Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review
Abstract Background Adult-onset Still’s disease (AOSD) often presents with systemic multiple lymphadenopathy. In addition to the common paracortical and mixed patterns in AOSD lymph node histopathological features, other morphological patterns include diffuse, necrotic, and follicular patterns. Howe...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2021-10-01
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| Series: | Diagnostic Pathology |
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| Online Access: | https://doi.org/10.1186/s13000-021-01159-3 |
| _version_ | 1818730073585352704 |
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| author | Zhonghua Huang Hua Xu Qinqin Min Zhenguo Li Jiaxin Bi Lingyun Liu Yingying Liang |
| author_facet | Zhonghua Huang Hua Xu Qinqin Min Zhenguo Li Jiaxin Bi Lingyun Liu Yingying Liang |
| author_sort | Zhonghua Huang |
| collection | DOAJ |
| description | Abstract Background Adult-onset Still’s disease (AOSD) often presents with systemic multiple lymphadenopathy. In addition to the common paracortical and mixed patterns in AOSD lymph node histopathological features, other morphological patterns include diffuse, necrotic, and follicular patterns. However, to date, there have been few reports on the histopathological description of AOSD lymph nodes. Case presentation An 18-year-old woman presented 2 months earlier with pain in her large joints with painless rash formation; bilateral posterior cervical lymph node, left supraclavicular lymph node, and left posterior axillary lymph node enlargement, and no tenderness. Left cervical lymph node resection was performed for pathological examination. The lymph node structure was basically preserved, and subcapsular and medullary sinus structures were observed. Many histiocytes in the sinus were observed, the cortical area was reduced, a few lymphoid follicles of different sizes were observed, and some atrophy and hyperplasia were noted. The lymphoid tissue in the paracortical region of the lymph node was diffusely proliferative and enlarged, mainly comprising histiocytes with abundant cytoplasm, immunoblasts and numerous lymphocytes with slightly irregular, small- to medium-sized nuclei. Nuclear karyorrhexis was easily observed, showing a few nuclear debris and the “starry sky” phenomenon, accompanied by abundantly branching high endothelial small vessels with few scattered plasma cells and eosinophil infiltration. Lymphoid follicle immunophenotype with reactive proliferative changes was observed. Approximately 40% of the cells in the paracortical region were positive for Ki-67, and the histiocytes expressed CD68, CD163, and some expressed S-100, with the absence of myeloperoxidase. The immunoblasts expressed CD30 and CD20, not ALK or CD15. Background small- to medium-sized T cells expressed CD2, CD3, CD5, CD7, CD4, and CD8; the number of CD8-positive T cells was slightly predominant, and a small number of T cells expressed granzyme B and T-cell intracellular antigen 1. The patient received a comprehensive medical treatment after the operation, and her condition was stable without progression at the 11-month follow-up evaluation. Conclusions The pathological features of AOSD lymphadenopathy raises the awareness of AOSD among pathologists and clinicians and aids in the diagnosis and differential diagnosis of AOSD lymphadenopathy from other reactive lymphadenopathies (lupus lymphadenitis, etc.) and lymphomas. |
| first_indexed | 2024-12-17T22:55:59Z |
| format | Article |
| id | doaj.art-5da564e121ed4d28906f0b5b1e4effe9 |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-12-17T22:55:59Z |
| publishDate | 2021-10-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-5da564e121ed4d28906f0b5b1e4effe92022-12-21T21:29:32ZengBMCDiagnostic Pathology1746-15962021-10-011611710.1186/s13000-021-01159-3Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature reviewZhonghua Huang0Hua Xu1Qinqin Min2Zhenguo Li3Jiaxin Bi4Lingyun Liu5Yingying Liang6Department of Pathology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese MedicineDepartment of Pathology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese MedicineDepartment of Pathology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese MedicineDepartment of Pathology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese MedicineDepartment of Pathology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese MedicineDepartment of Pathology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese MedicineDepartment of Pathology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese MedicineAbstract Background Adult-onset Still’s disease (AOSD) often presents with systemic multiple lymphadenopathy. In addition to the common paracortical and mixed patterns in AOSD lymph node histopathological features, other morphological patterns include diffuse, necrotic, and follicular patterns. However, to date, there have been few reports on the histopathological description of AOSD lymph nodes. Case presentation An 18-year-old woman presented 2 months earlier with pain in her large joints with painless rash formation; bilateral posterior cervical lymph node, left supraclavicular lymph node, and left posterior axillary lymph node enlargement, and no tenderness. Left cervical lymph node resection was performed for pathological examination. The lymph node structure was basically preserved, and subcapsular and medullary sinus structures were observed. Many histiocytes in the sinus were observed, the cortical area was reduced, a few lymphoid follicles of different sizes were observed, and some atrophy and hyperplasia were noted. The lymphoid tissue in the paracortical region of the lymph node was diffusely proliferative and enlarged, mainly comprising histiocytes with abundant cytoplasm, immunoblasts and numerous lymphocytes with slightly irregular, small- to medium-sized nuclei. Nuclear karyorrhexis was easily observed, showing a few nuclear debris and the “starry sky” phenomenon, accompanied by abundantly branching high endothelial small vessels with few scattered plasma cells and eosinophil infiltration. Lymphoid follicle immunophenotype with reactive proliferative changes was observed. Approximately 40% of the cells in the paracortical region were positive for Ki-67, and the histiocytes expressed CD68, CD163, and some expressed S-100, with the absence of myeloperoxidase. The immunoblasts expressed CD30 and CD20, not ALK or CD15. Background small- to medium-sized T cells expressed CD2, CD3, CD5, CD7, CD4, and CD8; the number of CD8-positive T cells was slightly predominant, and a small number of T cells expressed granzyme B and T-cell intracellular antigen 1. The patient received a comprehensive medical treatment after the operation, and her condition was stable without progression at the 11-month follow-up evaluation. Conclusions The pathological features of AOSD lymphadenopathy raises the awareness of AOSD among pathologists and clinicians and aids in the diagnosis and differential diagnosis of AOSD lymphadenopathy from other reactive lymphadenopathies (lupus lymphadenitis, etc.) and lymphomas.https://doi.org/10.1186/s13000-021-01159-3Adult-onset Still’s diseaseLymphadenopathyLupus lymphadenitisAutoinflammatory diseasesCase report |
| spellingShingle | Zhonghua Huang Hua Xu Qinqin Min Zhenguo Li Jiaxin Bi Lingyun Liu Yingying Liang Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review Diagnostic Pathology Adult-onset Still’s disease Lymphadenopathy Lupus lymphadenitis Autoinflammatory diseases Case report |
| title | Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review |
| title_full | Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review |
| title_fullStr | Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review |
| title_full_unstemmed | Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review |
| title_short | Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review |
| title_sort | adult onset still s disease with multiple lymphadenopathy a case report and literature review |
| topic | Adult-onset Still’s disease Lymphadenopathy Lupus lymphadenitis Autoinflammatory diseases Case report |
| url | https://doi.org/10.1186/s13000-021-01159-3 |
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