Nephrotic syndrome associated with angiofollicular lymph node hyperplasia

A women presented at the age of 22 with anemia, hepatosplenomegaly, polyclonal hypergammaglobulinemia and a mediastinal shadow. At thoracotomy she had enlarged mediastinal lymph nodes which displayed histological features typical of angiofollicular hyperplasia. Marrow plasmacytosis was present. She developed diabetes mellitus at the age of 29 yrs after she had received oral steroid treatment for one year. The nephrotic syndrome supervened another year later. Her kidneys were enlarged before the onset of diabetes. The glomerular changes included a marked increase of mesangial matrix and segmental hypercellularity. The association of the nephrotic syndrome and angiofollicular lymph node hyperplasia is extremely rare and their interrelation remains enigmatic.     

Extramedullary plasmacytoma associated with angiofollicular lymph node hyperplasia

We report a case of hyaline-vascular type of angiofollicular lymph node hyperplasia involving the left pulmonary lymph nodes and lung. It clinically presented as an obstruction of the left lower lobe main bronchus secondary to the development of an extramedullary plasmacytoma in the lesion. An immunoperoxidase stain revealed a monoclonal IgG-lambda pattern of the tumor, contrasting with the polyclonal plasma cell population of the angiofollicular lymph node hyperplasia. This case demonstrates the neoplastic potential of angiofollicular lymph node hyperplasia.     

Multicentric angiofollicular lymph node hyperplasia: a clinicopathologic study of 16 cases

A clinicopathologic analysis of 16 cases of multicentric angiofollicular lymph node hyperplasia (MAFH) was performed. Histologically, the disease was characterized by recognizable lymph node architecture that was at least partially intact, by paracortical hyperplasia with prominent vascular proliferation, and by numerous evenly distributed, apparently benign germinal centers of various types, usually including some typical hyaline-vascular centers. At the onset of the disease, 12 patients had the plasma cell (PC) type of MAFH, three patients had the hyaline-vascular (HV) type, and one patient presented with PC and HV types at separate sites. Transitions between the PC and HV types were observed in two cases. Immunologic studies demonstrated polyclonal populations of plasma cells in the lymph nodes of all patients and the absence of suppressor T lymphocytes in the one patient tested. Clinically, the patients had constitutional symptoms, multicentric lymphadenopathy, hepatosplenomegaly in many cases, and abnormal laboratory findings, including anemia, polyclonal hypergammaglobulinemia, and bone marrow plasmacytosis. The 16 patients were placed in four different clinical groups based on presentation and course: stable disease, chronic relapsing disease, aggressive disease, and development of malignant lymphoma. Ten of the 16 patients died (median survival, 26 months; range, eight to 170 months). Multicentric angiofollicular lymph node hyperplasia appears to be a variant of classic angiofollicular lymph node hyperplasia (Castleman's disease) and is associated with significant morbidity and mortality.     

Membranous nephropathy. Its association with multicentric angiofollicular lymph node hyperplasia.

Multicentric angiofollicular lymph node hyperplasia of the plasma cell type with systemic manifestations developed in a 51-year-old man. One year later, the nephrotic syndrome due to typical immune complex mediated membranous nephropathy developed. Elevated titers to Epstein-Barr viral antigens suggesting reactivation of a latent infection were present. The patient has required conservative medical management only. The association of membranous nephropathy with angiofollicular lymph node hyperplasia lends further evidence to the postulate that the disorder is a manifestation of chronic immune stimulation by a foreign antigen.     

Hyaline-vascular variant of angiofollicular lymph node hyperplasia with systemic manifestations and response to corticosteroids

We report two cases of angiofollicular lymph node hyperplasia of the hyaline-vascular type. The patients were atypical in having systemic complications, which are much more commonly seen in the plasma cell variant of this disorder. In each case, the diagnosis was established some years after the initial presentation. Both patients presented with unusual systemic manifestations. The exact mechanism underlying the associated features is unknown, but these cases provide some evidence that the disease is primarily a vascular proliferative or inflammatory disorder. All manifestations of the disease responded to the administration of prednisolone with rapid reduction of dosage to less than 10 mg daily. Maintenance therapy may be required to prevent relapse.     

Multicentric giant lymph node hyperplasia

A patient with retroperitoneal and axillary lymphadenopathy and splenomegaly was demonstrated histologically to have the hyaline vascular type of giant lymph node hyperplasia, with plasma cell infiltrates in each region. The abdominal lesions were not surgically resectable and did not respond to radiotherapy. The clinical findings included polyclonal gammopathy, high cold agglutinin titers, neuropathy, and bilateral papilledema. All of these abnormalities have persisted three years since the initial diagnosis.     

Multicentric angiofollicular lymph node hyperplasia associated with a solitary osteolytic costal IgG lambda myeloma. POEMS syndrome in a South American (Paraguayan) patient

We report the case of a 46-year-old woman presenting with a disorder characterized by the association of multicentric Castleman's disease, organomegaly, a solitary IgA lambda myeloma with lytic bone lesions but no serum monoclonal peak, peripheral neuropathy and skin changes. This unusual association resulted in an incomplete form of that peculiar multisystem syndrome described under a variety of names: Crow-Fukase syndrome, Shimpo's syndrome, Takatsuki's syndrome, PEP syndrome or POEMS syndrome. The possible relationship between localized and multicentric Castleman's disease with myeloma or extramedullary plasmacytoma on one hand and on the other hand with polyneuropathy and the other symptoms of the POEMS syndrome is discussed.     

Histopathological study of a case of systemic angiofollicular hyperplasia (Castleman's disease)

This report describes the nodal and hepatic lesions observed in a patient with generalized disorders that had been histologically diagnosed as a systemic angiofollicular hyperplasia. The diagnostic morphological findings were observed in the nodes of the axilla and were represented by diffuse marked plasmacytosis, prominence of the germinal centres, preservation of the architecture with a reactive proliferation of blood vessels and fibrous tissue in interfollicular areas.     

Angiofollicular lymph node hyperplasia (Castleman)

Six personally observed cases of this lesion are reported and the literature is reviewed. One hundred and thirty-four cases have been reported to date. The lesion has occurred in many sites, but is commonest in the thorax (60%), abdomen (11%), neck (14%), and axilla (4%).Ninety per cent of cases are symptomless or have only the pressure symptoms, 10% have systemic signs, namely, fever, raised ESR, anaemia, cured by removal of the tumour. These cases have a slightly different histology. All recorded cases have been benign. Microscopically the lesion is follicular but instead of germinal centres the follicles have one or more thick-walled arteries and often some surrounding histiocytes. Increased numbers of vessels occur between the follicles, whilst sinuses are absent. These vessels have thick, cellular walls like post-capillary venules. Sometimes they become hyaline.The cases with symptoms have true reaction centres as well as a few intrafollicular vessels. Between the follicles there are increased numbers of vessels and, in addition, there may be collections of plasma cells or eosinophils.     

T and B lymphocyte populations in human normal lymph node, regional tumour lymph node and inflammatory lymph node.

We have determined the T and B lymphocyte subpopulations in normal human lymph nodes. The lymphocyte profile was the same irrespective of the anatomical distribution and was similar to that found in peripheral blood with identical T and B cell values but with a lower Fc and a higher C3-receptor-bearing lymphocyte subpopulation. This pattern showed a marked change in the regional nodes of patients with mammary carcinoma and nodes draining a variety of other solid tumours, with a fall in T and pronounced elevation of B, Fc and C3 cells but with a persistence of C3 predominance. The lymphocyte profile found in tonsils and nodes draining inflammatory foci was a similar but further exaggeration of the tumour node pattern, with reversal of T and B cell ratios. The T and B lymphocyte percentages in the peripheral blood of patients with clinically localized breast cancer are identical to those of the healthy controls. Different Fc/C3 subsets exist in peripheral blood and lymphoid structures and probably represent a differential functional heterogeneity. Proximity of tumour to the draining node modified this profile.     

Variably acid-fast bacteria in vivo in a case of reactive lymph node hyperplasia occurring in a young male homosexual

Ziehl-Neelsen (acid-fast) and Giemsa-stained microscopic sections of enlarged cervical lymph nodes obtained from a 39 year-old male homosexual with acquired immunodeficiency syndrome (AIDS) were studied for the presence of cell wall-deficient bacteria. Intra- and extra-cellular, variably acid-fast forms were observed within the lymph nodes showing "benign reactive lymph node hyperplasia." Similar-appearing variably acid-fast bacteria have previously been demonstrated in Kaposi's sarcoma, a malignancy which may ultimately develop in gay men with AIDS. It is hypothesized that these microbes observed in vivo may be pathogenic in immunodepressed states, such as the acquired immunodeficiency syndrome.     

Massive hyperplasia of marginal zone B-cells with clear cytoplasm in the lymph node: a case report

An enlarged axillary lymph node from a 63-year-old woman showed proliferating marginal zone B-cells arranged in a vague nodular pattern or in band-forming aggregates throughout the cortex. Marginal zone B-cells, which also infiltrated the adjacent fatty tissue, had round or slightly indented nuclei of medium size and a moderate amount of clear cytoplasm. Immunohistochemically, these cells were CD20+, CD79a+, Bcl-2+, sIgD-, CD5-, CD10-, CD21-, CD23-, CD45RO-, Bcl-6-, and cyclin D-. A portion of the cells were sIgM- and CD43-positive. The polytypic nature of these cells was demonstrated by immunohistochemistry and polymerase chain reaction. Systemic bacterial infection appears to be the cause of marginal zone B-cell hyperplasia. This unusual marginal zone B-cell hyperplasia should be differentiated from low-grade B cell lymphomas, and particularly from nodal marginal zone B-cell lymphomas.