An Adult-Onset Chronic Granulomatous Disease Case with Hemophagocytic Lymphohistiocytosis Caused by Burkholderia and Aspergillus Infections

被引：0

作者：

Kaygusuz, Yunus ^{[1
]}

Ustun, Ceren ^{[2
]}

Er, Ahmet Gorkem ^{[3
]}

Kiki, Zehranur ^{[4
]}

Yaz, Ismail ^{[5
]}

Tuten-Dal, Sevda ^{[2
]}

Uner, Aysegul ^{[4
]}

Cagdas, Deniz ^{[2
]}

Inkaya, Ahmet cagkan ^{[3
]}

Akova, Murat ^{[3
]}

机构：

[1] Hacettepe Univ, Sch Med, Dept Internal Med, Ankara, Turkiye

[2] Hacettepe Univ, Sch Med, Dept Pediat, Div Immunol, Ankara, Turkiye

[3] Hacettepe Univ, Sch Med, Dept Infect Dis & Clin Microbiol, Ankara, Turkiye

[4] Hacettepe Univ, Sch Med, Dept Pathol, Ankara, Turkiye

[5] Hacettepe Univ, Dept Pediat Basic Sci, Div Immunol, Sch Med, Ankara, Turkiye

来源：

INFECTIOUS DISEASES AND CLINICAL MICROBIOLOGY | 2024年 / 6卷 / 04期

关键词：

granulomatous disease; hemophagocytic lymphohistiocytosis; invasive pulmonary aspergillosis; Burkholderia infections; PRIMARY IMMUNODEFICIENCIES;

D O I：

10.36519/idcm.2024.381

中图分类号：

R51 [传染病];

学科分类号：

100401 ;

摘要：

Chronic granulomatous disease (CGD) is a congenital disorder impairing phagocyte function, causing recurrent, life-threatening infections, and is rarely seen in adulthood. We present a 36-year-old male initially diagnosed with pneumonia. Bronchoalveolar lavage and blood cultures yielded Burkholderia multivorans/cepacia complex, sputum cultures Aspergillus niger. Despite the antimicrobial treatment, his condition deteriorated. His clinical and laboratory findings indicated hemophagocytic lymphohistiocytosis. He responded to steroids. Nitroblue tetrazolium and dihydroergotamine-123 tests confirmed CGD. Whole exome sequencing identified NCF1 deletion. He received interferon-gamma, voriconazole, and trimethoprim-sulfamethoxazole. Allogeneic hematopoietic stem cell transplantation was planned. This case report improves understanding of CGD in adults, aiming to enhance diagnostic and therapeutic strategies.

引用

页码：334 / 338

页数：5

共 48 条

[31] Hemophagocytic lymphohistiocytosis caused by herpes simplex virus type 1 in a young adult: a case report with literature review [J].

Zhang, Hanqing ;

Zhang, Peng ;

Xiao, Zhifang ;

Gao, Yang ;

Han, Na ;

He, Xianjun ;

Zhang, Jinfeng ;

Li, Yonghua .

JOURNAL OF HEMATOPATHOLOGY, 2024, 17 (02) :91-96

[32] Usefulness of Interleukin-18 as a Diagnostic Biomarker to Differentiate Adult-Onset Still's Disease With/Without Macrophage Activation Syndrome From Other Secondary Hemophagocytic Lymphohistiocytosis in Adults [J].

Shiga, Toshihiko ;

Nozaki, Yuji ;

Tomita, Daisuke ;

Kishimoto, Kazuya ;

Hirooka, Yasuaki ;

Kinoshita, Koji ;

Funauchi, Masanori ;

Matsumura, Itaru .

FRONTIERS IN IMMUNOLOGY, 2021, 12

[33] Unveiling the Mystery of Adult-Onset Still's Disease: A Compelling Case Report [J].

Sola, Daniele ;

Smirne, Carlo ;

Bruggi, Francesco ;

Sbaratta, Chiara Bottino ;

Njata, Aubin Cardin Tamen ;

Valente, Guido ;

Pavanelli, Maria Cristina ;

Vitetta, Rosetta ;

Bellan, Mattia ;

De Paoli, Lorenzo ;

Pirisi, Mario .

LIFE-BASEL, 2024, 14 (02)

[34] A case of adult-onset X-linked lymphoproliferative disease mimicking pulmonary infection [J].

Xu, Jiuyang ;

Wang, Yan ;

Zhang, Yuan ;

Zhai, Tianshu ;

Li, Zhenling ;

Zhong, Dingrong ;

Cao, Bin ;

Cui, Xiaojing .

BMC INFECTIOUS DISEASES, 2024, 24 (01)

[35] Adult onset hemophagocytic lymphohistiocytosis prognosis is affected by underlying disease and coexisting viral infection: analysis of a single institution series of 35 patients [J].

Cattaneo, Chiara ;

Oberti, Margherita ;

Skert, Cristina ;

Passi, Angela ;

Farina, Mirko ;

Re, Alessandro ;

Tozzi, Paola ;

Borlenghi, Erika ;

Rossi, Giuseppe .

HEMATOLOGICAL ONCOLOGY, 2017, 35 (04) :828-834

[36] Acute fibrinous and organizing pneumonia complicated with hemophagocytic lymphohistiocytosis caused by chronic active Epstein-Barr virus infection: a case report [J].

Wu, Xiaojing ;

Wang, Kejing ;

Gao, Yayue ;

Cai, Ying ;

Wang, Wenqiao ;

Zhong, Dingrong ;

Zhan, Qingyuan .

BMC INFECTIOUS DISEASES, 2021, 21 (01)

[37] Acute fibrinous and organizing pneumonia complicated with hemophagocytic lymphohistiocytosis caused by chronic active Epstein-Barr virus infection: a case report [J].

Xiaojing Wu ;

Kejing Wang ;

Yayue Gao ;

Ying Cai ;

Wenqiao Wang ;

Dingrong Zhong ;

Qingyuan Zhan .

BMC Infectious Diseases, 21

[38] Hemophagocytic lymphohistiocytosis resulting from a cytokine storm triggered by septicemia in a child with chronic granuloma disease: a case report and literature review [J].

Ang Wei ;

Honghao Ma ;

Liping Zhang ;

Zhigang Li ;

Qing Zhang ;

Dong Wang ;

Li Zhang ;

Hongyun Lian ;

Rui Zhang ;

Tianyou Wang .

BMC Pediatrics, 20

[39] Hemophagocytic lymphohistiocytosis resulting from a cytokine storm triggered by septicemia in a child with chronic granuloma disease: a case report and literature review [J].

Wei, Ang ;

Ma, Honghao ;

Zhang, Liping ;

Li, Zhigang ;

Zhang, Qing ;

Wang, Dong ;

Zhang, Li ;

Lian, Hongyun ;

Zhang, Rui ;

Wang, Tianyou .

BMC PEDIATRICS, 2020, 20 (01)

[40] A Challenging Case of Adult-Onset Still's Disease Complicated by Macrophage Activation Syndrome With Multiorgan Failure [J].

Mohamed, Abdelaziz ;

Yasin, Ahmed K. A. ;

Mohamed, Anas ;

Elamin, Nusiba ;

Mustafa, Salma ;

Fadul, Abdalla ;

Syed, Ghulam ;

Al-Tikrity, Mustafa .

CUREUS JOURNAL OF MEDICAL SCIENCE, 2023, 15 (08)

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