Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case report
Multiple opportunistic coinfections during corticosteroid therapy for the nephrotic syndrome are uncommon. Infection-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal multisystem disorder, manifesting as a state of hypercytokinemia in response to an infectious trigg...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2021-01-01
|
| Series: | Journal of Pediatric Critical Care |
| Subjects: | |
| Online Access: | http://www.jpcc.org.in/article.asp?issn=2349-6592;year=2021;volume=8;issue=6;spage=295;epage=298;aulast=Varadaraju |
| _version_ | 1797995529643229184 |
|---|---|
| author | Sireesh Varadaraju Priyanka Khandelwal Jhuma Sankar Pankaj Hari |
| author_facet | Sireesh Varadaraju Priyanka Khandelwal Jhuma Sankar Pankaj Hari |
| author_sort | Sireesh Varadaraju |
| collection | DOAJ |
| description | Multiple opportunistic coinfections during corticosteroid therapy for the nephrotic syndrome are uncommon. Infection-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal multisystem disorder, manifesting as a state of hypercytokinemia in response to an infectious trigger. We describe a 4½-year-old boy with steroid-dependent nephrotic syndrome receiving high-dose steroids, who developed respiratory failure due to pulmonary tuberculosis, Cytomegalovirus, and Pneumocystis jirovecii coinfections; HLH complicated the course. Aggressive management of the underlying infections with antitubercular, antiviral, and antifungal therapy, prompt recognition of HLH, and immunomodulation with intravenous (IV) immunoglobulin and IV methylprednisolone enabled convalescence. This report emphasizes the serious risks of immunosuppression and the need for strict vigilance for rare opportunistic infections with multiple pathogens in patients receiving oral steroids for nephrotic syndrome. Clinical distinctions between severe sepsis and HLH may be ambiguous; a high level of suspicion is required for timely recognition and management. |
| first_indexed | 2024-04-11T10:03:11Z |
| format | Article |
| id | doaj.art-a4d8e6ada6084a3a9e0d68992201d0fc |
| institution | Directory Open Access Journal |
| issn | 2349-6592 2455-7099 |
| language | English |
| last_indexed | 2024-04-11T10:03:11Z |
| publishDate | 2021-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Pediatric Critical Care |
| spelling | doaj.art-a4d8e6ada6084a3a9e0d68992201d0fc2022-12-22T04:30:20ZengWolters Kluwer Medknow PublicationsJournal of Pediatric Critical Care2349-65922455-70992021-01-018629529810.4103/jpcc.jpcc_64_21Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case reportSireesh VaradarajuPriyanka KhandelwalJhuma SankarPankaj HariMultiple opportunistic coinfections during corticosteroid therapy for the nephrotic syndrome are uncommon. Infection-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal multisystem disorder, manifesting as a state of hypercytokinemia in response to an infectious trigger. We describe a 4½-year-old boy with steroid-dependent nephrotic syndrome receiving high-dose steroids, who developed respiratory failure due to pulmonary tuberculosis, Cytomegalovirus, and Pneumocystis jirovecii coinfections; HLH complicated the course. Aggressive management of the underlying infections with antitubercular, antiviral, and antifungal therapy, prompt recognition of HLH, and immunomodulation with intravenous (IV) immunoglobulin and IV methylprednisolone enabled convalescence. This report emphasizes the serious risks of immunosuppression and the need for strict vigilance for rare opportunistic infections with multiple pathogens in patients receiving oral steroids for nephrotic syndrome. Clinical distinctions between severe sepsis and HLH may be ambiguous; a high level of suspicion is required for timely recognition and management.http://www.jpcc.org.in/article.asp?issn=2349-6592;year=2021;volume=8;issue=6;spage=295;epage=298;aulast=Varadarajucorticosteroidcytomegalovirusintravenous immunoglobulinpneumocystis jiroveciituberculosis |
| spellingShingle | Sireesh Varadaraju Priyanka Khandelwal Jhuma Sankar Pankaj Hari Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case report Journal of Pediatric Critical Care corticosteroid cytomegalovirus intravenous immunoglobulin pneumocystis jirovecii tuberculosis |
| title | Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case report |
| title_full | Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case report |
| title_fullStr | Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case report |
| title_full_unstemmed | Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case report |
| title_short | Multiple opportunistic infection-associated hemophagocytic lymphohistiocytosis in nephrotic syndrome: A case report |
| title_sort | multiple opportunistic infection associated hemophagocytic lymphohistiocytosis in nephrotic syndrome a case report |
| topic | corticosteroid cytomegalovirus intravenous immunoglobulin pneumocystis jirovecii tuberculosis |
| url | http://www.jpcc.org.in/article.asp?issn=2349-6592;year=2021;volume=8;issue=6;spage=295;epage=298;aulast=Varadaraju |
| work_keys_str_mv | AT sireeshvaradaraju multipleopportunisticinfectionassociatedhemophagocyticlymphohistiocytosisinnephroticsyndromeacasereport AT priyankakhandelwal multipleopportunisticinfectionassociatedhemophagocyticlymphohistiocytosisinnephroticsyndromeacasereport AT jhumasankar multipleopportunisticinfectionassociatedhemophagocyticlymphohistiocytosisinnephroticsyndromeacasereport AT pankajhari multipleopportunisticinfectionassociatedhemophagocyticlymphohistiocytosisinnephroticsyndromeacasereport |