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Article ; Online: How I treat erythropoietic protoporphyria and X-linked protoporphyria.

Leaf, Rebecca Karp / Dickey, Amy K

2023 Volume 141, Issue 24, Page(s) 2921–2931

Abstract: Erythropoietic protoporphyria (EPP) is an inherited cutaneous porphyria caused by reduced expression of ferrochelatase, the enzyme that catalyzes the final step in heme biosynthesis. The resultant accumulation of protoporphyrin IX leads to severe, ... ...

Abstract	Erythropoietic protoporphyria (EPP) is an inherited cutaneous porphyria caused by reduced expression of ferrochelatase, the enzyme that catalyzes the final step in heme biosynthesis. The resultant accumulation of protoporphyrin IX leads to severe, painful cutaneous photosensitivity, as well as potentially life-threatening liver disease in a small percentage of patients. X-linked protoporphyria (XLP) is clinically similar to EPP but results from increased activity of δ-aminolevulinic acid synthase 2, the first step in heme biosynthesis in the bone marrow, and also causes protoporphyrin accumulation. Although historically the management of EPP and XLP (collectively termed protoporphyria) centered around avoidance of sunlight, novel therapies have recently been approved or are in development, which will alter the therapeutic landscape for these conditions. We present 3 patient cases, highlighting key treatment considerations in patients with protoporphyria, including (1) approach to photosensitivity, (2) managing iron deficiency in protoporphyria, and (3) understanding hepatic failure in protoporphyria.
MeSH term(s)	Humans ; Protoporphyria, Erythropoietic/therapy ; Protoporphyria, Erythropoietic/complications ; Ferrochelatase/genetics ; Ferrochelatase/metabolism ; Liver Diseases ; Photosensitivity Disorders/etiology ; Photosensitivity Disorders/therapy ; Protoporphyrins ; Heme/metabolism
Chemical Substances	Ferrochelatase (EC 4.99.1.1) ; Protoporphyrins ; Heme (42VZT0U6YR)
Language	English
Publishing date	2023-03-09
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood.2022018688
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Article ; Online: Update on the Porphyrias.

Dickey, Amy K / Leaf, Rebecca Karp / Balwani, Manisha

Annual review of medicine

2023 Volume 75, Page(s) 321–335

Abstract: The porphyrias are a group of rare diseases, each resulting from a defect in a different enzymatic step of the heme biosynthetic pathway. They can be broadly divided into two categories, hepatic and erythropoietic porphyrias, depending on the primary ... ...

Abstract	The porphyrias are a group of rare diseases, each resulting from a defect in a different enzymatic step of the heme biosynthetic pathway. They can be broadly divided into two categories, hepatic and erythropoietic porphyrias, depending on the primary site of accumulation of heme intermediates. These disorders are multisystemic with variable symptoms that can be encountered by physicians in any specialty. Here, we review the porphyrias and describe their clinical presentation, diagnosis, and management. We discuss novel therapies that are approved or in development. Early diagnosis is key for the appropriate management and prevention of long-term complications in these rare disorders.
MeSH term(s)	Humans ; Porphyrias/diagnosis ; Porphyrias/genetics ; Porphyrias/therapy ; Heme
Chemical Substances	Heme (42VZT0U6YR)
Language	English
Publishing date	2023-08-04
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	207930-6
ISSN	1545-326X ; 0066-4219
ISSN (online)	1545-326X
ISSN	0066-4219
DOI	10.1146/annurev-med-042921-123602
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Article ; Online: Case 11-2022: An 80-Year-Old Woman with Pancytopenia.

Leaf, Rebecca K / Dhami, Ranjodh S / Chun, Nancy S / Ta, Robert

The New England journal of medicine

2022 Volume 386, Issue 15, Page(s) 1453–1461

MeSH term(s)	Aged, 80 and over ; Female ; Humans ; Pancytopenia/etiology
Language	English
Publishing date	2022-04-13
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	207154-x
ISSN	1533-4406 ; 0028-4793
ISSN (online)	1533-4406
ISSN	0028-4793
DOI	10.1056/NEJMcpc2201232
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Article: Risk Factors for Death or Cardiovascular Events after Acute Coronary Syndrome in Patients with Myeloproliferative Neoplasms.

Leiva, Orly / Jenkins, Andrew / Rosovsky, Rachel P / Leaf, Rebecca K / Goodarzi, Katayoon / Hobbs, Gabriela

Hematology reports

2023 Volume 15, Issue 2, Page(s) 398–404

Abstract: ... 3.84, 95% CI 1.44-10.19), WBC ≥ 20 K/µL (HR 9.10, 95% CI 2.71-30.52), ...

Abstract	Patients with myeloproliferative neoplasms (MPNs) are at increased risk of cardiovascular disease (CVD), including acute coronary syndrome (ACS). However, data on long-term outcomes of patients with MPN who have had ACS and risk factors for all-cause death or CV events post-ACS hospitalization are lacking. We conducted a single-center study of 41 consecutive patients with MPN with ACS hospitalization after MPN diagnosis. After a median follow-up of 80 months after ACS hospitalization, 31 (76%) experienced death or a CV event (myocardial infarction, ischemic stroke, or heart failure hospitalization). After multivariable Cox proportional hazards regression, index ACS within 12 months of MPN diagnosis (HR 3.84, 95% CI 1.44-10.19), WBC ≥ 20 K/µL (HR 9.10, 95% CI 2.71-30.52),
Language	English
Publishing date	2023-06-07
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2586645-X
ISSN	2038-8330 ; 2038-8322
ISSN (online)	2038-8330
ISSN	2038-8322
DOI	10.3390/hematolrep15020040
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Article: Chemoprophylaxis and Management of Venous Thromboembolism in Microvascular Surgery.

Mirzamohammadi, Fatemeh / Nnamani Silva, Ogonna N / Leaf, Rebecca K / Eberlin, Kyle R / Valerio, Ian L

Seminars in plastic surgery

2023 Volume 37, Issue 1, Page(s) 57–72

Abstract: This review aims to highlight the common pharmacological and nonpharmacological interventions utilized for thromboprophylaxis as well as flap salvage in microsurgery. A literature review was conducted in PubMed/National Center for Biotechnology ... ...

Abstract	This review aims to highlight the common pharmacological and nonpharmacological interventions utilized for thromboprophylaxis as well as flap salvage in microsurgery. A literature review was conducted in PubMed/National Center for Biotechnology Information, Scopus, Web of Science, and MEDLINE databases. Articles with a focus on thromboprophylaxis in microsurgical procedures spanning head and neck surgery, breast and extremity microvascular reconstruction, deep venous thrombosis/pulmonary embolus in microvascular surgery, and flap thrombosis and salvage were included in this review. The majority of available evidence supports mechanical venous thromboembolism (VTE) prophylaxis in all patients undergoing microsurgery given the presence of multiple risk factors for VTE within this particular patient population. Based on the literature review, addition of VTE chemoprophylactic agents is beneficial and an algorithmic approach to thromboprophylaxis in microsurgery patients and management of patients with thrombosis based on literature review and senior authors' experience is recommended and outlined.
Language	English
Publishing date	2023-02-09
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2077828-4
ISSN	1535-2188
ISSN	1535-2188
DOI	10.1055/s-0042-1760381
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Article ; Online: ABO phenotype and death in critically ill patients with COVID-19.

Leaf, Rebecca K / Al-Samkari, Hanny / Brenner, Samantha K / Gupta, Shruti / Leaf, David E

British journal of haematology

2020 Volume 190, Issue 4, Page(s) e204–e208

MeSH term(s)	ABO Blood-Group System/blood ; Aged ; COVID-19/blood ; COVID-19/mortality ; Critical Illness ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; SARS-CoV-2
Chemical Substances	ABO Blood-Group System
Keywords	covid19
Language	English
Publishing date	2020-07-30
Publishing country	England
Document type	Clinical Trial ; Letter ; Multicenter Study ; Research Support, N.I.H., Extramural
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1111/bjh.16984
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Article ; Online: Avatrombopag for adults with early versus chronic immune thrombocytopenia.

Virk, Zain M / Leaf, Rebecca K / Kuter, David J / Goodarzi, Katayoon / Connell, Nathan T / Connors, Jean M / Al-Samkari, Hanny

American journal of hematology

2023 Volume 99, Issue 2, Page(s) 155–162

Abstract: Avatrombopag is a newer thrombopoietin receptor agonist (TPO-RA) currently approved to treat chronic ITP (duration >12 months). No studies have yet evaluated the safety and effectiveness of avatrombopag in newly diagnosed ITP (duration <3 months) or ... ...

Abstract	Avatrombopag is a newer thrombopoietin receptor agonist (TPO-RA) currently approved to treat chronic ITP (duration >12 months). No studies have yet evaluated the safety and effectiveness of avatrombopag in newly diagnosed ITP (duration <3 months) or persistent ITP (duration 3-12 months), and so its use in these populations is presently off-label worldwide. We hypothesize that avatrombopag has similar safety and effectiveness irrespective of ITP disease phase. To evaluate this, we performed a multicenter observational cohort study of adults with ITP treated with avatrombopag, comparing patient outcomes by disease phase (newly diagnosed/persistent versus chronic). Seventy-five patients were included, 23 with newly diagnosed/persistent ITP (17.7 patient-years of avatrombopag treatment) and 52 with chronic ITP (65.3 patient-years of avatrombopag treatment). On avatrombopag, 91% of newly diagnosed/persistent patients versus 96% of chronic patients (p = .58) achieved a platelet response (≥50 × 10
MeSH term(s)	Adult ; Humans ; Purpura, Thrombocytopenic, Idiopathic/drug therapy ; Purpura, Thrombocytopenic, Idiopathic/chemically induced ; Platelet Count ; Blood Platelets ; Thiazoles/adverse effects ; Recombinant Fusion Proteins ; Thrombopoietin/adverse effects ; Thiophenes
Chemical Substances	avatrombopag (3H8GSZ4SQL) ; Thiazoles ; Recombinant Fusion Proteins ; Thrombopoietin (9014-42-0) ; Thiophenes
Language	English
Publishing date	2023-12-08
Publishing country	United States
Document type	Observational Study ; Multicenter Study ; Journal Article
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.27080
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Article ; Online: Acute hepatic porphyrias: Recommendations for diagnosis and management with real-world examples.

Moghe, Akshata / Dickey, Amy / Erwin, Angelika / Leaf, Rebecca K / O'Brien, Alan / Quigley, John G / Thapar, Manish / Anderson, Karl E

Molecular genetics and metabolism

2023 Volume 140, Issue 3, Page(s) 107670

Abstract: Acute hepatic porphyria (AHP) is a group of four rare inherited diseases, each resulting from a deficiency in a distinct enzyme in the heme biosynthetic pathway. Characterized by acute neurovisceral symptoms that may mimic other medical and psychiatric ... ...

Abstract	Acute hepatic porphyria (AHP) is a group of four rare inherited diseases, each resulting from a deficiency in a distinct enzyme in the heme biosynthetic pathway. Characterized by acute neurovisceral symptoms that may mimic other medical and psychiatric conditions, lack of recognition of the disease often leads to a delay in diagnosis and initiation of effective treatment. Biochemical testing for pathway intermediates that accumulate when the disease is active forms the basis for screening and establishing a diagnosis. Subsequent genetic analysis identifies the pathogenic variant, supporting screening of family members and genetic counseling. Management of AHP involves avoidance of known exogenous and hormonal triggers, symptomatic treatment, and prevention of recurrent attacks. Here we describe six case studies from our own real-world experience to highlight current recommendations and challenges associated with the diagnosis and long-term management of the disease.
MeSH term(s)	Humans ; Porphobilinogen ; Porphyrias, Hepatic/diagnosis ; Porphyrias, Hepatic/genetics ; Porphyrias, Hepatic/therapy ; Porphobilinogen Synthase ; Heme/genetics
Chemical Substances	Porphobilinogen (74KHC72QXK) ; Porphobilinogen Synthase (EC 4.2.1.24) ; Heme (42VZT0U6YR)
Language	English
Publishing date	2023-07-27
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1418518-0
ISSN	1096-7206 ; 1096-7192
ISSN (online)	1096-7206
ISSN	1096-7192
DOI	10.1016/j.ymgme.2023.107670
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Article ; Online: Case 25-2021: A 48-Year-Old Man with Fatigue and Leg Swelling.

Moore, Amber B / Wing, Jonathan R / Goiffon, Reece J / Leaf, Rebecca K / Tsao, Lana / Misdraji, Joseph

The New England journal of medicine

2021 Volume 385, Issue 8, Page(s) 745–754

MeSH term(s)	Cardiomyopathies/etiology ; Diabetes Mellitus/etiology ; Diagnosis, Differential ; Edema/etiology ; Fatal Outcome ; Fatigue/etiology ; Hemochromatosis/complications ; Hemochromatosis/diagnosis ; Hemochromatosis/drug therapy ; Hemochromatosis/genetics ; Humans ; Iron/blood ; Iron/metabolism ; Iron Chelating Agents/therapeutic use ; Leg ; Male ; Middle Aged ; Transferrin/metabolism
Chemical Substances	Iron Chelating Agents ; Transferrin ; Iron (E1UOL152H7)
Language	English
Publishing date	2021-08-18
Publishing country	United States
Document type	Case Reports ; Clinical Conference ; Journal Article
ZDB-ID	207154-x
ISSN	1533-4406 ; 0028-4793
ISSN (online)	1533-4406
ISSN	0028-4793
DOI	10.1056/NEJMcpc2100282
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Article ; Online: Observational pilot study of multi-wavelength wearable light dosimetry for erythropoietic protoporphyria.

Dickey, Amy K / Berkovich, Jaime / Leaf, Rebecca K / Jiang, Paul Y / Lopez-Galmiche, Gisela / Rebeiz, Lina / Wheeden, Kristen / Kochevar, Irene / Savage, William / Zhao, Sophia / Campisi, Elizabeth / Heo, Seung Y / Trueb, Jacob / LaRochelle, Ethan P M / Rogers, John / Banks, Anthony / Chang, Jan-Kai

International journal of dermatology

2024

Abstract: Background: Erythropoietic protoporphyria (EPP) causes painful light sensitivity, limiting quality of life. Our objective was to develop and validate a wearable light exposure device and correlate measurements with light sensitivity in EPP to predict ... ...

Abstract	Background: Erythropoietic protoporphyria (EPP) causes painful light sensitivity, limiting quality of life. Our objective was to develop and validate a wearable light exposure device and correlate measurements with light sensitivity in EPP to predict and prevent symptoms. Methods: A wearable light dosimeter was developed to capture light doses of UVA, blue, and red wavelengths. A prospective observational pilot study was performed in which five EPP patients wore two light dosimeters for 3 weeks, one as a watch, and one as a shirt clip. Results: Standard deviation (SD) increases from the mean in the daily blue light dose increased the odds ratio (OR) for symptom risk more than the self-reported outdoor time (OR 2.76 vs. 2.38) or other wavelengths, and a one SD increase from the mean in the daily blue light wristband device dose increased the OR for symptom risk more than the daily blue light shirt clip (OR 2.45 vs. 1.62). The area under the receiver operator curve for the blue light wristband dose was 0.78, suggesting 78% predictive accuracy. Conclusion: These data demonstrate that wearable blue light dosimetry worn as a wristband is a promising method for measuring light exposure and predicting and preventing symptoms in EPP.
Language	English
Publishing date	2024-04-11
Publishing country	England
Document type	Journal Article
ZDB-ID	412254-9
ISSN	1365-4632 ; 0011-9059 ; 1461-1244
ISSN (online)	1365-4632
ISSN	0011-9059 ; 1461-1244
DOI	10.1111/ijd.17166
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