Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report
Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized w...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Yeungnam University College of Medicine, Yeungnam University Institute Medical Science
2022-04-01
|
| Series: | Journal of Yeungnam Medical Science |
| Subjects: | |
| Online Access: | http://www.e-jyms.org/upload/pdf/yujm-2021-01039.pdf |
| _version_ | 1811304218594639872 |
|---|---|
| author | Hyun-Je Kim Young-Hoon Hong |
| author_facet | Hyun-Je Kim Young-Hoon Hong |
| author_sort | Hyun-Je Kim |
| collection | DOAJ |
| description | Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia. |
| first_indexed | 2024-04-13T08:02:07Z |
| format | Article |
| id | doaj.art-14fa097bc5004108b33d3f296702cae5 |
| institution | Directory Open Access Journal |
| issn | 2799-8010 |
| language | English |
| last_indexed | 2024-04-13T08:02:07Z |
| publishDate | 2022-04-01 |
| publisher | Yeungnam University College of Medicine, Yeungnam University Institute Medical Science |
| record_format | Article |
| series | Journal of Yeungnam Medical Science |
| spelling | doaj.art-14fa097bc5004108b33d3f296702cae52022-12-22T02:55:15ZengYeungnam University College of Medicine, Yeungnam University Institute Medical ScienceJournal of Yeungnam Medical Science2799-80102022-04-0139215316010.12701/yujm.2021.010392652Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case reportHyun-Je Kim0Young-Hoon Hong1 Division of Rheumatology, Department of Internal Medicine, CHA Gumi Medical Center, CHA University, Gumi, Korea Division of Rheumatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, KoreaMulticentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.http://www.e-jyms.org/upload/pdf/yujm-2021-01039.pdfimmunoglobulin g4-related diseaseinterstitial lung diseaseslangerhans-cell histiocytosismulticentric castleman disease |
| spellingShingle | Hyun-Je Kim Young-Hoon Hong Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report Journal of Yeungnam Medical Science immunoglobulin g4-related disease interstitial lung diseases langerhans-cell histiocytosis multicentric castleman disease |
| title | Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report |
| title_full | Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report |
| title_fullStr | Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report |
| title_full_unstemmed | Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report |
| title_short | Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report |
| title_sort | idiopathic multicentric castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin g and immunoglobulin g4 dominant hypergammaglobulinemia a case report |
| topic | immunoglobulin g4-related disease interstitial lung diseases langerhans-cell histiocytosis multicentric castleman disease |
| url | http://www.e-jyms.org/upload/pdf/yujm-2021-01039.pdf |
| work_keys_str_mv | AT hyunjekim idiopathicmulticentriccastlemandiseasepresentingprogressivereticularhoneycombinfiltrationoflungandimmunoglobulingandimmunoglobuling4dominanthypergammaglobulinemiaacasereport AT younghoonhong idiopathicmulticentriccastlemandiseasepresentingprogressivereticularhoneycombinfiltrationoflungandimmunoglobulingandimmunoglobuling4dominanthypergammaglobulinemiaacasereport |