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Coronary pseudoaneurysm and superior vena cava occlusion in a young patient with multicentric Castleman's disease and antiphospholipid antibody positivity

Zafer Isilak, Mehmet Uzun, Mehmet Incedayi, Abdullah Haholu, Ersin Ozturk, Bekir Sıtkı Cebeci
DOI: http://dx.doi.org/10.1093/ejechocard/jer092 E35 First published online: 4 July 2011


Castleman disease is a non-malignant proliferative disease of the lymphoid system. It can be unicentric or multicentric. In this report, we present a case of multicentric Castleman disease to which coronary artery pseudoaneurysm, vena cava superior occlusion, and antiphospholipid antibody positivity was accompanying.

  • Castleman disease
  • Coronary pseudoaneurysm
  • Antiphospholipid antibody


Castleman disease is a non-malignant proliferative disease of lymphoid system, generally characterized by a single (unicentric) or multiple (multicentric) lymphadenopathy. The unicentric one has a good prognosis but multicentric one has a worse prognosis depending on the involved systems.1 It is generally a disease of fifth or sixth decade. Human immunodeficiency virus (HIV) positivity is frequent, especially in younger ones.2 Cardiovascular involvement is rare, limited to a few case reports. In this case report, we present a case with Castleman disease, to which coronary and systemic venous abnormalities are accompanying. Also, the antiphospholipid antibody positivity is present.


A 21-year-old male patient was admitted to our outpatient clinic for the examination for appropriateness to military service. He had complaints of exertional dyspnoea, atypical chest pain over left precordium, and swelling on neck and axilla. There were no risk factors for atherosclerosis. On admission, arterial blood pressure was 110/70 mmHg, pulse was 98 bpm, and temperature was 37.1°C. There were multiple lymphadenomegalies over the neck and axilla, venous distention over the neck, entire thorax and abdomen (Figure 1), and a moderate systolic murmur best heard over apical area. On electrocardiogram, lack of R progression over precordial leads and T negativity in DI and aVL was noted. The transthoracic echocardiography revealed a left ventricle of 55 mm diastolic diameter, ejection fraction of 45–50%, moderate mitral regurgitation, pericardial effusion (15 mm in width), pulmonary hypertension (40 mmHg), and a mass located between aortic valve, left atrium, and pulmonary artery (Figure 2, Supplementary data online, Video S1). The mass had an irregular wall with spontaneous echo contrast in it. The patient was referred to transoesophageal echocardiography, which confirmed the transthoracic echocardiographic findings and added the finding of a flow in the mass. Also, it was seen that the mass was very close to the left main coronary artery (Figure 3, Supplementary data online, Video S2AC). The patient undergone magnetic resonance imaging and computerized tomography. The magnetic resonance imaging showed that the superior vena cava is totally occluded (Figure 4). The occlusion was later confirmed by the use of agitated saline contrast study; bubbles given through both arms entered the right atrium via vena cava inferior. The computerized tomography confirmed the echocardiographic findings and defined the cardiac mass as a possible coronary pseudoaneurysm. Therefore, the patient was performed coronary angiography, which showed that the cardiac mass is a coronary pseudoaneurysm, originating from the left anterior descending artery. Besides, the distal part of the left anterior descending artery was filling retrogradely from distal branches of circumflex (Figure 5, Supplementary data online, Video S3). The right coronary artery and its branches were normal. The laboratory test results performed meanwhile are shown in Table 1. During the diagnostic process, the biopsy from lymph nodes of neck was also reported as ‘consistent with Castleman disease’. Histopathological examination of lymph node revealed involutional changes, including small follicles (Figure 6A) with hyalinized germinal centres. Some follicles have targetoid appearance (Figure 6B) and penetrating vessels through germinal centres causing so-called ‘lollipop’ appearance (Figure 6C). The patient was referred to cardiovascular surgery, but he refused the operation due to high risk of mortality. The patient was transferred to a more equipped hospital for medical treatment and regular follow-up. The patient died during the post-operative period at that centre.

View this table:
Table 1

Laboratory analysis

ParameterNormal rangeResult
Haemoglobin13.5–18 mg/dL9.8 mg/dL
Fibrinogen200–400 mg/dL577 mg/dL
Sedimentation rate<20 mm/h72 mm/h
C-reactive protein0–8 mg/L49.6 mg/L
Total IgG7.51–15.6 g/LNormal
Total IgM0.46–3.049 g/LNormal
Total IgA0.8–4.5 g/L4.63 g/L
Anti-phospholipid antibodyNegativePositive
Homocystein6–12 mcmol/L20.37 mcmol/L
Anti-HIV 1, 2NegativeNegative
Anti-HAV IgM<1.2 S/CONegative
Brucella Rose BengalNegativeNegative
Brucella WrightNegativeNegative
CMV IgMNegativeNegative
EBV IgMNegativeNegative
Group agglutinationNegativeNegative
Complement C40.16–0.38 g/L0.38 g/L
Complement C30.79–1.52 g/L1.62 g/L
Cardiolipin antibody IgGNegativeNegative
Cardiolipin antibody IgMNegativeNegative
Rheumatoid factor0–20 IU/mL20 IU/mL
Factor II prothrombin mutationNegativeNegative
Factor V H1299-FVR2 mutationNegativeNegative
MTHFR C677T mutationNegativeNegative
MTHFR A1298C mutationNegativeHeterozygote mutant
Factor V Leiden mutationNegativeNegative
Figure 1

The venous distention seen over chest and abdomen.

Figure 2

Transthoracic echocardiographic appearance of the mass. It should be noted that the mass is surrounded by aorta, left atrium, and pulmonary artery. Different positions of the transducer also revealed that the mass is anterolateral to the left ventricle. LA, left atrium; Ao, aortic valve; RVOT, right ventricular outflow tract; RV, right ventricle.

Figure 3

Transoesophageal echocardiographic appearance of the mass. Single-headed arrow indicates the mass, while double-headed arrow indicates the left main coronary artery. LA, left atrium; Ao, aortic valve; RVOT, right ventricular outflow tract; PA, pulmonary artery.

Figure 4

In the magnetic resonance imaging of the venous system, the superior vena cava could not be imaged. The black arrow shows where vena cava superior should have been. The white arrows indicate the collateral veins. It should be noted that the left subclavian vein is also occluded. SA, subclavian artery; SV, subclavian vein; ArA, arcus aorta; RA, right atrium; DA, descending aorta; VCI, vena cava inferior; LV, left ventricle; PsA, coronary pseudoaneurysm; IJ, internal jugular vein.

Figure 5

Coronary angiographic image of the coronary pseudoaneurysm. LMA, left main coronary artery; CFX, circumflex artery; LAD, left anterior descending artery; PsAn, coronary pseudoaneurysm.

Figure 6

Histological view of the lymph node. (A) Small follicles with hyalinized germinal centres; (B) a follicle with targetoid appearance; (C) penetrating vessels through germinal centres causing so-called ‘lollipop’ appearance.


Castleman's disease is a lymphoproliferative disease usually presenting as a solitary lesion in the mediastinum. For that reason, it was probably confused with thymoma until Castleman described it as a separate entity.3 Although it was first thought to be benign, determination of the multicentric form located it in a more malignant state. In unicentric cases, the complete resection of the mass is the choice of treatment but, in multicentric forms, there are not any definitive gold standard for therapy. The splenectomy may provide a temporal improvement in haematological sequels. Chemotherapy protocol, based on lymphoma schedules, resulted in intolerance, possibly due to accompanying HIV infection-induced immunological compromise. The monoclonal anti-interleukin-6 or anti-interleukin-6 receptor antibody treatment may alleviate the systemic manifestations,4,5 and remains the most promising target for therapy. In the present case, no treatment was started due to need for a more rigorous follow-up, which was beyond the task of the hospital, and therefore, transferred to a more equipped one.

The multicentric Castleman disease is a clinical entity of fourth or fifth decade; however, the present case is only 21 years old. Besides, the disease is frequently accompanied by HIV infection while the anti-HIV antibody was negative in the present case. The cardiovascular involvement is very low in multicentric Castleman disease. Malaisre et al.6 have reported a 59-year-old case of multicentric Castleman's disease with coronary pseudoaneurysm. That patient had ischaemic heart failure, as is in the present case, who had decreased ejection fraction. Systolic deterioration related to multicentric Castleman's disease without coronary involvement has not been reported. For this reason, we think that the decrease in ejection fraction is due to coronary lesions. In the report of Just et al.,7 coronary involvement was limited to perivascular pseudotumor without any pseudoaneurysm formation. Including the present case, common feature of all three cases is that the proximal segments of left anterior coronary arteries are involved.

Our case differs from the other two that it was associated with vena cava superior occlusion. This was evident by both physical examination, agitated saline contrast echocardiography, and magnetic resonance imaging. The lymphoma, which was the initial diagnosis of this patient, might also have caused hypercoagulable state. However, the biopsies obtained from lymph nodes and bone marrow excluded the lymphoma. There are no definite information about venous thrombosis in Castleman syndrome. However, interleukin 6, which is increased in Castleman disease, might have caused venous thrombosis.8 Another possible explanation may be the presence of antiphospholipid antibody positivity, which is shown to be associated with increased risk for thrombo-embolic events.9

In conclusion, the Castleman disease may present with very unusual symptoms. In patients with atypical pathologies involving the coronary arteries, Castleman disease should be suspected, especially, if accompanying lymphadenopathy is present. We also suggest that venous thrombosis should be looked due to the link between interleukin-6 and thrombosis. Whether antiphospholipid antibody positivity is only a coincidence or a component of the disease remains to be illuminated in further studies. This case is a good example for thinking about these relations.

Supplementary data

Supplementary data are available at European Journal of Echocardiography online.

Conflict of interest: none declared.


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