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Case Study
Attention! Cardiac tamponade may be caused by underlying Castleman’s disease
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313513772 aan.sagepub.com
Hilmi Atay1, Engin Kelkitli2, Muhammed Okuyucu3, Levent Yildiz4 and Mehmet Turgut5
Abstract Castleman’s disease is a rarely observed lymphoproliferative disease. In the literature, various signs and symptoms of the disease have been reported; one of these is secondary cardiac tamponade. We describe the case of a 41-year-old man who developed cardiac tamponade during examination, and who was later diagnosed with Castleman’s disease, based on his lymph node biopsies.
Keywords Biopsy, cardiac tamponade, diagnosis, differential, giant lymph node hyperplasia
Introduction Castleman’s disease (CD) is a rare lymphoproliferative disease. It was first described in 1954 as a benign lymphoproliferative disease.1 Other names for CD include giant lymph node hyperplasia, angiomatous lymphoid hamartoma, and lymph node hamartoma. Cardiac tamponade secondary to CD is an even rarer condition. We report a case of CD that presented cardiac tamponade.
Case report A 41-year-old man came to our hospital with complaints of weakness and night sweats for 2 months. Physical examination revealed lymphadenopathies on the liver, bilateral axillae, and bilateral inguinal sites, of 2, 1, and 2 cm, respectively, as well as a closed Traube’s space. Laboratory tests revealed white blood cells 10,300/mL, neutrophils 8700/mL, hemoglobin 10.6 gdL 1, platelets 673,000/mL, sedimentation rate 102 mmh 1, C-reactive protein 83 mgmL 1, total protein 8.1 gdL 1, albumin 3.4 gdL 1, blood urea nitrogen 10 mgdL 1, creatinine 0.76 mgdL 1, and negative for hepatitis B surface and core antibodies and HIV antibodies. Abdominal computed tomography identified the liver (177 mm) and spleen (143 mm) as well as numerous lymphadenopathies of 24 mm on the aortocaval, paraaortic, iliac, and both inguinal locations. Oval, homogenous, and hypodense mass lesions of 23 14 mm on the right adrenal gland and of 20 15 mm on the left
adrenal gland were noted; however, adrenal gland hormones were normal. In thorax computed tomography, lymph nodes of 14 28 mm were observed in both axillae, as well as loculated pleural fluid in the right lung, reaching 4 cm in diameter (Figure 1). Thoracentesis was carried out on the pleural fluid; the collected fluid was considered transudate. An excisional biopsy was planned for the axillary lymph nodes, but while an examination was being performed, the patient suddenly developed shortness of breath and hypotension. Distant heart sounds were heard. Echocardiography revealed pericardial fluid compressing the right ventricle and collapsing the right atrium. Cardiac tamponade was diagnosed. Due to the emergency nature of the situation, an echocardiograph record could not be taken, but an 1 Van Training and Research Hospital Department of Hematology, Van, Turkey 2 Erzurum Region Training and Research Hospital Department of Hematology, Erzurum, Turkey 3 19 Mayis University Medical School Department of Internal Medicine, Samsun, Turkey 4 19 Mayis University Medical School Department of Pathology, Samsun, Turkey 5 19 Mayis University Medical School Department of Hematology, Samsun, Turkey
Corresponding author: Engin Kelkiti, Erzurum Bo¨lge Eg˘itim ve Aras tırma Hastanesi 25770 Erzurum, Turkey. Email: [email protected]
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electrocardiogram was obtained (Figure 2). Approximately 300 cc of bloody fluid was drained by pericardiocentesis, and a biopsy was taken from the pericardium. In thorax tomography after drainage, a certain amount of effusion was observed around the heart (Figure 3). A cytological test on the collected fluid was negative for malignancy. The pericardial biopsy revealed fibrinous pericarditis. After the patient’s general condition improved, a large number of plasma cells were observed in the interfollicular area, hyaline vascular structures, and parafollicular areas of an axillary lymph node biopsy (Figure 4). The diagnosis was reported as mixed type Castleman’s disease.
Discussion Two main histological patterns of CD have been described: a hyaline vascular type (HVT) and a plasma cell type (PCT). A mixed type of CD has also been described. HVT is observed in 80%–90% of all cases, and PCT is observed in 10%–20% of cases. Clinical categorization of CD is either unicentric or multicentric.2 HVT is asymptomatic in most cases; it
Figure 1. Thorax computed tomography before drainage.
involves the thorax, and is generally observed in patients less than 30 years of age. PCT, on the other hand, is more symptomatic with fever, night sweating, weakness, weight loss, anemia, a high sedimentation rate, and hypergammaglobulinemia being frequently observed. Most patients with PCT are elderly.3 Clinically, HVT is observed more frequently in patients with unicentric CD, whereas multicentric CD is observed more frequently in PCT, and its progression is more aggressive. Hepatosplenomegaly, widespread lymphadenopathy, and systemic symptoms are common with multicentric CD. Unicentric CD is usually treated by surgery, and multicentric CD is more often treated by chemotherapy and radiotherapy.2 The development of cardiac tamponade in CD is rare with only 3 cases reported in the literature to date. Two of these cases presented with cardiac tamponade. In our case, cardiac tamponade developed while the patient was still undergoing examination for diagnosis.3–5 The pathology of CD has not been fully elucidated. Regarding the development of cardiac
Figure 3. Thorax computed tomography showing that the intimal effusion continued after drainage.
Figure 2. Electrocardiogram showing typical sinus tachycardia and no low-voltage QRS complexes.
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Figure 4. Axillary lymph node biopsy showing: (a) sheets of mature plasma cells within interfollicular tissues (hematoxylin and eosin stain, original magnification 2 40); (b) involuted follicle centers surrounded by expanded follicular mantle zones (‘‘lollipop follicles’’). Hematoxylin and eosin stain, original magnification 4 20.
tamponade, it is reported that an underlying inflammatory pathogenesis might be responsible for the condition. Due to inflammation in the serous membranes resulting from chronic antigenic stimulation (such as infection) or an autoimmune response, fluid resorption is impaired, leading to fluid accumulation between the layers of the pericardium.4 During differential diagnosis, clinicians should consider that lymphoproliferative diseases may be observed in cases that develop cardiac tamponade for unknown reasons. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Castleman B and Towne VW. Case records of the Massachusetts General Hospital: Case No. 40231. N Engl J Med 1954; 250: 1001–1005. 2. Saeed-Abdul-Rahman I and Al-Amri AM. Castleman disease. Korean J Hematol 2012; 47: 163–177. 3. Kim TU, Kim S, Lee JW, et al. Plasma cell type of Castleman’s disease involving renal parenchyma and sinus with cardiac tamponade: case report and literature review. Korean J Radiol 2012; 13: 658–663. 4. Frizzera G, Peterson BA, Bayrd ED and Goldman A. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease: clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985; 3: 1202–1216. 5. Zhang C, Miao Q, Chen G, et al. Pericardial tamponade secondary to Castleman’s disease. Ann Thorac Surg 2009; 88: 2039.
Downloaded from aan.sagepub.com at University of Birmingham on May 29, 2015
(AAN)
[1–3] [PREPRINTER stage]
Case Study
Attention! Cardiac tamponade may be caused by underlying Castleman’s disease
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313513772 aan.sagepub.com
Hilmi Atay1, Engin Kelkitli2, Muhammed Okuyucu3, Levent Yildiz4 and Mehmet Turgut5
Abstract Castleman’s disease is a rarely observed lymphoproliferative disease. In the literature, various signs and symptoms of the disease have been reported; one of these is secondary cardiac tamponade. We describe the case of a 41-year-old man who developed cardiac tamponade during examination, and who was later diagnosed with Castleman’s disease, based on his lymph node biopsies.
Keywords Biopsy, cardiac tamponade, diagnosis, differential, giant lymph node hyperplasia
Introduction Castleman’s disease (CD) is a rare lymphoproliferative disease. It was first described in 1954 as a benign lymphoproliferative disease.1 Other names for CD include giant lymph node hyperplasia, angiomatous lymphoid hamartoma, and lymph node hamartoma. Cardiac tamponade secondary to CD is an even rarer condition. We report a case of CD that presented cardiac tamponade.
Case report A 41-year-old man came to our hospital with complaints of weakness and night sweats for 2 months. Physical examination revealed lymphadenopathies on the liver, bilateral axillae, and bilateral inguinal sites, of 2, 1, and 2 cm, respectively, as well as a closed Traube’s space. Laboratory tests revealed white blood cells 10,300/mL, neutrophils 8700/mL, hemoglobin 10.6 gdL 1, platelets 673,000/mL, sedimentation rate 102 mmh 1, C-reactive protein 83 mgmL 1, total protein 8.1 gdL 1, albumin 3.4 gdL 1, blood urea nitrogen 10 mgdL 1, creatinine 0.76 mgdL 1, and negative for hepatitis B surface and core antibodies and HIV antibodies. Abdominal computed tomography identified the liver (177 mm) and spleen (143 mm) as well as numerous lymphadenopathies of 24 mm on the aortocaval, paraaortic, iliac, and both inguinal locations. Oval, homogenous, and hypodense mass lesions of 23 14 mm on the right adrenal gland and of 20 15 mm on the left
adrenal gland were noted; however, adrenal gland hormones were normal. In thorax computed tomography, lymph nodes of 14 28 mm were observed in both axillae, as well as loculated pleural fluid in the right lung, reaching 4 cm in diameter (Figure 1). Thoracentesis was carried out on the pleural fluid; the collected fluid was considered transudate. An excisional biopsy was planned for the axillary lymph nodes, but while an examination was being performed, the patient suddenly developed shortness of breath and hypotension. Distant heart sounds were heard. Echocardiography revealed pericardial fluid compressing the right ventricle and collapsing the right atrium. Cardiac tamponade was diagnosed. Due to the emergency nature of the situation, an echocardiograph record could not be taken, but an 1 Van Training and Research Hospital Department of Hematology, Van, Turkey 2 Erzurum Region Training and Research Hospital Department of Hematology, Erzurum, Turkey 3 19 Mayis University Medical School Department of Internal Medicine, Samsun, Turkey 4 19 Mayis University Medical School Department of Pathology, Samsun, Turkey 5 19 Mayis University Medical School Department of Hematology, Samsun, Turkey
Corresponding author: Engin Kelkiti, Erzurum Bo¨lge Eg˘itim ve Aras tırma Hastanesi 25770 Erzurum, Turkey. Email: [email protected]
Downloaded from aan.sagepub.com at University of Birmingham on May 29, 2015
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electrocardiogram was obtained (Figure 2). Approximately 300 cc of bloody fluid was drained by pericardiocentesis, and a biopsy was taken from the pericardium. In thorax tomography after drainage, a certain amount of effusion was observed around the heart (Figure 3). A cytological test on the collected fluid was negative for malignancy. The pericardial biopsy revealed fibrinous pericarditis. After the patient’s general condition improved, a large number of plasma cells were observed in the interfollicular area, hyaline vascular structures, and parafollicular areas of an axillary lymph node biopsy (Figure 4). The diagnosis was reported as mixed type Castleman’s disease.
Discussion Two main histological patterns of CD have been described: a hyaline vascular type (HVT) and a plasma cell type (PCT). A mixed type of CD has also been described. HVT is observed in 80%–90% of all cases, and PCT is observed in 10%–20% of cases. Clinical categorization of CD is either unicentric or multicentric.2 HVT is asymptomatic in most cases; it
Figure 1. Thorax computed tomography before drainage.
involves the thorax, and is generally observed in patients less than 30 years of age. PCT, on the other hand, is more symptomatic with fever, night sweating, weakness, weight loss, anemia, a high sedimentation rate, and hypergammaglobulinemia being frequently observed. Most patients with PCT are elderly.3 Clinically, HVT is observed more frequently in patients with unicentric CD, whereas multicentric CD is observed more frequently in PCT, and its progression is more aggressive. Hepatosplenomegaly, widespread lymphadenopathy, and systemic symptoms are common with multicentric CD. Unicentric CD is usually treated by surgery, and multicentric CD is more often treated by chemotherapy and radiotherapy.2 The development of cardiac tamponade in CD is rare with only 3 cases reported in the literature to date. Two of these cases presented with cardiac tamponade. In our case, cardiac tamponade developed while the patient was still undergoing examination for diagnosis.3–5 The pathology of CD has not been fully elucidated. Regarding the development of cardiac
Figure 3. Thorax computed tomography showing that the intimal effusion continued after drainage.
Figure 2. Electrocardiogram showing typical sinus tachycardia and no low-voltage QRS complexes.
Downloaded from aan.sagepub.com at University of Birmingham on May 29, 2015
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Figure 4. Axillary lymph node biopsy showing: (a) sheets of mature plasma cells within interfollicular tissues (hematoxylin and eosin stain, original magnification 2 40); (b) involuted follicle centers surrounded by expanded follicular mantle zones (‘‘lollipop follicles’’). Hematoxylin and eosin stain, original magnification 4 20.
tamponade, it is reported that an underlying inflammatory pathogenesis might be responsible for the condition. Due to inflammation in the serous membranes resulting from chronic antigenic stimulation (such as infection) or an autoimmune response, fluid resorption is impaired, leading to fluid accumulation between the layers of the pericardium.4 During differential diagnosis, clinicians should consider that lymphoproliferative diseases may be observed in cases that develop cardiac tamponade for unknown reasons. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Castleman B and Towne VW. Case records of the Massachusetts General Hospital: Case No. 40231. N Engl J Med 1954; 250: 1001–1005. 2. Saeed-Abdul-Rahman I and Al-Amri AM. Castleman disease. Korean J Hematol 2012; 47: 163–177. 3. Kim TU, Kim S, Lee JW, et al. Plasma cell type of Castleman’s disease involving renal parenchyma and sinus with cardiac tamponade: case report and literature review. Korean J Radiol 2012; 13: 658–663. 4. Frizzera G, Peterson BA, Bayrd ED and Goldman A. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease: clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985; 3: 1202–1216. 5. Zhang C, Miao Q, Chen G, et al. Pericardial tamponade secondary to Castleman’s disease. Ann Thorac Surg 2009; 88: 2039.
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