A unique association of giant lymph node hyperplasia (Castelman's disease) and cardiac tamponade is at handed Although pleural effusions have been previously described with Castleman's disease.


A unique association of giant lymph node hyperplasia (Castelman's disease) and cardiac tamponade is at handed Although pleural effusions have been previously described with Castleman's disease, the authors believe this to be the first report of pericardial effusion and tamponade with this entity. The unravelling of effusions may be to be paid to an inflammatory syndrome sometimes seen with the plasma confined apartment variant of this disease.

Castleman's disease is a rare entity characterized on benign hyperplasia of lymph nodes, occurring most numerous commonly in the mediastinum. Clinically, it usually currents as an asymptomatic mass onward routine chest radiograph. Although this condition has been associated with returning pleural effusions, symptomatic pericardial effusion has not, to our knowledge, been described to date with this disease.

CASE REPORT



A 54-year-old woman was admitted to an outlying hospital for progressive dyspnea from one side of to the other several days. Bilateral cervical and axillary lymphadenopathy had been ready for 1 month, and painful erythema of the right breast had make knowned 1 week prior to admission. Within 12 h of presentation, the patient became hypotensive, and a comput tomographic scan of the chest showed a large pericardial effusion, as well as an anterior mediastinal mass (Fig 1) Pericardiocentesis yielded 250 ml of sanguinary fluid with immediate resolution of the one and the other the symptoms and the hemodynamic embarrassment.

The patient was transferred to our institution for further evaluation and care. about arrival, she was noted to have jugular venous distention and muffl heart wholes In addition, there was bilateral axillary, supraclavicular, and cervical lymphadenopathy. The right breast was moderately delicate and erythematous, but there was no palpable mass. A after mammogram was negative for any mass. Laboratory values were notable for mild anemia and an elevated alkaline phosphatase concentration. An echocardiogram revealed a large pericardial effusion with diastolic collapse of the right ventricle, as well as multiple bands and septa suggestive of fibrinous adhesions. Repeat pericardiocentesis yielded 550 ml of murderous fluid.

Pericardiectomy performed between the sides of a left anterior thoracotomy revealed hemorrhagic pericarditis with a thickened, inflamed pericardium that was adherent to the epicardium in several areas and thick fibrinous bands and septa, indicative of an early constrictive proces There was a large, hard, yellowish anterior mediastinal mass overlying the ascending aorta. Part of this mass was remov in continuity with the pericardium. The anterior pericardium was resect to the phrenic endurance on each side, from the diaphragm inferiorly to the aortic reflection superiorly. Cytology and cultivations of the pericardial fluid were negative, and histologic thought of the pericardium suggested nonspecific fibrinous pericarditis. The histologic diagnosis of the resect mediastinal mass was thymic tissue with acute and chronic inflammation and hyaline deposition. A right axillary lymph node biopsy specimen obtained several days later showed sheets of plasma confined apartments widely separating atrophic germinal center (Fig 2) Immunoperoxidase stain indicateed a polyclonal, rather than a monoclonal, plasma solitary abode; squalid population. This pattern was consistent with a mixed histologic form of Castleman's disease as well. Retrospective review of the thymic tissue showed changes consistent with the diagnosis of Castleman's disease. The patient was discharged with no specific therapy for the lymph node hyperplasia and is well 18 month later.

DISCUSSION

Castleman first described the entity of localized mediastinal lymph node hyperplasia resembling thymoma in 1956[1] and later characterized the disease further in a review of 81 cases.[2] The main lesion was located in the mediastinum in 70 of these patients; however, extrathoracic sites of origin were noted in 8 cases. Histologically, the disease waits to occur in two distinct forms. The hyaline-vascular image accounts for approximately 90 percent of cases and is characterized by means of atrophic germinal centers separated by the agency of large areas of extensive capillary proliferation and fibrosis. The plasma lonely dwelling type, which represents the remaining 10 percent of cases is identified by the agency of sheets of mature plasma solitary abode; squalids in the interfollicular tissue and the port of normal to large follicle center While the hyaline-vascular token tends to be diagnosed in asymptomatic individuals by the agency of routine chest roentgenogram, the plasma confined apartment type is often associated with a clinical syndrome that consists of febrile affection fatigue, and peripheral lymphadenopathy. Laboratory abnormalities seen with this syndrome may include mild anemia, elevated sedimentation rate, hyperglobulinemia, hypoalbuminemia, and elevated alkaline phosphatase concentration. Because of its association with these clinical markers of an acute inflammatory answer it has been suggested that the plasma small cavity type represents an earlier, more active stage of the disease proces while the hyaline-vascular token represents a later stage.[2] The underlying etiology of the disease remains speculative.

...