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Clinical spectrum of intrathoracic Castleman disease: a retrospective analysis of 48 cases in a single Chinese hospital.

Luo JM, Li S, Huang H, Cao J, Xu K, Bi YL, Feng RE, Huang C, Qin YZ, Xu ZJ, Xiao Y - BMC Pulm Med (2015)

Bottom Line: Among MCD, 18 cases was improved, 7 cases was stable, 4 cases lost follow-up and 3 cases died.Mass, pleural effusion, BO and diffuse pulmonary shadows, including LIP-like images, multiple nodules of different size and sites, patchy, GGO and consolidations were showed in our MCD.Most of MCD cases were arranged with chemotherapy and their prognosis were worse than UCD's.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan Street, Dongcheng District, Beijing, 100730, China. palmljm@126.com.

ABSTRACT

Background: Thorax is the common place to develop Castleman disease (CD), but there is no systemic clinical analysis for intrathoracic CD.

Methods: We conducted a retrospective analysis of 48 intrathoracic CD patients with definite pathological diagnosis who were hospitalized between 1992 and 2012 in a Chinese tertiary referral hospital.

Results: The study included 16 cases with unicentric CD (UCD) and 32 cases with multicentric CD (MCD). UCD were younger than MCD (30.5y vs 41.6ys, P < 0.05). MCD were more symptomatic (50% vs 96.9%, P < 0.001) and sicker than UCD, including more fever, hepatomegaly and/or splenomegaly and hypoalbuminemia. All of UCD showed solitary mass in various sites and two of them were complicated by small pleural effusion. In the MCD group, their chest CT showed obvious lymphadenopathy in the hilum and/or mediastinum (100%), diffuse parenchymal lung shadows (43.75%), pleural effusion (40.6%), mass in the mediastinum (6.25%) or hilum (3.12%) and bronchiolitis obliterans (BO) (3.12%). Besides LIP-like images, multiple nodules of different size and sites, patchy, ground-glass opacities and consolidation were showed in their chest CT. Surgery were arranged for all UCD for diagnosis and treatment and all were alive. In MCD group, superficial lymph nodes biopsies (21 cases), surgery biopsy (9 cases) and CT-guided percutaneous lung biopsy (2 cases) were performed. Hyaline vascular (HV) variant were more common in the UCD group (75% vs 37.5%, P < 0.05). In MCD group, 28 cases were prescribed with chemotherapy, one refused to receive therapy and the rest three were arranged for regular follow-up. Among MCD, 18 cases was improved, 7 cases was stable, 4 cases lost follow-up and 3 cases died.

Conclusions: Intrathoracic MCD was more common than UCD in our hospital. MCD was older, more symptomic and sicker than UCD. HV variant were more common in UCD. All of UCD showed mass in various intrathoracic locations and surgery resection was performed for all and all were alive. Mass, pleural effusion, BO and diffuse pulmonary shadows, including LIP-like images, multiple nodules of different size and sites, patchy, GGO and consolidations were showed in our MCD. Most of MCD cases were arranged with chemotherapy and their prognosis were worse than UCD's.

No MeSH data available.


Related in: MedlinePlus

A 34-year-old man was diagnosed with multicentric Castleman disease of plasma cell variant after 11 years. He was complained of multiple superficial lymphadenopathy and intermittent low-grade fever for 11 years and exertional dyspnea for 4 months. His local multiple biopsies showed lymph node reactive hyperplasia. Short-term prednisone and repeated kinds of antibiotics were prescribed for him but his symptoms recurred. In our hospital, erythrocyte sedimentation rate (ESR) was 140 mm/h and IgG was 133 g/l, which was polycolonal. His high resolution chest CT showed multiple cysts and nodules, consolidations, diffuse bronchovascular thickening (LIP-like images) and multiple hilar and mediastinal lymphadenopathy (A-C). The fifth biopsy was tried in his left epitrochlear lymph nodes and the pathological photomicrographs showed marked interfollicular infiltration of plasma cells [D (Hematoxylin and eosin, ×200) and E (CD138 staining, ×200)]. Then he was diagnosed with Castleman disease of plasma cell variant. He and his families refused chemotherapy, but he showed up in the local clinic regularly.
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Fig2: A 34-year-old man was diagnosed with multicentric Castleman disease of plasma cell variant after 11 years. He was complained of multiple superficial lymphadenopathy and intermittent low-grade fever for 11 years and exertional dyspnea for 4 months. His local multiple biopsies showed lymph node reactive hyperplasia. Short-term prednisone and repeated kinds of antibiotics were prescribed for him but his symptoms recurred. In our hospital, erythrocyte sedimentation rate (ESR) was 140 mm/h and IgG was 133 g/l, which was polycolonal. His high resolution chest CT showed multiple cysts and nodules, consolidations, diffuse bronchovascular thickening (LIP-like images) and multiple hilar and mediastinal lymphadenopathy (A-C). The fifth biopsy was tried in his left epitrochlear lymph nodes and the pathological photomicrographs showed marked interfollicular infiltration of plasma cells [D (Hematoxylin and eosin, ×200) and E (CD138 staining, ×200)]. Then he was diagnosed with Castleman disease of plasma cell variant. He and his families refused chemotherapy, but he showed up in the local clinic regularly.

Mentions: All cases had significant abnormalities on their chest CT (Table 2), including obvious lymphadenopathy in the hilum and/or mediastinum (32/100%), diffuse parenchymal lung disease (14/43.75%), pleural effusion (13/40.6%), mass in the mediastinum (2/6.25%) or hilum (1/3.12%) and bronchiolitis obliterans (BO) (1/3.12%). Thoracentesis was performed in 8 of the cases. Exudative fluid was acquired in 6 of the cases and among them, 5 cases had bilateral effusions. None of the patients had chylous effusions. All of these 6 cases also had high levels of serum IgG. But only one of the cases underwent a pleural biopsy, which showed unspecific chronic inflammation. High-resolution chest CT in 14 of the cases with parenchymal involvement showed lymphocytic interstitial pneumonia (LIP)-like changes in 3 of the cases, multiple nodules of different sizes and sites in 6 of the cases, multiple patchy infiltrates in 6 of the cases, ground-glass opacities (GGO) in 5 of the cases and consolidation in 3 cases (Figure 2).Table 2


Clinical spectrum of intrathoracic Castleman disease: a retrospective analysis of 48 cases in a single Chinese hospital.

Luo JM, Li S, Huang H, Cao J, Xu K, Bi YL, Feng RE, Huang C, Qin YZ, Xu ZJ, Xiao Y - BMC Pulm Med (2015)

A 34-year-old man was diagnosed with multicentric Castleman disease of plasma cell variant after 11 years. He was complained of multiple superficial lymphadenopathy and intermittent low-grade fever for 11 years and exertional dyspnea for 4 months. His local multiple biopsies showed lymph node reactive hyperplasia. Short-term prednisone and repeated kinds of antibiotics were prescribed for him but his symptoms recurred. In our hospital, erythrocyte sedimentation rate (ESR) was 140 mm/h and IgG was 133 g/l, which was polycolonal. His high resolution chest CT showed multiple cysts and nodules, consolidations, diffuse bronchovascular thickening (LIP-like images) and multiple hilar and mediastinal lymphadenopathy (A-C). The fifth biopsy was tried in his left epitrochlear lymph nodes and the pathological photomicrographs showed marked interfollicular infiltration of plasma cells [D (Hematoxylin and eosin, ×200) and E (CD138 staining, ×200)]. Then he was diagnosed with Castleman disease of plasma cell variant. He and his families refused chemotherapy, but he showed up in the local clinic regularly.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4404013&req=5

Fig2: A 34-year-old man was diagnosed with multicentric Castleman disease of plasma cell variant after 11 years. He was complained of multiple superficial lymphadenopathy and intermittent low-grade fever for 11 years and exertional dyspnea for 4 months. His local multiple biopsies showed lymph node reactive hyperplasia. Short-term prednisone and repeated kinds of antibiotics were prescribed for him but his symptoms recurred. In our hospital, erythrocyte sedimentation rate (ESR) was 140 mm/h and IgG was 133 g/l, which was polycolonal. His high resolution chest CT showed multiple cysts and nodules, consolidations, diffuse bronchovascular thickening (LIP-like images) and multiple hilar and mediastinal lymphadenopathy (A-C). The fifth biopsy was tried in his left epitrochlear lymph nodes and the pathological photomicrographs showed marked interfollicular infiltration of plasma cells [D (Hematoxylin and eosin, ×200) and E (CD138 staining, ×200)]. Then he was diagnosed with Castleman disease of plasma cell variant. He and his families refused chemotherapy, but he showed up in the local clinic regularly.
Mentions: All cases had significant abnormalities on their chest CT (Table 2), including obvious lymphadenopathy in the hilum and/or mediastinum (32/100%), diffuse parenchymal lung disease (14/43.75%), pleural effusion (13/40.6%), mass in the mediastinum (2/6.25%) or hilum (1/3.12%) and bronchiolitis obliterans (BO) (1/3.12%). Thoracentesis was performed in 8 of the cases. Exudative fluid was acquired in 6 of the cases and among them, 5 cases had bilateral effusions. None of the patients had chylous effusions. All of these 6 cases also had high levels of serum IgG. But only one of the cases underwent a pleural biopsy, which showed unspecific chronic inflammation. High-resolution chest CT in 14 of the cases with parenchymal involvement showed lymphocytic interstitial pneumonia (LIP)-like changes in 3 of the cases, multiple nodules of different sizes and sites in 6 of the cases, multiple patchy infiltrates in 6 of the cases, ground-glass opacities (GGO) in 5 of the cases and consolidation in 3 cases (Figure 2).Table 2

Bottom Line: Among MCD, 18 cases was improved, 7 cases was stable, 4 cases lost follow-up and 3 cases died.Mass, pleural effusion, BO and diffuse pulmonary shadows, including LIP-like images, multiple nodules of different size and sites, patchy, GGO and consolidations were showed in our MCD.Most of MCD cases were arranged with chemotherapy and their prognosis were worse than UCD's.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan Street, Dongcheng District, Beijing, 100730, China. palmljm@126.com.

ABSTRACT

Background: Thorax is the common place to develop Castleman disease (CD), but there is no systemic clinical analysis for intrathoracic CD.

Methods: We conducted a retrospective analysis of 48 intrathoracic CD patients with definite pathological diagnosis who were hospitalized between 1992 and 2012 in a Chinese tertiary referral hospital.

Results: The study included 16 cases with unicentric CD (UCD) and 32 cases with multicentric CD (MCD). UCD were younger than MCD (30.5y vs 41.6ys, P < 0.05). MCD were more symptomatic (50% vs 96.9%, P < 0.001) and sicker than UCD, including more fever, hepatomegaly and/or splenomegaly and hypoalbuminemia. All of UCD showed solitary mass in various sites and two of them were complicated by small pleural effusion. In the MCD group, their chest CT showed obvious lymphadenopathy in the hilum and/or mediastinum (100%), diffuse parenchymal lung shadows (43.75%), pleural effusion (40.6%), mass in the mediastinum (6.25%) or hilum (3.12%) and bronchiolitis obliterans (BO) (3.12%). Besides LIP-like images, multiple nodules of different size and sites, patchy, ground-glass opacities and consolidation were showed in their chest CT. Surgery were arranged for all UCD for diagnosis and treatment and all were alive. In MCD group, superficial lymph nodes biopsies (21 cases), surgery biopsy (9 cases) and CT-guided percutaneous lung biopsy (2 cases) were performed. Hyaline vascular (HV) variant were more common in the UCD group (75% vs 37.5%, P < 0.05). In MCD group, 28 cases were prescribed with chemotherapy, one refused to receive therapy and the rest three were arranged for regular follow-up. Among MCD, 18 cases was improved, 7 cases was stable, 4 cases lost follow-up and 3 cases died.

Conclusions: Intrathoracic MCD was more common than UCD in our hospital. MCD was older, more symptomic and sicker than UCD. HV variant were more common in UCD. All of UCD showed mass in various intrathoracic locations and surgery resection was performed for all and all were alive. Mass, pleural effusion, BO and diffuse pulmonary shadows, including LIP-like images, multiple nodules of different size and sites, patchy, GGO and consolidations were showed in our MCD. Most of MCD cases were arranged with chemotherapy and their prognosis were worse than UCD's.

No MeSH data available.


Related in: MedlinePlus