1/10. Idiopathic CD4 T cell lymphocytopenia evolving to monoclonal immunoglobulins and progressive renal damage responsive to IL-2 therapy.Idiopathic CD4 T cell lymphocytopenia was unexpectedly detected in a 33-year-old, otherwise healthy young woman with no hiv or other viral infection, autoimmune, or neoplastic disease or increased susceptibility to infection. CD4 T cell levels were 60-140/microl over a 3.5-year period. Following an uneventful pregnancy, the patient developed anemia and interstitial nephritis associated with a plasma cell dyscrasia with a monoclonal IgA gammopathy and a shifting immunoglobulin pattern that included IgG and IgA monoclonal proteins and increased urinary light chains. Osteolytic lesions were never detected and bone marrow aspirations revealed up to 10% atypical plasma cells. Various therapies often used in treating multiple myeloma only temporarily controlled the increasing renal damage. IL-2 therapy of 600,000 to 1 million units subcutaneously daily resulted in increased CD4 T cells to normal levels, a decrease in the gammopathy, a return of renal function, energy, and weight gain, and apparently normal health status sustained for 2 years. The findings are compatible with a potentially fatal but nonmalignant immunoregulatory disorder that can be controlled by IL-2 administration.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
2/10. Angio-immunoblastic lymphadenopathy. diagnosis and clinical course.The clinical and pathologic findings in 24 patients with "angio-immunoblastic lymphadenopathy with dysproteinemia" (AILD) are presented. The patients' ages ranged from 44 to 80 years, with a median age of 68 years. The disease has an acute onset. In many respects, the clinical presentation is suggestive of malignant lymphoma. Generalized lymphadenopathy was always present. hepatomegaly was found in 20 patients, splenomegaly in 17, constitutional symptoms in 20 and skin rashes in nine. Twenty patients had anemia, with positive Coombs' test in eight of 14 tested. Polyclonal hypergammaglobulinemia was found in 17 of 22 patients. Two patterns of evolution were recognizable: (1) long survival (24 to 67 months) without treatment or after the administration of intensive combination chemotherapy; and (2) rapid progression (one to 19 months) regardless of the treatment given. Sixteen patients died; postmortem examination in 10 cases showed the cause of death to be attributable to severe infection in eight patients, to renal disease in one and to cardiovascular disease in one. No evidence of malignant lymphoma was seen in any of these autopsies. Histologically, the disease is systemic, with specific lesions in the lymph nodes. The spleen, liver, bone marrow, skin and lung are also involved, but the changes are less characteristic than in the lymph nodes. In the patients in whom sequential biopsies were performed, a trend toward restoration of the nodal architecture was observed. AILD is a clinical-pathologic entity in a spectrum of yet to be defined immune reactions. The clinical, laboratory and pathologic manifestations of AILD are consistent with an autoimmune disorder, in which a deficiency of the T-cell regulatory functions probably predisposes to an abnormal proliferative and autoaggressive reaction of the B-cell system. Surgical staging procedures do not appear to be indicated. Intensive cytotoxic treatment may be hazardous in some patients, precipitating their death, but long survival after such therapy has been observed in others. Supportive therapy and small doses of steroids appear to be a safer therapeutic approach.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
3/10. Increased expression of c-maf in pure red cell aplasia secondary to plasma cell dyscrasia.Th2 dominancy in the peripheral T helper (Th) cell subsets were reported to be involved in the pathogenesis of pure red cell aplasia (PRCA). We encountered a PRCA case secondary to plasma cell dyscrasia that showed Th2 dominancy at the relapse of PRCA. Increased expression of c-maf, a transcriptional factor which induces Th2 differentiation of naive T-cells, and elevated expression of interleukin (IL)-4 were observed in the rna derived from patient's bone marrow at relapse of PRCA. Following the administration of methylprednisolone which improved PRCA, normalization of Th1/Th2 ratio and decreased expression of c-maf and IL-4 were observed, which suggests that the upregulation of c-maf might have played a role in the pathogenesis of PRCA secondary to plasma cell dyscrasia.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
4/10. association of von Willebrand's disease with plasma cell dyscrasia and gastrointestinal angiodysplasia.A patient with acquired von Willebrand's disease (type I) had both plasma cell dyscrasia and angiodysplasia of the intestine. Based on examination of the components of F VIII/von Willebrand's factor (VWF) and the results obtained following the administration of cryoprecipitate and DDAVP (1-desamino-8-D-arginine-vasopressin), it was suggested that the production and release of VIII/VWF was normal and the rapid disappearance from the circulation was related to adsorption by abnormal cells in the bone marrow. The relation of acquired von Willebrand's disease, plasma cell dyscrasia and angiodysplasia is discussed and the literature reviewed. Our patient is added to three previous reported cases of such an association of these three disorders.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
5/10. Chronic myelomonocytic leukemia with polyneuropathy and IgA-paraprotein.A 65-year-old woman had chronic myelomonocytic leukemia with peripheral neuropathy and IgA paraprotein with kappa type light chain. plasma cells with "flaming" cytoplasma were seen in the bone marrow specimens. The findings suggest that chronic myelomonocytic leukemia may involve b-lymphocytes in the proliferative process and that it may be a clonal disease. Moreover, this case was associated with peripheral sensori-motor polyneuropathy. The disorder was responsive to prednisolone, as oral administration of prednisolone improved not only the peripheral polyneuropathy, but also the hematological findings. serum vitamin B12 and lysozyme in the serum and urine were decreased by the steroid therapy. The administration of prednisolone is effective not only for polyneuropathy but also chronic myelomonocytic leukemia.- - - - - - - - - - ranking = 2keywords = administration (Clic here for more details about this article) |
6/10. Inhibtion of cold insolubility of serum cryoglobulin by epsilon aminocaproic acid.Cold-induced insolubility of serum cryoglobulins can be inhibited by various carbohydrates and aminocarboxylic acids. Among the inhibitors, Epsilon amino caproic acid (EACA) was found to be effective at therapeutic levels in a patient who had mixed-type cryoglobulinemia. In vivo administration of EACA completely prevented cryoprecipitation; the inhibition being accompanied by a parallel decrease in the viscosity of the precooled serum. The data along with kinetics of serum EACA levels suggest specific binding of the drug to the cryoglobulins in this patient. This binding may be the basis for the observed inhibitory activity. The in vivo use of specific inhibitors of cryoglobulin precipitation may prove to have therapeutic application in the management of cryoglobulinemic patients.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
7/10. Nonsecretory plasma cell dyscrasia followed by acute granulocytic leukemia 25 years after Thorotrast administration.Twenty-three years after an injection of Thorotrast, profound depression of humoral and cellular immunity associated with extensive marrow plasmocytosis developed in a man aged 75 years. Twelve months of chemotherapy with L-phenylalanine mustard, resulted in hematologic remission. Radioautograph of his marrow showed signs of alpha-radiation; whole body counting revealed a significant thorium burden. One year after chemotherapy was stopped, and two years after initial presentation, aleukemic acute granulocytic leukemia led to his death. The role of thorium in the etiology of possibly two successive hematopoietic neoplasms is discussed.- - - - - - - - - - ranking = 4keywords = administration (Clic here for more details about this article) |
8/10. Granulocyte-colony stimulating factor concentrations in a patient with plasma cell dyscrasia and clinical features of chronic neutrophilic leukaemia.In order to study the pathogenesis of plasma cell dyscrasias with associated clinical features of chronic neutrophilic leukaemia, the concentration of granulocyte-colony stimulating factor (G-CSF) was measured in a patient, a 73 year old man, who underwent steroid pulse therapy. High G-CSF concentrations and leucocyte counts prior to treatment declined rapidly on administration of dexamethazone, but rose subsequently. G-CSF was not detected in primary cultures of bone marrow cells, but large amounts of interleukin-6 were found in the culture supernatant. These observations suggest that the neutrophilia observed in the patient represented a reactive response to G-CSF secreted from abnormal plasma cells or stromal cells rather than the existence of a genuine myeloproliferative disorder.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
9/10. Successful treatment by radiation and hormone therapy of isolated local recurrence of breast cancer 24 years after mastectomy accompanied by immune thrombocytopenia: a case report.We report a case of isolated local recurrence of breast cancer, which was accompanied by idiopathic thrombocytopenic purpura (ITP) and benign monoclonal gammopathy that presented 24 years after the patient underwent mastectomy. A 72-year-old female patient with a chest wall tumor was referred to our hospital in November 1994. Twenty-four years previously she had surgically treated breast cancer, of which the pathology was scirrhous carcinoma. Needle biopsy of the tumor revealed tubular carcinoma, which is compatible with local recurrence of breast cancer. She had no evidence of regional lymph node involvement or distant metastasis. Hematological and serological examination revealed a low platelet count accompanied by M-proteinemia (IgG, kappa-type) and a mild increase in platelet-associated IgG. She was initially treated with extensive-field chest wall radiation (60 Gy), followed by systemic administration of tamoxifen. Complete local control of isolated local recurrence (LR) was achieved after radiation therapy (RT) and the patient has been progression-free for more than 2 years. platelet count recovered gradually to a normal level after achievement of complete remission induced by radiation and tamoxifen. This may be the first case suggestive of a paraneoplastic syndrome of immune thrombocytopenia accompanied by local recurrence of breast cancer.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
10/10. Long-term follow-up of two patients with metastatic neuroendocrine tumours treated with octreotide.Two patients are described with metastatic neuroendocrine tumours of the pancreas head and region of Vater. After surgery, administration of the long-acting somatostatin analogue octreotide was started. In the first patient we found an IgG-lambda paraproteinaemia and a parathyroid hormone-related protein (PTHrP) driven hypercalcaemia. By increasing the dose of octreotide the paraproteinaemia disappeared. In the second patient with a metastasized somatostatin producing neuroendocrine tumour, octreotide showed a long-term stabilizing effect on symptoms and progression of disease. The role of octreotide in the induction of changes in biological behaviour of malignant neuroendocrine cells is discussed.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
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