Reply to Lissing et al. Comment on “Ramai et al. Risk of Hepatocellular Carcinoma in Patients with Porphyria: A Systematic Review. Cancers 2022, 14, 2947”
by
, , , , , , , , and
Daryl Ramai
1,
Smit S. Deliwala
2,
Saurabh Chandan
3,
Janice Lester
4,
Jameel Singh
5,
Jayanta Samanta
6,
Sara di Nunzio
7,
Fabio Perversi
7,
Francesca Cappellini
7,
Aashni Shah
7,
Michele Ghidini
8,
Rodolfo Sacco
9,
Antonio Facciorusso
9,†
and
Luca Giacomelli
7,*,† 1
Division of Gastroenterology and Hepatology, University of Utah, Salt Lake City, UT 84112, USA
2
Department of Internal Medicine, Hurley Medical Center, Michigan State University, Flint, MI 48503, USA
3
Division of Gastroenterology & Hepatology, CHI Health Creighton University Medical Center, Omaha, NE 68131, USA
4
Health Science Library, Long Island Jewish Medical Center, Northwell Health, New Hyde Park, NY 11040, USA
5
Department of Internal Medicine, Mather Hospital, Northwell Health, Port Jefferson, NY 11777, USA
6
Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, Sector 12, Chandigarh 160012, India
7
Polistudium SRL, 20135 Milan, Italy
8
Division of Medical Oncology, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, 20122 Milan, Italy
9
Gastroenterology Unit, Department of Surgical and Medical Sciences, University of Foggia, 71122 Foggia, Italy
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
Cancers 2023, 15(4), 1187; https://doi.org/10.3390/cancers15041187
Submission received: 13 January 2023
/
Accepted: 30 January 2023
/
Published: 13 February 2023
(This article belongs to the Special Issue Hepatobiliary and Pancreatic Neoplasms: Biomarkers and Targeted Therapy)
We thank Dr. Lissing and colleagues for providing us with these helpful comments [1]. We are appreciative of your expertise and critical review of our work [2]. We have recognized that there were errors and will take the necessary steps to correct them.
After removing the overlapping articles, which in some cases were not very clear to us but following your assertions, we identified 13 articles, of which 3757 patients had porphyria (of any subtype) (Table 1). Overall, from this cohort, we identified 166 patients who developed cancer. We have also clearly laid out different types of porphyria and the number of cancer cases for each of those subtypes as per your recommendations; please see the table below.
While disease severity would be interesting to assess, however, this is difficult to extract from these studies. However, we agree that a large cohort study with this information, including age, would be important for future research efforts.
Again, thank you for your comments.
Author Contributions
Conceptualization, D.R. and A.F.; methodology, D.R. and A.F.; validation, A.F.; data curation, D.R., S.S.D., S.C., J.L., J.S. (Jameel Singh), J.S. (Jayanta Samanta) and A.S.; writing—original draft preparation, D.R. and S.S.D.; writing—review and editing, D.R., S.d.N., M.G., F.P., F.C. and A.S.; supervision, D.R., A.F., R.S. and L.G. All authors have read and agreed to the published version of the manuscript.
Funding
This research received no external funding.
Data Availability Statement
Not applicable.
Conflicts of Interest
The authors declare no conflict of interest.
References
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Table 1.
Characteristics of patients with porphyria.
| Author/Year | Design | Location | Total Patients (n) | Total female n (%) | Age (Years), Mean ± SD | Age at Cancer Diagnosis (Years), Mean ± SD | α-Fetoprotein Levels | Porphyria Subtype with Cancer | Type of Cancer |
|---|---|---|---|---|---|---|---|---|---|
| Solis 1982 [3] | * Single-center | Spain | 138 PCT | 3 (2) | NR | 64 ± 7 | 780 ng/mL (1) 1320 ng/mL (1) 2150 ng/mL (1) Positive (5) ND (2) | 10 PCT | HCC (7) Unknown (3) |
| Salata 1985 [4] | Retrospective, single-center | Spain | 83 PCT | 6 (7.2) | 57 | 60 ± 5 | Elevated in 3 out of 9 HCC cases | 13 PCT | HCC (13) |
| Siersema 1992 [5] | Prospective, single-center | Netherlands | 38 PCT | 13 (34) | 48 ± 12 | 54 ± 4 | None were elevated | 5 PCT | HCC (5) |
| Kauppinen 1992 [6] | Retrospective, single-center | Finland | 206 (184 AIP, 61 VP) | 121 (58.7) | 49 (Range 21–96) | NR | NR | 6 AIP 1 VP | HCC (7) |
| Andant 2000 [7] | Prospective, single-center | France | 650 (430 AIP, 136 VP, 84 HC) | 347 (53) | 41 ± 7 | 50 ± 10 | >200 IU/mL (7) | 5 AIP 1 VP 1 HC | HCC (7) |
| Fracanzani 2001 [8] | Case–control, single-center | Italy | 53 PCT | 2 (3.8) | 56 ± 8 | NR | >400 UI/mL (1) | 18 PCT | HCC (18) |
| Gisbert 2004 [9] | Retrospective, Single-center | Spain | 39 PCT | 4 (10) | 55 ± 16 | 69 | Elevated (1) | 1 PCT | HCC (1) |
| Cassiman 2008 [10] | Retrospective, single-center | Belgium | 17 Sporadic PCT | 7 (41) | 43 ± 3 | NR | NR | 1 PCT | HCC (1) |
| Lang 2015 [11] | Questionnaire | Germany | 122 (97 AIP, 20 VP, 4 HC, 1 ADDP) | NR | NR | NR | NR | 1 AIP | HCC (1) |
| Baravelli 2019 [12] | Retrospective, multicenter | Norway | 589 (243 sporadic PCT, 245 familial PCT, 101 unknown) | 319 (52) | 52 ± 13 | NR | NR | Did not classify | HCC (6) |
| Baravelli 2017 [13] | Retrospective, population registry | Norway | 251 (222 AIP, 21 VP, 8 HC) | 151 (60.2) | Median (range) 53 (19–96) | NR | NR | 8 AIP 1 VP | HCC (9) |
| Saberi 2020 [14] | Retrospective, multicenter | USA | 327 (270 AIP, 19 HC, 38 VP) | 266 (81) | 32 ± 5 | 69 ± 5 | <10 ng/mL (4) | 4 AIP 1 VP | HCC (5) |
| Lissing 2022 [15] | Retrospective, population registry | Sweden | 1244 (1063 AIP, 125 VP, 56 HC) | 654 (53) | Median (range) 36 (19–53) | Median (range) 71 (53–89) | NR | 81 AIP 1 VP 1 HCP | HCC (67), CC (3), unspecified (13) |
* Study design (i.e., prospective, retrospective, etc.) unclear. AHP—Acute hepatic porphyrias, AIP—Acute intermittent porphyria, VP—Variegate porphyria, HC— Hereditary coproporphyria, PCT—Porphyria cutanea tarda, ADDP—δ-aminolaevulinic acid dehydratase-deficient porphyria, NR—Not reported, HCC—Hepatocellular carcinoma, and CC—Cholangiocarcinoma.
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MDPI and ACS Style
Ramai, D.; Deliwala, S.S.; Chandan, S.; Lester, J.; Singh, J.; Samanta, J.; di Nunzio, S.; Perversi, F.; Cappellini, F.; Shah, A.; et al. Reply to Lissing et al. Comment on “Ramai et al. Risk of Hepatocellular Carcinoma in Patients with Porphyria: A Systematic Review. Cancers 2022, 14, 2947”. Cancers 2023, 15, 1187. https://doi.org/10.3390/cancers15041187
AMA Style
Ramai D, Deliwala SS, Chandan S, Lester J, Singh J, Samanta J, di Nunzio S, Perversi F, Cappellini F, Shah A, et al. Reply to Lissing et al. Comment on “Ramai et al. Risk of Hepatocellular Carcinoma in Patients with Porphyria: A Systematic Review. Cancers 2022, 14, 2947”. Cancers. 2023; 15(4):1187. https://doi.org/10.3390/cancers15041187
Chicago/Turabian StyleRamai, Daryl, Smit S. Deliwala, Saurabh Chandan, Janice Lester, Jameel Singh, Jayanta Samanta, Sara di Nunzio, Fabio Perversi, Francesca Cappellini, Aashni Shah, and et al. 2023. "Reply to Lissing et al. Comment on “Ramai et al. Risk of Hepatocellular Carcinoma in Patients with Porphyria: A Systematic Review. Cancers 2022, 14, 2947”" Cancers 15, no. 4: 1187. https://doi.org/10.3390/cancers15041187
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