Fibrillary glomerulonephritis related to serum fibrillar immunoglobulin-fibronectin complexes

Fibrillary glomerulonephritis is a disease of uncertain origin and pathogenesis characterized by nonamyloidotic fibrils in glomeruli. We report immunohistological, immunochemical, and biochemical studies of a serum fibrillar cryoprecipitate obtained from a patient with fibrillary glomerulonephritis, that formed on prolonged storage at 4 degrees C. By Western blot and amino acid sequence analysis, the cryoprecipitated fibril components consisted of immunoglobulins, heavy chains gamma and mu, light chains kappa and lambda, and fibronectin, similar to the proteins identified by immunofluorescence and immunoelectron microscopy in the glomerular fibrils. These findings support the hypothesis that serum precursors may be the source of the fibrillar deposits and suggest a role for immunoglobulin-fibronectin complexes in the pathogenesis of fibrillary glomerulonephritis.     

Nasopharyngeal Castleman's disease

A 19-year-old male was admitted to our clinic because of nasal obstruction and intermittent postnasal drip of 3 to 4 years' duration. Physical examination revealed a wide-based, smooth-surfaced nasopharyngeal tumor which was suspected to be a nasopharyngeal angiofibroma after examination of computed tomographic scans and an angiogram. However, after the tumor was removed by surgical excision via a transpalatal approach, the pathologic examination revealed Castleman's disease of the hyaline-vascular type. There was neither evidence of recurrence nor nasal problems at 4 years' follow-up. To our knowledge, Castleman's disease, or angiofollicular lymph node hyperplasia, may present as a local or generalized tumor-like condition, usually in the chest or abdomen, and may involve both the lymph nodes and non-nodal tissues. A review of previous articles reveals that there has not been any report of Castleman's disease found in the nasopharynx. This rare disorder is presented and discussed.     

Castleman's disease. A case report

Angiofollicular lymph node hyperplasia or Castleman's Disease (CD) is a rare lymphoproliferative disorder that manifests itself as a local or generalized tumor-like condition affecting both lymph nodes and non-nodal tissues, usually in the chest and abdomen. Hyaline vascular and plasma cell types have been identified histologically. A new clinical form of CD with multisystemic involvement has been defined as multicentric Castleman's disease. It is very rare in childhood. In this paper we present an eight-year-old boy with multicentric Castleman's disease.     

Castleman's disease restricted to the infratemporal fossa

Giant lymph node hyperplasia (Castleman's disease) is usually reported as a solitary mediastinal tumour, although involvement of other anatomical sites and a multicentric form have been reported. We describe a rare case of Castleman's disease due to its localisation (the left infratemporal fossa) and histology (plasma-cell variant). A brief review of the main clinico-histological characteristics of Castleman's disease is also presented.     

Retroperitoneal Castleman's disease. Presentation of a clinical case

Castelman disease (CD) is a rare lymphadenopathy that usually presents as a solitary, slow growing mass; its etiology is still unknown. The authors described a case of CD localized in the retro-pancreatic space. A 33-year old patient, female, underwent abdominal ultrasonography because of upper right abdominal pain. The ultrasound showed gallbladder stones and a mass of 4 cm in diameter behind the pancreatic head, modifying the shape of the inferior vena cava. The patient underwent surgical excision and histological examination showed a hyaline type of CD. In the 70% of the cases, the disease is located in the mediastinum, only in 14% of the cases in the retroperitoneal space. The case we report is a hyaline type of CD that is the most frequent histological type, constitutes 90% of the cases, occurs usually in the young population and after surgical excision has a very good prognosis. Different is the clinical behaviour of the plasma cell type of CD that has an aggressive and often fatal clinical outcome. The patient we treated is still free of any symptoms one year after the operation.     

Rare origin of a retroperitoneal mass: Castleman's disease

Castleman's disease (angiofollicular hyperplasia of the lymphatic nodes) can exceptionally appear as a retroperitoneal mass of difficult differential diagnosis relative to other malignant retroperitoneal masses. Because of its rarity, one case report of a retroperitoneal mass with histologic study corresponding to Castleman's disease is contributed. A revision of the different histologic varieties of Castleman's disease, specific treatment and prognosis is included.     

Fibrillary structures in tumor myoblasts

An electron microscope study was made of fibrillar structures in various types of cell elements in mice rhabdomyosarcoma. Different stages of myofibril development were followed in such differentiated cell elements as large spindle cells, large round cells and middle spindle cells. In the former two cells, the evolution has riched a stage of bundles of myofibrills with Z-like bands. The highest degree of myofibril maturation occurs in multinuclear myosymplasts. The occurrence of myofibrils being at different stages of evolution within the same cells is characteristic of tumor myoblasts in addition to an excentric unipolarity of cell differentiation and atypical structure of Z-brands. Cytotypical differentiation (myofibrillogenesis in uninuclear cells) may be the case only in those types of rhabdomyosarcoma cell elements which are able to undergo histottypical differentiation (fussion and formation of multinuclear myosymplasts).     

Isolated leptomeningeal Castleman's disease with viral particles in the follicular dendritic cells

To our knowledge, five cases of Castleman's disease involving only the central nervous system have been reported previously. We report a sixth case, which occurred in a 47-year-old woman with a 3-month history of headaches and a large superior frontal lobe mass on neuroimaging. Excisional biopsy revealed confluent lymphoid nodular areas with multiple well-developed germinal centers surrounded by concentrically layered proliferations of small B lymphocytes typical of Castleman's disease. Ultrastructural study found 100-nm virallike particles within follicular dendritic cells as well as intercellular spaces. These particles were suggestive of a D-type retrovirus. The patient underwent postoperative radiotherapy and was neurologically normal 3 months after surgery.     

Plasma cell interstitial pneumonia as a manifestation of multicentric Castleman's disease

A 12-year-old boy was admitted to our hospital because of abnormal shadows on a chest radiograph, slight fever, and superficial lymphadenopathy. Laboratory examination showed anemia (Hb 9.9 g/dl) and hyperimmunoglobulinemia (IgG 5469 mg/dl) without M protein. A chest CT scan showed bilateral diffuse shadows and bilateral hilar lymphadenopathy. Biopsy specimens of an inguinal lymph node and a lung showed many lymphoid follicles with germinal centers, and marked infiltration of mature plasma cells in the interfollicular area without destruction of follicular structures. The polyclonality of the plasma cells was confirmed by immunohistochemistry. The patient was not treated because these results excluded malignant disease and he was asymptomatic. At the age of 17 years, he was admitted to our hospital again because of dyspnea and a tendency to bleed. Interstitial pneumonia, hyperimmunoglobulinemia (IgG 13900 mg/dl), and anemia (Hb 6.6 g/dl) were found, along with thrombocytopenia (2.5 x 10(4)/mm3) and proteinuria. The serum interleukin-6 level was high: 177 pg/ml. Bronchoalveolar lavage fluid contained many plasma cells. Therapy with corticosteroids and immunosuppressant medication was effective. Our diagnosis was plasma cell interstitial pneumonia as a manifestation of multicentric Castleman's disease.     

A transitional variant of Castleman's disease presenting as a chylous pleural effusion

Castleman's disease is an uncommon clinicopathologic entity that results in unregulated growth of lymphoid tissue. It may present as benign involvement of one lymph node group or as multicentric disease with serious systemic symptoms. Pleural effusions are an uncommon manifestation of Castleman's disease. We present a patient with Castleman's disease who initially presented with a chylous pleural effusion.     

Tropism of human herpesvirus 8 for peripheral blood lymphocytes in patients with Castleman's disease

Multicentric Castleman's disease (MCD), also called multicentric angiofollicular lymphoid hyperplasia, is a systemic lymphoproliferative disorder causing fever, lymphadenopathy and splenomegaly. Recently, Kaposi's sarcoma-associated herpesvirus/human herpesvirus 8 (KSHV/HHV-8) DNA sequences have been detected in cases of MCD. We examined HHV-8 DNA sequences in the peripheral blood mononuclear cells (PBMCs) of two HIV-negative patients with MCD and in PBMCs and the lymph node of a HIV-negative patient with localized Castleman's disease (LCD) by the polymerase chain reaction. The novel sequences were detected in all DNA samples. Furthermore, the sequences were detected in only the CD19+ B-lymphocyte fraction of the patient with LCD as previously reported. However, the sequences were detected in CD19+ B-lymphocyte and CD2+ T-lymphocyte fractions of two patients with MCD. These results suggest that HHV-8 has tropisms for both B lymphocytes and T lymphocytes in Castleman's disease.     

Castleman's disease--difficult diagnosis

A case of Mb. Castleman of the localized plasma cell type is reported. This disease expresses several symptoms from different organ systems and therefore an extensive investigation program is often performed. Diagnosis is possible through consideration of all clinical components at the same time: Refractory anaemia, high and refractory SR, weight loss, B-symptoms, but at the same time a relatively good health. CT-scan-demonstration of a localized tumour is an important clue. Histopathologically, the tumour shows vascular hyperproliferation and plasmacytosis of varying maturation. Immunophenotyping of the plasma cells and immunoblasts usually reveals a polyclonal population. Needle biopsies from several regions may be necessary to detect the polyclonality, because monoclonality is often widespread locally in the tumour. HHV8 is correlated to the multicentric PC-type of Mb. Castleman. However, no HHV8 was found in this case.