J Hematol

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

Journal website http://www.thejh.org

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
Date	Presenting symptoms	Treatment
Note the evolution from primarily abdominal and genitourinary symptoms, then to neuropsychiatric symptoms, and back again to abdominal and other physical manifestations.
2005 - 2008	Lethargy, abdominal pain, ileus, frequent UTIs, red urine, poor school performance	Sought 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 2009	Recurrent attacks of abdominal pain, nausea, red urine	Dextrose 5% in water
June 2009	Abdominal pain; diffuse erythematous, pruritic, urticarial lesions	Hematin 4 mg/kg × 2 doses
December 2009	Admitted to the hospital for severe abdominal pain, dysuria, red urine	Hematin 4 mg/kg × 2 doses; Ciprofloxacin
February 2011	Admitted 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 2011	1 - 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 fed	Hematin 4 mg/kg × 1 dose
December 2011	Admitted for generalized weakness, malaise, dizziness, headache, shortness of breath, and confusion while 7 weeks pregnant	Hematin 4 mg/kg × 3 doses
June - July 2012	Word-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 2012	Continued confusion as above; pruritic, urticarial lesions; arm pain	Hematin 4 mg/kg 5 days per week × 3 weeks, resulting in total clearing of her cognitive symptoms
November 2012	Confusion, difficulty concentrating, abdominal pain, paresthesias in upper extremities	Hematin 4 mg/kg × 7 doses
December 2012 - January 2014	Confusion, difficulty concentrating, paresthesias in upper extremities	Hematin 4 mg/kg twice weekly, then once weekly beginning in May 2013, then tapered to PRN-basis
March 2014	Fatigue, ileus, nausea, vomiting	Hematin 4 mg/kg × 1 dose
June 2014	Fatigue, myalgias while 26 weeks pregnant	Hematin 4 mg/kg × 2 doses
September 2014	Fatigue, myalgias, arthralgias, headache	Hematin 4 mg/kg × 1 dose
January 2015	Abdominal pain, right arm pain	Hematin 4 mg/kg × 2 doses
March 2015	Abdominal pain, dysuria, generalized numbness and tingling	Hematin 4 mg/kg × 1 dose

Table 1. Summary of the Course of the Patient’s Symptoms Since Her Presentation to Our Clinic

Date

Presenting symptoms

Treatment

Note the evolution from primarily abdominal and genitourinary symptoms, then to neuropsychiatric symptoms, and back again to abdominal and other physical manifestations.

2005 - 2008

Lethargy, abdominal pain, ileus, frequent UTIs, red urine, poor school performance

Sought 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 2009

Recurrent attacks of abdominal pain, nausea, red urine

Dextrose 5% in water

June 2009

Abdominal pain; diffuse erythematous, pruritic, urticarial lesions

Hematin 4 mg/kg × 2 doses

December 2009

Admitted to the hospital for severe abdominal pain, dysuria, red urine

Hematin 4 mg/kg × 2 doses; Ciprofloxacin

February 2011

Admitted 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 2011

1 - 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 fed

Hematin 4 mg/kg × 1 dose

December 2011

Admitted for generalized weakness, malaise, dizziness, headache, shortness of breath, and confusion while 7 weeks pregnant

Hematin 4 mg/kg × 3 doses

June - July 2012

Word-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 2012

Continued confusion as above; pruritic, urticarial lesions; arm pain

Hematin 4 mg/kg 5 days per week × 3 weeks, resulting in total clearing of her cognitive symptoms

November 2012

Confusion, difficulty concentrating, abdominal pain, paresthesias in upper extremities

Hematin 4 mg/kg × 7 doses

December 2012 - January 2014

Confusion, difficulty concentrating, paresthesias in upper extremities

Hematin 4 mg/kg twice weekly, then once weekly beginning in May 2013, then tapered to PRN-basis

March 2014

Fatigue, ileus, nausea, vomiting

Hematin 4 mg/kg × 1 dose

June 2014

Fatigue, myalgias while 26 weeks pregnant

Hematin 4 mg/kg × 2 doses

September 2014

Fatigue, myalgias, arthralgias, headache

Hematin 4 mg/kg × 1 dose

January 2015

Abdominal pain, right arm pain

Hematin 4 mg/kg × 2 doses

March 2015

Abdominal pain, dysuria, generalized numbness and tingling

Hematin 4 mg/kg × 1 dose