Castleman disease

Abstract

Castleman and Towne described a disease presenting as a mediastinal mass resembling thymoma. It is also known as "giant lymph node hyperplasia", "lymph node hamartoma", "angiofollicular mediastinal lymph node hyperplasia", and "angiomatous lymphoid hyperplasia". The pathogenesis is unknown, but the bulk of evidence points toward faulty immune regulation, resulting in excessive B-lymphocyte and plasma-cell proliferation in lymphatic tissue. In addition to the mediastinal presentation, extrathoracic involvement in the neck, axilla, mesentery, pelvis, pancreas, adrenal gland, and retroperitoneum also have been described. There are 2 major pathologic variations of Castleman disease: (1) hyaline-vascular variant, the most frequent, characterized by small hyaline-vascular follicles and capillary proliferation; and (2) the plasma-cell variant, in which large lymphoid follicles are separated by sheets of plasma cells. The hyaline-vascular cases usually are largely asymptomatic, whereas the less common plasma-cell variant may present with fever, anemia, weight loss, and night sweats, along with polyclonal hypergamma-globulinemia. Castleman disease is a rare lymphoproliferative disorders. Few cases have been described world widely. In this article we reviewed the classification, pathogenesis, pathology, radiological features and up to date treatment with special emphasis on the role of viral stimulation, recent therapeutic modalities and the HIV-associated disease.

Clinical and morphological features of different types of Castleman’s disease

“…Данный вирус чаще выявляется у больных БК, серопозитивных по ВИЧ (по данным Н. El-Osta и соавт. [14], в 100% случаев), одна-ко описаны случаи инфицированности HHV-8 и у сероне-гативных по ВИЧ пациентов [15][16][17][18][19][20][21].…”

“…Морфологическая картина в ЛУ напоминала тако-вую при ПКВ, но имела отличие: в мантийной зоне содер-жатся крупные плазматические клетки -плазмобласты с обильной цитоплазмой и заметными одним или несколь-кими ядрышками [22][23][24]. Поэтому в настоящее время авторы предпочитают классифицировать БК в соответ-ствии с гистопатогенным типом на ГВВ, ПКВ, МБК и ас-социированный с HHV-8 (плазмобластный) варианты (в противоположность традиционной уницентрической/ мультицентрической модели) [15][16][17][18][19][20][21].…”

Intrathoracic Castleman disease

“…It was reported that calcification might be observed in 5%-10% of patients. On the other hand, multicentric lesions can typically be observed in the form of bilateral hilar and mediastinal lymphadenopathy, centrilobular nodular opacity, and rarely ground glass, consolidation, and bronchiectasis (10,12). The value of PET in diagnosis is disputed, as it is not able to distinguish between malignant disease and CD.…”

“…Hilar CD can be confused with lung cancer. It is rarely located in pleural, pericardial, intercostal, and intrapulmonary areas (5,10,11). Thorax CT scans identified 3 presentations for the localized form of the disease: solitary noninvasive masses were most prevalent (50%), followed by infiltrative masses accompanied by lymphadenopathy (40%) and lymphadenopathy with no masses (10%).…”

“…An accepted approach for both diagnostic and therapeutic reasons is surgical excision of these lesions (15). The patients who underwent surgical excision showed that surgery is almost always curative for either the hyaline vascular type or plasma cell localized CD (8,10,16). Due to the invasive nature of lymphadenopathy in multicentric diseases, complete surgical excision is rarely possible (17).…”