Increased interleukin-6 activity in cardiac myxoma with mediastinal lymphadenopathy.

We report a case of cardiac myxoma with mediastinal lymphadenopathy which seems to be a very rare feature. Laboratory examination revealed high level of interleukin-6 (IL-6) activity in serum. Interestingly, after removal of cardiac myxoma, mediastinal lymphadenopathy disappeared on the chest CT and the level of serum IL-6 was decreased. These findings suggest that IL-6 which was probably derived from myxoma, may have played an important role in the development of lymphadenopathy.     

An unusual case of mediastinal lymphadenopathy caused by amyloidosis

A 71-year-old Japanese female Nagasaki-atomic-bomb survivor was admitted for evaluation of a mediastinal mass. She was infected with human T-cell leukemia virus type I. Histological examination of the biopsy specimen, obtained thoracoscopically from the mass, revealed amyloid lymphadenopathy confirmed by Congo-red staining and electron-microscopic examination. Amyloid deposits and the serum monoclonal peak consisted of immunoglobulin lambda light chains. No atypical cells were detected in bone marrow. The case was diagnosed as an unusual amyloidosis affecting the mediastinal lymph node. No other sites were found to be affected by amyloidosis, although systemic involvement could not be conclusively ruled out.     

A case of cardiac myxoma associated with congenital oculocutaneous albinism

This case is a 43 year-old-woman of left atrial myxoma associated with oculocutaneous albinism, who was admitted to our hospital because of nocturnal cough everyday. Electrocardiogram showed normal sinus rhythm and complete right bundle branch block. Chest X-ray disclosed slight enlargement of left atrium. Echocardiography revealed the left atrial pedunculated tumor, arising from atrial septum, prolapsing into left ventricle during diastole, which was coincided with lucent tumor by levophase of pulmonary arteriography. Feeder artery of this tumor was visualized by right coronary arteriography. Cardiac catheterization represented normal pressure of right-sided heart. Thus the diagnosis of left atrial myxoma was established. At the operation, radical excision of the base of tumor and septum and patch closure was undergone. The gross morphology and microscopic findings were consistent with myxoma.     

Elevated interleukin-6 levels are associated with impaired outcome in cardiac transthyretin amyloidosis

BACKGROUNDElevated interleukin (IL)-6-levels have been described in familial variant transthyretin amyloidosis (ATTRv) associated polyneuropathy and heart failure. However, IL-6 in cardiac ATTR amyloidosis (ATTR-CM) and its prognostic value have not been investigated yet.AIMWe aim to study the correlation between IL-6 levels with clinical presentation (Gillmore-class) and outcome [heart transplantation or death (htx/death)], or the combined endpoint of cardiac decompensation or htx/death in ATTR-CM. METHODSIL-6 levels of 106 ATTR-CM patients [54 wild-type ATTRwt, 52 ATTRv-CM], 15 asymptomatic carriers of ATTR mutations (aATTRv-CM) and 27 healthy donors were quantified using Luminex technology. Statistical analysis was performed using parametric survival regression models. RESULTSWe found that IL-6 levels from wild-type ATTR patients were significantly elevated compared to healthy controls, while aATTRv-CM carriers and ATTRv-CM patients did not show a significant difference. IL-6 levels showed significantly higher values in increasing Gillmore classes. Univariate analyses revealed association of low IL-6 levels with cardiac decompensation and htx/death [odds ratio: 0.26 (0.09-0.72), P = 0.01] and htx/death [odds ratio: 0.15 (0.04-0.58), P = 0.006]. However, in the multivariate model, no significant improvement of risk prediction was seen for IL-6, while established prognostic factors were significantly associated with outcome. CONCLUSIONRaised IL-6 levels correlate with clinical presentation and are associated with worse outcome in ATTR-CM but do not improve stratification in addition to established risk factors.     

Idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia with elevated level of serum interleukin-6]

A 38-year-old male was admitted in January 1984 due to lymphadenopathies with hyperimmunoglobulinemia with a serum IgG level of 2,872 mg/dl. Following this, he was observed as an outpatient in regard to lymphadenopathies of unknown origin. In 1989, after the fourth lymph node biopsy he was diagnosed as having idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia. At that time his serum IgG level was 8,090 mg/dl. The elevated serum interleukin-6 (IL-6) level, up to 21.1 pg/ml, was particularly interesting, because IL-6 is involved in the oncogenesis of plasmacytoma/myeloma. The patient also had thrombocytosis, hematuria, and a serum increased level of C reactive protein which seemed to be related to the effects of IL-6 i.e. thrombopoiesis, induction of the proliferation of mesenchymal cells, and induction of the production of acute phase proteins by hepatocytes, respectively. Even though he displayed no outward symptoms before and after treatment with prednisolone and melphalan, elevated immunoglobulin levels were still present.     

An approach to mediastinal masses associated with hyperthyroidism

Benign thymic hyperplasia (BTH) is a known feature of hyperthyroidism, but is infrequently appreciated by clinicians. In most cases thymic enlargement is minimal; however, it may occasionally present as an appreciable anterior mediastinal mass. While surgical resection is a common approach to such a mass, recognition of the benign nature of BTH and its regression following treatment of hyperthyroidism would prevent a major surgical procedure. We present three cases of BTH associated with hyperthyroidism and describe our approach to this syndrome.     

Severe Churg-Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy

We report a case of severe Churg-Strauss syndrome (CSS) with mediastinal eosinophilic lymphadenopathy, with relapse after standard therapy with steroids and cyclophosphamide, subsequently treated with interferon (IFN) alpha 2b. Our report shows that mediastinal lymph nodes mimicking lymphoma may be one of the clinical manifestations of CSS. We also show that patients with CSS who are resistant to first-line therapy and for whom hypereosinophilia is thought to play an important role may benefit from treatment with IFN.     

Bilateral ankle arthritis with mediastinal lymphadenopathy: a clinician's perspective

Aim: This study is a clinician's perspective of the association of bilateral ankle arthritis with mediastinal lymphadenopathy. Method: Forty‐three patients with bilateral ankle arthritis with mediastinal lymphadenopathy were included in a 14‐month prospective follow‐up study in our hospital. Complete history, examination and investigations were carried out. Result: There were 27 female and 16 male patients. Ankle arthritis with tuberculous mediastinal lymphadenopathy was associated in 58.13%, sarcoidosis in 32.5% and 9.3% were non‐specific. The patients were clustered in the spring‐summer months. Erythema nodosum was found in 14.3%, polyarticular presentation in 25%. Thirteen out of 43 patients (28.2%) had biopsy/fine needle aspiration (FNA), of which 69.23% had histopathological evidence of tuberculosis. Conclusion: The aetiology of bilateral ankle arthritis associated with mediastinal lymphadenopathy may be tuberculosis as opposed to sarcoidosis. There is a seasonal clustering of these cases. FNA of mediastinal lymphadenopathy is a fairly safe procedure and should be carried out when feasible, for confirmation of diagnosis.     

Multipotent stromal cells skew monocytes towards an anti-inflammatory interleukin-10-producing phenotype by production of interleukin-6

Multipotent stromal cells have immunomodulatory capacities and have been used in transplantation and autoimmune diseases. One of the effects of multipotent stromal cells involves the inhibition of dendritic cell differentiation. Since interleukin-6 and interleukin-10 are known to play a role in inhibiting immature dendritic cell differentiation, we hypothesized that these cytokines may also mediate the inhibitory effect of human multipotent stromal cells in immature dendritic cell differentiation. In order to test this hypothesis monocytes were cultured with interleukin-4 and granulocyte-monocyte colony-stimulating factor in the presence or absence of culture-expanded bone marrow-derived multipotent stromal cells. Neutralization and cytokine-depletion strategies were applied to reveal the cellular source and effect of interleukin-6 and interleukin-10. Addition of multipotent stromal cells to monocyte cultures significantly reduced the generation of immature dendritic cells (CD14⁻CD1a⁺) and resulted in the generation of CD14⁺CD1a⁻ cells that displayed a significantly reduced immunostimulatory effect. We found that culture supernatants of co-cultures of multipotent stromal cells and monocytes contained higher concentrations of interleukin-6 and interleukin-10. Multipotent stromal cells produced interleukin-6 and neutralizing this interleukin-6 reversed the inhibitory effect of the multipotent cells. Interleukin-10 was not produced by multipotent stromal cells, but exclusively by monocytes after exposure to multipotent stromal cell-produced interleukin-6. In conclusion, through constitutive production of interleukin-6, multipotent stromal cells prevent the differentiation of monocytes towards antigen-presenting immunogenic cells and skew differentiation towards an anti-inflammatory interleukin-10-producing cell type.     

"Syndrome myxoma": a subset of patients with cardiac myxoma associated with pigmented skin lesions and peripheral and endocrine neoplasms

From January 1954 to December 1985 cardiac myxoma was diagnosed in 75 patients at the Mayo Clinic. The clinical presentation was typical in 70 cases and was referred to as "sporadic myxoma". Forty four other cases of cardiac myxomas (five from the Mayo Clinic) presented with a combination of distinctive clinical features and these cases are described as "syndrome myxoma". The patients with syndrome myxoma were younger (mean age, 25 vs 56 years) and had unusual skin freckling (68%), associated benign non-cardiac myxomatous tumours (57%), endocrine neoplasms (30%), and a high frequency of familial cardiac myxoma (25%) and familial endocrine tumours (14%). The two types of cardiac tumour were different (syndrome vs sporadic): atrial location, 87% vs 100%; ventricular location, 13% vs 0%; single tumour, 50% vs 99%; multiple tumours, 50% vs 1%; and recurrent tumour, 18% vs 0%. It is concluded that patients with syndrome myxoma represent a distinctive subgroup in which there are important clinical, surgical, and genetic implications. More importantly, syndrome myxoma appears to be only one expression of a much larger disease entity.     

Hilar and mediastinal lymphadenopathy with hypersensitivity pneumonitis induced by penicillin.

A 54-year-old Japanese man demonstrated a sultamicillin-induced hilar and mediastinal lymphadenopathy with hypersensitivity pneumonitis. A positive lymphocyte stimulation test for sultamicillin and a decreased CD4/CD8 ratio of lymphocytes in BAL fluid suggested that an alteration in cell-mediated mechanisms was responsible for the patient's symptoms.     

Angioimmunoblastic lymphadenopathy associated with polyarthritis.

A patient with angioimmunoblastic lymphadenopathy with dysproteinaemia is described. The patient had a severe clinical illness with a pronounced haemolytic anaemia, which followed well-established polyarthritis and gold therapy. It was accompanied by in-vitro evidence of suppressed cell-mediated immune responses and by development of serum antismooth muscle antibodies. These features are unusual and support theories that this disease has an immunological basis. Improvement occurred with prednisone and azathioprine therapy, suggesting that combined use of these agents is useful in some patients with AILD. The patient subsequently developed a lymphoma.