1/23. Disseminated BCG infection following bone marrow transplantation for X-linked severe combined immunodeficiency.An 8-month-old boy with X-linked severe combined immunodeficiency (XSCID) developed disseminated bacille Calmette-Guerin (BCG) infection following BCG vaccination at birth. He initially presented with an abscess at the site of BCG vaccination and was begun on three-drug antituberculous treatment (rifampicin, isoniazid, and pyrazinimide). Dissemination was subclinical prior to a human leukocyte antigen (HLA)-identical bone marrow transplant (BMT) from his sister, following which he presented with an acute erythroderma. A skin biopsy specimen revealed granulomas with epithelial histiocytes and giant cells in the reticular dermis, and numerous acid-fast bacilli (AFB) were present on Ziehl-Nielsen stain. A diagnosis of disseminated BCG disease was made. Despite the addition of a fourth antituberculous agent, ethambutol, he did not recover and developed numerous skin abscesses over the following weeks. Examination of pus from these lesions demonstrated numerous AFB. clarithromycin was added as a fifth antituberculous agent. Despite five-drug antituberculous therapy and monthly intravenous immunoglobulin infusions, recurrent abscesses containing AFB developed intermittently until 7 months posttransplant. At follow-up 1 year post-BMT he showed good general physical improvement. All abscesses had healed with scarring, and no further skin lesions had occurred.- - - - - - - - - - ranking = 1keywords = immunodeficiency (Clic here for more details about this article) |
2/23. A preterm baby with Omenn syndrome.A preterm baby born with scaly skin who later developed recurrent infections and was subsequently diagnosed to have Omenn syndrome is presented. CONCLUSION: Any baby with ichthyotic skin and recurrent infections should have immunodeficiency considered in the differential diagnosis.- - - - - - - - - - ranking = 0.2keywords = immunodeficiency (Clic here for more details about this article) |
3/23. Erythrodermic cutaneous T-cell lymphoma with disseminated pustulosis. Production of high levels of interleukin-8 by tumour cells.Interleukin (IL) -8 is a neutrophil chemoattractant cytokine with proinflammatory and growth-promoting activities, which is involved in the pathogenesis of several inflammatory diseases. It is found in high amounts in lesional biopsies of pustular diseases such as psoriasis and palmoplantar pustulosis. We report a 50-year-old woman with a 10-year history of erythroderma with disseminated pustulosis. skin biopsies showed an epidermotropic infiltrate composed of atypical CD4 CD8 lymphocytes with numerous admixed neutrophils. Peripheral blood flow cytometric analysis revealed a major clonal subset of CD3 CD4 CD8 T-cell receptor Vbeta22 atypical lymphocytes. Bone marrow biopsy, lymph node biopsy and computed thoracoabdominal tomography were normal. Serologies for human T-cell lymphotropic virus type I and human immunodeficiency virus were negative. Our patient's status deteriorated despite topical (nitrogen mustard, psoralen plus ultraviolet A) and systemic (interferon, methotrexate, multiagent chemotherapy) treatments, and she finally died. We showed that our patient's peripheral blood lymphocytes (PBL) spontaneously produced high amounts of IL-8. In contrast, PBL of patients with classical sezary syndrome produced lower amounts of IL-8. The production of IL-8 by tumour T cells could explain this unusual clinical and histopathological presentation of cutaneous T-cell lymphoma as disseminated pustulosis.- - - - - - - - - - ranking = 0.2keywords = immunodeficiency (Clic here for more details about this article) |
4/23. Treatment with cyclosporin A in a patient with Omenn's syndrome.Unless treated with haematopoetic stem cell transplantation, Omenn's syndrome, a rare variant of severe combined immunodeficiency, is associated with a fatal outcome. We describe a male infant showing all the typical features of Omenn's syndrome, who was successfully treated with cyclosporin A to improve clinical condition prior to haematopoetic stem cell transplantation.- - - - - - - - - - ranking = 0.2keywords = immunodeficiency (Clic here for more details about this article) |
5/23. A case of T-cell lymphoma with Sezary cells in the blood and bone marrow accompanied by peripheral T and B lymphocytosis.A case of T-cell lymphoma occurred in a man, aged 66, whose symptoms started as itching and the appearance of 1-2 cm indurated plaques with central pustules on the skin of the trunk and face. A few months later generalized lymphomas and splenomegaly were present. Investigations of the lymphocytic subpopulations in situ in fresh frozen sections of lymphomas and skin showed only T cells. Samples of blood and bone marrow disclosed lymphocytosis and Sezary cells. Both T and B lymphocytes were definitely increased in the blood. The value of tissue studies in analyzing lymphocytic subpopulations is emphasized as a differential diagnostic aid in classifying lymphomas. A relationship between the T-cell neoplasia and the T and B lymphocytosis to mycosis fungoides and the sezary syndrome is discussed.- - - - - - - - - - ranking = 0.00074657326195528keywords = aid (Clic here for more details about this article) |
6/23. Continuous- and semicontinuous-flow blood centrifugation systems: therapeutic applications, with plasma-, platelet, lympha-, and eosinapheresis.blood centrifugation, with a continuous-flow or semicontinuous-flow system, was used in the treatment of 17 patients with various hematologic disorders. Total plasma exchange (TPE) controlled symptoms of hyperviscosity and arrested bleeding in three patients. In two patients, TPE made is possible to perform urgent surgical procedures. It was a very valuable adjuvant in the treatment of one patient with a factor viii inhibitor. Eosinapheresis was not of significant value in the therapy of syndromes associated with eosinophilia. Lymphapheresis significantly reduced peripheral lymphocytosis, aiding in the chemotherapeutic control of chronic lymphosarcoma cell leukemia in one patient. It was of questionable value in the maintenance therapy of two patients with sezary syndrome. Plateletapheresis significantly reduced peripheral platelet count in thrombocytosis, making it possible to minimize the chemotherapeutic dose and permitting urgent surgical procedures in two patients.- - - - - - - - - - ranking = 0.00074657326195528keywords = aid (Clic here for more details about this article) |
7/23. Omenn syndrome: therapeutic effects of cyclosporin.Omenn syndrome is a severe combined immunodeficiency with features of generalised erythroderma alopecia and evidence of Th2 inflammation (eosinophilia and raised IgE). We describe a differential effect of 2 calcineurin inhibitors, cyclosporine A (CsA) and tacrolimus, with CsA rapidly improving the erythroderma and lymphocytosis but tacrolimus having little effect.- - - - - - - - - - ranking = 0.2keywords = immunodeficiency (Clic here for more details about this article) |
8/23. Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive.A 4-month-old male infant had a 2-month history of an exfoliative erythroderma and alopecia. Recurrent mucosal infections, diffuse lymphadenopathy, hepatosplenomegaly, lymphocytosis and eosinophilia, anemia, and failure to thrive later developed. Investigation revealed a combined immunodeficiency with T cells of an unusual phenotype in his peripheral blood, skin, and lymph nodes. Our patient's clinical manifestations most closely resemble Omenn's syndrome, a rare form of autosomal recessive combined immunodeficiency.- - - - - - - - - - ranking = 2.3248931909278keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
9/23. Erythroderma as the initial presentation of the acquired immunodeficiency syndrome.A 32-year-old black homosexual man, seronegative for human immunodeficiency virus antibody, presented with erythroderma. His peripheral blood was significant for circulating Sezary-like cells bearing the CD8( ) phenotype. Eighty-eight percent of his lymphocytes were CD8( ) as well. He seroconverted 3 months after the initial presentation. We conclude that erythroderma was the presenting sign of the acquired immunodeficiency syndrome.- - - - - - - - - - ranking = 2.6061164886597keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
10/23. hypereosinophilic syndrome with unusual cutaneous manifestations in two men with hiv infection.We report two men with human immunodeficiency virus infection who had unusual cutaneous manifestations of hypereosinophilic syndrome, exfoliative erythroderma, and linear flagellate plaques. In the first patient the cutaneous disease was the initial manifestation, and in the second the skin was the only organ affected. We also describe a favorable response to systemic corticosteroids and to psoralen with UVA phototherapy in one patient.- - - - - - - - - - ranking = 0.2keywords = immunodeficiency (Clic here for more details about this article) |
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