Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc
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Case Report

Volume 6, Number 1, March 2017, pages 21-24

A Unique Neuropsychiatric Syndrome in Variant Hereditary Coproporphyria: Case Report and Review of the Literature


Table 1. Summary of the Course of the Patient’s Symptoms Since Her Presentation to Our Clinic
DatePresenting symptomsTreatment
Note the evolution from primarily abdominal and genitourinary symptoms, then to neuropsychiatric symptoms, and back again to abdominal and other physical manifestations.
2005 - 2008Lethargy, abdominal pain, ileus, frequent UTIs, red urine, poor school performanceSought treatment out of state
May 2008 (age 19)Nausea, dizziness, abdominal pain, red urine, frequent UTIs (first presentation to our clinic)Recommended increased carbohydrate intake, increased exercise
August 2008 - May 2009Recurrent attacks of abdominal pain, nausea, red urineDextrose 5% in water
June 2009Abdominal pain; diffuse erythematous, pruritic, urticarial lesionsHematin 4 mg/kg × 2 doses
December 2009Admitted to the hospital for severe abdominal pain, dysuria, red urineHematin 4 mg/kg × 2 doses; Ciprofloxacin
February 2011Admitted to the hospital for arthralgias, abdominal pain, fever while 18 weeks pregnant (otherwise, symptoms had been very mild during pregnancy)Hematin 4 mg/kg × 2 doses
June 20111 - 2 days after cesarean section delivery of daughter, experienced abdominal pain, headache, and difficulty with concentration and memory, including being unable to recall when her daughter had last fedHematin 4 mg/kg × 1 dose
December 2011Admitted for generalized weakness, malaise, dizziness, headache, shortness of breath, and confusion while 7 weeks pregnantHematin 4 mg/kg × 3 doses
June - July 2012Word-finding difficulties, trouble concentrating, performing tasks out of normal order (e.g., drying dishes before rinsing them), get lost while driving to familiar places, initiating tasks but then forgetting to complete them (e.g., only cleaning half of a surface at home)Hematin 4 mg/kg × 5 doses
October 2012Continued confusion as above; pruritic, urticarial lesions; arm painHematin 4 mg/kg 5 days per week × 3 weeks, resulting in total clearing of her cognitive symptoms
November 2012Confusion, difficulty concentrating, abdominal pain, paresthesias in upper extremitiesHematin 4 mg/kg × 7 doses
December 2012 - January 2014Confusion, difficulty concentrating, paresthesias in upper extremitiesHematin 4 mg/kg twice weekly, then once weekly beginning in May 2013, then tapered to PRN-basis
March 2014Fatigue, ileus, nausea, vomitingHematin 4 mg/kg × 1 dose
June 2014Fatigue, myalgias while 26 weeks pregnantHematin 4 mg/kg × 2 doses
September 2014Fatigue, myalgias, arthralgias, headacheHematin 4 mg/kg × 1 dose
January 2015Abdominal pain, right arm painHematin 4 mg/kg × 2 doses
March 2015Abdominal pain, dysuria, generalized numbness and tinglingHematin 4 mg/kg × 1 dose