1/18. Treatment of EBV driven lymphoproliferation with erythrophagocytosis: 12 year follow up.This is a report of a case of Epstein-Barr virus (EBV) associated haemophagocytic syndrome in a 17 year old woman with antibody deficiency. For two years before this presentation, serology showed abnormally high titres to EBV early antigen, suggestive of persistent infection with EBV. She became acutely unwell with clinical features consistent with virus associated haemophagocytic syndrome (VAHS). histology showed lymphoproliferation with erythrophagocytosis and evidence of EBV encoded RNAs in liver, spleen, and lymph node. VAHS is often fatal, particularly when it occurs in patients with underlying immunodeficiencies. In this case, treatment with intravenous immunoglobulin, aciclovir, and alpha interferon was followed by a dramatic recovery. Twelve years later the patient remains relatively well on regular intravenous immunoglobulin.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
2/18. Fatal B-cell lymphoproliferative syndrome in allogeneic marrow graft recipients. A clinical, immunobiological and pathological study.We have studied four cases of fatal B-cell lymphoproliferative syndrome (LPS) developing among 333 patients (incidence 1.2%) treated with allogeneic bone marrow transplantation (BMT). All four patients had received a T-cell depleted graft. Onset of the first clinical symptoms (palpable lymph node enlargement in three and IgA-lambda paraproteinemia in two patients) occurred between 41 and 188 days post-BMT (median 76 days). The course of the LPS was rapidly progressive in all cases, leading to death in 2-5 weeks. The peripheral blood showed progressive pancytopenia with disproportionally high numbers of activated NK cells, apparently compensating for the T-cell deficiency. Post-mortem histological studies disclosed polymorphic B-cell proliferations, most pronounced in the lymph nodes, spleen, liver, lungs and kidneys. Lymphohemopoietic cells were of donor origin in three patients. In the fourth patient, graft failure suggested a host origin for the proliferating cells. immunophenotyping and gene rearrangement analysis revealed polyclonal proliferation in one patient, monoclonal proliferation in another patient, and an oligoclonal pattern in the other two patients. The clinical behavior of the LPS was independent of clonality. Immunohistologically, the proliferating cells showed characteristics of relatively mature B-cells in three cases, and pre-B-cell features in one case. Epstein Barr virus (EBV) serology indicated seroconversion (primary infection) in one child, and chronic active EBV infection in both adults. EBV dna as well as EBV nuclear antigen (EBNA) were detected in infiltrated tissues of all four patients. The labeling pattern on in situ hybridization suggested a replicative EBV infection comparable to that in lymphoblastoid cell lines. We conclude that EBV-associated LPS developing as a result of post-transplant immunodeficiency is a distinct clinicopathologic entity, differing from non-Hodgkin's lymphoma (including Burkitt's lymphoma) and infectious mononucleosis of the immunocompetent host.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
3/18. Pathomorphology of humoral, cellular and combined primary immunodeficiencies.Histologic, immunohistologic and electron microscopic findings in three children with primary immunodeficiencies are reported. Classical X-linked infantile agammaglobulinemia Bruton was present in case 1 (male, aged 16 years), selective cellular immunodeficiency with thrombopenia in case 2 (male, aged 2 1/2 years) and non-lymphopenic severe combined immunodeficiency in case 3 (male, aged 1 3/4 years). At autopsy, all three cases exhibited unusual types of pneumonia. In case 2 a generalized cytomegalovirus infection was present. Case 3 disclosed panmyelopathia and chronic liver lesions due to severe GvH-reaction subsequent to bone marrow transplantation. A detailed morphologic study of the immune system revealed distinct alterations in the thymus, spleen, and lymph nodes and the lymphatic tissues of the gastrointestinal tract characteristic of an immunodeficiency state, either humoral (case 1), cellular (case 2) or combined (case 3).- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
4/18. The periarteriolar lymphocyte sheath in immunodeficiency T- or B-lymphocyte area?T- and B-lymphocyte populations in peripheral lymphoid tissues occur in distinct compartments (e.g., the periarteriolar lymphocyte sheath of the splenic white pulp is a T-cell area). The authors report on two patients with severe combined immunodeficiency (SCID) and one patient with immunodeficiency after anti-T-cell treatment for rejection of a heart transplant, in which the area surrounding the central arteriole in spleen white pulp was well-populated despite T-cell deficiency (documented by, for example, severe depletion of lymph node paracortex). Immunologic phenotyping showed the B-lymphoid lineage of lymphocytes at this location. The framework in the periarteriolar area consisted of follicular dendritic cells, which are typical framework components of B-cell areas. We conclude that assessment of only conventional histopathology of the spleen in these patients leads to erroneous conclusions about the type of immunodeficiency and that immunologic phenotyping is required to document the exact nature of the deficiency.- - - - - - - - - - ranking = 2keywords = spleen (Clic here for more details about this article) |
5/18. Polyfungal systemic infections in pediatric oncology patients.Three cases of two fungal agents causing simultaneous systemic infection in immunocompromised pediatric patients are presented and the literature is reviewed. All three patients had several underlying factors that predispose to systemic fungal infections. A species of candida was identified initially as an etiologic agent in all of the three patients causing subcutaneous abscesses, urinary tract infections, fungemia, catheter exit site infection, or pneumonia. However, a few days later blood cultures grew aspergillus species in two of the three patients; in the third patient aspergillus was identified on microscopic examination of the spleen. All three patients had an associated bacteremia with either staphylococcus aureus or S. epidermidis requiring vancomycin therapy. Presence of aspergillus infection required treatment with amphotericin. Difficulties in making a definitive diagnosis of systemic fungal disease may explain paucity of reports in the literature with simultaneous polyfungal systemic infection.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
6/18. Visualization of the spleen with radiophosphate in severe combined immunodeficiency disease.This is a case report of a 12-month-old male suffering from severe combined immunodeficiency disease who demonstrated an intense concentration of MDP in the spleen. The precise mechanism for this accretion is not known and remains speculative.- - - - - - - - - - ranking = 5keywords = spleen (Clic here for more details about this article) |
7/18. Hepatic candidiasis: an increasing problem in immunocompromised patients.Hepatic candidiasis has been increasingly recognized as a variant of disseminated candidiasis in immunocompromised patients. Five leukemic patients with antemortem diagnosis of hepatic candidiasis are described, and 32 additional cases reported in the literature are reviewed. Cultures of the liver and/or spleen and blood cultures usually give negative results; histopathologic demonstration of candida organisms in tissue specimens is necessary for a definitive diagnosis. Response to conventional therapy with amphotericin b is poor, and 34.4 percent of the patients died with evidence of active fungal disease. Liposome-encapsulated amphotericin b, which has been successfully used in a limited number of patients with invasive fungal disease, may be an effective and relatively nontoxic drug.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
8/18. adult severe combined immunodeficiency and sarcoid-like granulomas with hypersplenism.A patient presented with lymphopenia, anergy, hypogammaglobulinemia and hypersplenism. Histologic examination of the spleen and lymph node revealed noncaseating sarcoid-like granulomas. Despite a significant rise in circulating lymphocytes after splenectomy there was in vivo and in vitro evidence of B- and T-lymphocyte dysfunction. A histologic picture mimicking sarcoidosis may occur in patients with immune deficiency. The granulomatous proliferation may represent an altered host response to antigen.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
9/18. The Omenn's syndrome: histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100 /T-6 Langerhans-like cells.A 7 month old female infant was affected by a rapidly fatal familial disease highly reminiscent of Omenn's syndrome. She presented with widespread eczematous lesions, hepatosplenomegaly, superficial lymphadenopathy, peripheral blood lymphocytosis, eosinophilia and hyper-IgE. An axillary lymph node was involved by a marked proliferation of T-3 /T-10-- lymphocytes admixed with S-100 /T-6 /Leu-3a /Ia reticular cells which lacked typical LC granules; cell suspension study revealed that 90%-96% of the lymph node cells were T-11 /T-3 lymphocytes characterized by low expression of Leu-3a and T-8 antigens and by high expression of Ia antigens (52%). Peripheral blood T lymphocytes exhibited a similar distribution of surface phenotypes. The patient died of interstitial pneumonia and an autopsy was performed. The thymus was markedly atrophic and completely devoid of lymphocytes. The peri-arteriolar lymphoid sheets of the spleen were poorly developed and were mainly composed of T-8 lymphocytes. The mediastinal nodes were rudimentary and were populated by T-3 /T-10 lymphocytes with low expression of Leu-3a and T-8 antigens. Our results raise the possibility that Omenn's syndrome is a peculiar primary immunodeficiency in which, despite early thymic involution, some abnormal T lymphocytes still develop in the peripheral lymphoid organs. Antigenic triggering of these cells might result in prominent proliferations of T lymphocytes and Langerhans-like cells which lead to the clinical manifestation of the disease.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
10/18. Tubulo-interstitial nephritis associated with polyomavirus (BK type) infection.We studied viral injury to the kidney in a six-year-old boy with hyperimmunoglobulin M immunodeficiency who presented with irreversible acute renal failure and eventually died after five months of dialysis. Renal biopsy at the time of his presentation revealed a predominantly tubulo-interstitial process with numerous viral inclusions that were identified as polyomavirus. urine cultures showed a massive viruria with BK-type, polyomavirus. The kidney disease was end stage, with persistence of bk virus identified by morphologic techniques and by culture. dna hybridization analysis showed virus in low concentration in the lymph nodes, spleen, and lungs. The marked viruria, the high concentration of bk virus, and the extensive distribution of viral antigen throughout the kidney all suggest that infection with bk virus was the basis of the severe renal parenchymal injury.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
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