Mar 15 2005;142(6):439-50. [Medline].

  9. Bickers DR, Pathak MA, Lim HW. The Porphyrias. In: Fitzpatrick B, et al, eds. Dermatology in General Medicine. New York, NY:. McGraw-Hill, Inc;1993:1854-1893.

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Keywords

hereditary coproporphyria, porphyria, heme, porphyrin, acute porphyria, porphyrias, acute intermittent porphyria, cutaneous porphyria, porphyria tarda

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Contributor Information and Disclosures

Author

Thomas G DeLoughery, MD, Professor of Medicine and Pathology, Divisions of Hematology/Oncology and Laboratory Medicine, Associate Director, Department of Transfusion Medicine, Division of Clinical Pathology, Oregon Health Sciences University
Thomas G DeLoughery, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American College of Physicians, American Society of Hematology, International Society on Thrombosis and Haemostasis, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Clarence Sarkodee-Adoo, MD, Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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