Scientific Letters
Vol. 18 No. 1 (2026): Mediterranean Journal of Hematology and Infectious Diseases
Acute fulminant Clostridium-Perfrigens sepsis with massive intravascular hemolysis in a young woman with sickle cell disease. Case presentation and review of the literature
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Published: October 31, 2026
0
Views
0
Downloads
Authors
1) Hemoglobinopathies Unit, Hematology Department, S. Eugenio Hospital (ASL Roma 2), Rome, Italy., Italy.
2) Department of Anesthesiology, Sant'Eugenio Hospital, Rome, Italy., Italy.
1) Hemoglobinopathies Unit, Hematology Department, S. Eugenio Hospital (ASL Roma 2), Rome, Italy., Italy.
1) Hemoglobinopathies Unit, Hematology Department, S. Eugenio Hospital (ASL Roma 2), Rome, Italy., Italy.
3) Department of Haematology, University of Rome Tor Vergata, Rome, Italy., Italy.
1) Hemoglobinopathies Unit, Hematology Department, S. Eugenio Hospital (ASL Roma 2), Rome, Italy. 3) Department of Haematology, University of Rome Tor Vergata, Rome, Italy., Italy.
To the Editor
Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a single amino acid substitution at the sixth residue of the beta (β)-globin subunit (p.Glu6Val)
Vaso-occlusion, hemolytic anemia and vasculopathy are the hallmarks of SCD pathophysiology, leading to high morbility and mortality.
REFERENCES
1) Pinto VM., Balocco M., Quintino S., Forni GL. Sickle cell disease: a review for the internist. Intern Emerg Med. 2019 Oct;14(7):1051-1064. doi: 10.1007/s11739-019-02160-x. Epub 2019 Aug 5. PMID: 31385153.
doi : 10.1007/s11739-019-02160-x
2) Inusa BP., Atoyebi W., Andemariam B., Hourani JN., Omert L. Global burden of transfusion in sickle cell disease. Transfus Apher Sci. 2023 Oct;62(5):103764. Epub 2023 Jul 17. PMID: 37541800
doi: 10.1016/j.transci.2023.103764..
3) Gianesin B., Piel FB., Musallam KM., Barella S., Casale M., Cassinerio E., Di Maggio R., Gigante A., Gamberini MR., Graziadei G., Lisi R., Longo F., Maggio A., Origa R., Pasanisi A., Perrotta S., Piga AG., Pinto VM., Rosso R., Robello G., Russo G., Zecca M., De Franceschi L., Forni GL.,National Survey Group IH. Prevalence and mortality trends of hemoglobinopathies in Italy: a nationwide study. Haematologica. 2025 May 1;110(5):1211.
Epub 2025 Jan 23. Erratum in: Haematologica. 2025 Jun 1;110(6):1449-1450. PMID: 39844751; PMCID: PMC12050947.
doi: 10.3324/haematol.2025.287581.
4) Butt, H., & Tisdale, J. F. (2024). Gene therapies on the horizon for sickle cell disease: a clinician’s perspective. Expert Review of Hematology, 17(9), 555–566. https://doi.org/10.1080/17474086.2024.2386366
5) Ogun GO., Ebili H., Kotila TR.. Autopsy findings and pattern of mortality in Nigerian sickle cell disease patients. Pan Afr Med J. 2014 May 8;18:30. PMID: 25368719; PMCID: PMC4215364.
doi10.11604/pamj.2014.18.30.4043.
6) Cannas G., Merazga S., Virot E. Sickle Cell Disease and Infections in High- and Low-Income Countries. Mediterr J Hematol Infect Dis. 2019 Jul 1;11(1):e2019042. PMID: 31308918; PMCID: PMC6613623.
doi: 10.4084/MJHID.2019.042.
7) Scourfield LEA., Nardo-Marino A., Williams TN., Rees DC. Infections in sickle cell disease. Haematologica. 2025 Mar 1;110(3):546-561. PMID: 39568431; PMCID: PMC11873697.
doi: 10.3324/haematol.2024.285066.
8) Piel FB., Rees DC., DeBaun MR., Nnodu O., Ranque B., Thompson AA., Ware RE., Abboud MR., Abraham A., Ambrose EE., Andemariam B., Colah R., Colombatti R., Conran N., Costa FF., Cronin RM., de Montalembert M., Elion J., Esrick E., Greenway AL., Idris IM, Issom DZ, Jain D, Jordan LC, Kaplan ZS, King AA, Lloyd-Puryear M, Oppong SA, Sharma A, Sung L., Tshilolo L., Wilkie DJ., Ohene-Frempong K.. Defining global strategies to improve outcomes in sickle cell disease: a Lancet Haematology Commission. Lancet Haematol. 2023 Aug;10(8):e633-e686. Epub 2023 Jul 11. PMID: 37451304; PMCID: PMC11459696.
doi: 10.1016/S2352-3026(23)00096-0.
9) Piel FB., DeBaun MR., Nnodu O. Commission authors. Sickle cell disease strategies and priorities - Authors' reply. Lancet Haematol. 2023 Oct;10(10):e795. 3026(23)00269-7. PMID: 37793767.
doi: 10.1016/S2352-
10) GBD 2021 Sickle Cell Disease Collaborators. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of Disease Study 2021. Lancet Haematol. 2023 Aug;10(8):e585-e599. Epub 2023 Jun 15. Erratum in: Lancet Haematol. 2023 Aug;10(8):e574. doi: 10.1016/S2352-3026(23)00215-6. PMID: 37331373; PMCID: PMC10390339.
doi: 10.1016/S2352-3026(23)00118-7.
11) Kojima H., Shimizu Y., Sugiyama K. Clostridium perfringens bacteremia and intravascular hemolysis. QJM. 2023 Feb 24;116(2):139-140.
doi: 10.1093/qjmed/hcac236. PMID: 36222591.
12) Poletti G., Massari E., Fabbri A., Monti M., Rosetti M., Sambri V. Massive intravascular hemolysis associated with Clostridium perfringens bacteremia. Blood Res. 2021 Mar 31;56(1):5. PMID: 33542164; PMCID: PMC7987482.
doi: 10.5045/br.2021.2020281.
13) Hifumi T. Spontaneous Non-Traumatic Clostridium perfringens Sepsis. Jpn J Infect Dis. 2020 May 22;73(3):177-180Epub 2019 Dec 25. PMID: 31875607.
doi: 10.7883/yoken.JJID.2019.382.
14) Yamakawa Y., Ohsaka A. Purification and some properties of phospholipase C (alpha-toxin) of Clostridium perfringens. J Biochem. 1977 Jan;81(1):115-26. PMID: 191435.
doi: 10.1093/oxfordjournals.jbchem.a131426.
15) Hara-Kudo Y., Yamakawa Y., Kumagai S. Purification and some properties of Clostridium sporogenes hemorrhagic toxin. Biochem Biophys Res Commun. 1996 Oct 14;227(2):413-8. doi: 10.1006/bbrc.1996.1521. PMID: 8878529.
16) Kubo T., Kawano Y., Miyanishi K . Kazuma I., Sakurada A., Sato M., Hoki T., Tamura F., Tanaka S., Takada K., Kobune M, Kato J.Two cases of liver abscess caused by Clostridium perfringens that potentially cured by combined modality therapy. Kanzo 2017; 58: 105-14.
17) Yamamoto Y., Itoh N., Sugiyama T., Kurai H. Clinical features of Clostridium bacteremia in cancer patients: A case series review. J Infect Chemother. 2020 Jan;26(1):92-94. Epub 2019 Aug 10. PMID: 31409524.
doi: 10.1016/j.jiac.2019.07.019.
18) Liu F., Xue S., Zhang Y., Yang J., Hu J., Li D., Ma X., Wang J. Clostridium perfringens sepsis in three patients with acute leukemia and review of the literature. Int J Hematol. 2021 Apr;113(4):508-517. Epub 2021 Jan 2. PMID: 33387294; PMCID: PMC7776304.
doi: 10.1007/s12185-020-03060-z.
19) Vaiopoulos G., Calpadaki C., Sinifakoulis H., Konstantopoulos K., Avlami A., Stefanou J., Pangalis GA. Massive intravascular hemolysis: a fatal complication of clostridium perfringens septicemia in a patient with acute myeloid leukemia. Leuk Lymphoma. 2004 Oct;45(10):2157-9. PMID: 15370265.
doi: 10.1080/10428190410001697331.
20) Yamamoto H., Mizutani Y., Iriyama C., Goto N., Okamoto A., Kato T., Shintani C., Yamamoto N., Miyama T., Mihara K., Okamoto M., Tomita A. Acute fulminant intravascular hemolysis induced by Clostridium perfringens in a symptomatic multiple myeloma patient under immuno-chemotherapy. Ann Hematol. 2022 Dec;101(12):2813-2815. . Epub 2022 Nov 1. PMID: 36318289
doi: 10.1007/s00277-022-05010-7.
21) Patel I., Odak M., Douedi S., Alshami A., Upadhyaya VD. Hossain M, Anne M, Patel SV. Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease. Eur J Case Rep Intern Med. 2021 Aug 7;8(10):002824. PMID: 34790624; PMCID: PMC8592653
doi: 10.12890/2021_002824..
Ethics Approval
sickle cell disease, sepsis, asplenia, clostridium perfrigens, hemolysisSupporting Agencies
None. No specific funding was received for this study.How to Cite
“Acute fulminant Clostridium-Perfrigens sepsis with massive intravascular hemolysis in a young woman with sickle cell disease. Case presentation and review of the literature” (2026) Mediterranean Journal of Hematology and Infectious Diseases, 18(1), p. e2026080. doi:10.4084/MJHID.2026.080.
Copyright (c) 2026 Lorenza Torti, Mauro Polzoni, Nicolina Ardu, Laura Maffei, Beatrice Basso, Maria Ilaria Del Principe


This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.






