[Leiomyosarcoma: unusual presentation].
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Biomedical subjects
Publications and source records attributed to C Hopfner.
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INTRODUCTION: In 30 p. 100 of Kikuchi's disease (or necrotizing histiocytic lymphadenitis), polymorphous and non specific cutaneous manifestations are present. We report herein an original case of Kikuchi's disease in which eyelid edema was the first sign. CASE REPORT: An 18-year-old girl was referred to our department for fever, arthralgia, cervical lymphadenitis and an important eyelid edema which had begun 4 days before. The rest of physical examination was normal. Laboratory tests revealed pancytopenia, elevated sedimentation rate, increased transaminases and normal muscular and thyroid tests. Various serologic studies were also negative. Thoracic CT scan, abdominal ultrasound and bone marrow biopsy showed no sign of lymphoma. Cervical lymph node biopsy revealed necrotizing histiocytic lymphadenitis, without neutrophils, suggesting the diagnosis of Kikuchi's disease. Eyelid edema due to lacrimal gland inflammation was resolved after local injections of cortisone. Our patient recovered without therapy within 3 weeks. No recurrence was observed after 4 months. DISCUSSION: Kikuchi's disease is rare and benign. It is clinically manifested by cervical or generalized lymphadenopathy, with fever. Diagnosis is made by lymph node biopsy showing necrotizing histiocytic lymphadenitis. The etiology is not yet well known, although a viral cause is often suspected. The main differential diagnoses of Kikuchi's disease are lupus erythematosus and lymphoma. Skin lesions are not well described. To our knowledge, we report herein the first case of eyelid edema revealing Kikuchi's disease. Therefore, Kikuchi's disease should now be considered as a new cause of eyelid edema.
Non-Hodgkin's lymphoma (NHL) occasionally involves the placenta, and information of such occurrence should be useful for management of the mother and fetus. We report the first case of anaplastic large cell lymphoma (ALCL) disseminated to the placenta. The diagnosis was made via excisional biopsy of cervical lymphadenopathy in a 20-year-old woman at 27 weeks' gestation. Involvement of the placenta was noted on gross examination after cesarean section delivery of a girl at 30 weeks' gestation. The ALCL was microscopically confined to intervillous spaces in a manner similar to previous reports of other NHLs. The immunophenotype was characteristic (CD30+, EMA+, BNH9+), and the now frequently associated t(2;5)(p23;q35) translocation with this lymphoma was detected by the recently produced monoclonal antibody ALK1 against the nucleophosmin/anaplastic lymphoma kinase (NPM/ALK) chimeric protein. Complete remission was induced in the mother after delivery. Both mother and child are healthy at 10 years' follow-up. The case is reported in light of the sparse literature on lymphomatous involvement of the placenta.
INTRODUCTION: Congenital rubella, which should disappear with widespread vaccination and mandatory obstetrical care, can occur as a purpuric eruption in the newborn. We report a case of blueberry muffin baby. CASE REPORT: An infant delivered after an "uneventful" pregnancy presented a generalized "purpuric" eruption and had axial hypotonia. Histology of a biopsy showed evidence of cutaneous erythropoiesis. The complete workup led to the diagnosis of congenital rubella. DISCUSSION: Cutaneous erythropoiesis is a well defined clinical and histological entity. There are several causes including infection and hematology disorders. Metastasis of a neuroblastoma, which must be eliminated by early biopsy, is the main differential diagnosis. CONCLUSION: Blueberry muffin rash is never idiopathic. The prognosis depends on the cause. Physicians should remember that congenital rubella has not yet been completely eradicated in France.
An IgG kappa light-chain myeloma, remarkable for the presence of inclusions in plasmocytes and proximal renal tubular cells, was discovered during investigations for renal failure associated with partial Fanconi's syndrome. An immunohistochemical study showed that the crystalline inclusions were positive for IgG and kappa light-chain in plasmocytes and exclusively for kappa light-chain in the renal tubular cells. An ultrastructural study showed that the inclusions were localized in lysosomes and had a 55 A periodicity. This case is similar to the forty odd myelomas or light-chain diseases reported in the literature and revealed by Fanconi's syndrome. Their prognosis is good, with a slow progression towards late renal failure. In our patient the preference of lesions for proximal tubular cells, the site of protein catabolism, was particular since in myelomas they are usually located in the distal tubular cells.
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The study was designed to test the efficacy of a PVP-iodine antiseptic agent on potentially pathogenic oral bacteria. A 0.5% concentration of the test compound was sufficient to inhibit the growth of 7 out of 8 tested strains. Only bacteroides gingivalis continued to grow during exposure to a 0.5 and 1% concentration of the test compound. Quantitative suspension experiments demonstrated that the onset of action occurred within five minutes. Quantitative suspension experiments with protein loading resulted in a lower efficacy of the tested agent. Results indicate that the tested compound may be recommended as an oral disinfectant.
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ACKD is frequent in chronic dialysis patients: its incidence is proportional to the length of time on dialysis. It occurs also in uremic patients before dialysis and persists, despite a tendency towards involution, in transplanted patients. ACKD is frequently associated with adenoma which can evolve into adenocarcinoma. Screening studies by sonography, eventually completed by CT, are essential to discover patients with ACKD, to follow them up and propose bilateral nephrectomy if ACKD evolves towards malignancy. ACKD should be considered as a pre-malignancy state.
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A case is reported of krükenberg's tumor, this rare lesion characterized by the constant presence of typical signet ring cells being an ovarian metastasis from a general digestive cancer. The primary neoplasm is often overlooked while the ovarian tumor is already large and bilateral. In a pregnant patient with this lesion virilization of mother and child may occur, this endocrine activity being poorly documented and the subject of a literature review. The pathogenicity of this tumor is still unknown. Endocrine investigations should be enlarged in the fairly rare cases when diagnosis is suspected prior to ovarian excision. Treatment is currently surgical but survival is little improved. Other therapy is only poorly effective.
A seventeen years old girl presented a HUS. The usual gastrointestinal symptoms of the prodromal phase involved secondary complications leading to emergency surgical treatment. Laparotomy exploration revealed segmental necrosis of the colon which was treated by colectomy: histological studies revealed its ischemic nature with specific lesions of thrombotic microangiopathy. Later course was uneventful with the exception of renal failure requiring maintenance hemodialysis. Review of literature confirms the rare and severe nature of gastrointestinal lesions requiring surgery. Great care should be taken on one hand, not to undertake a useless operation and on the other hand to operate in time colonic necrosis involving or not intestinal perforation or intussusception.
A hemolytic and uremic syndrome is described in a 41 year old woman on oral contraceptives for 18 years, only discontinued during the course of 4 normal pregnancies. A renal biopsy performed on the 20th day, after correction of the thrombopenia, confirms the typical aspect of advanced thrombotic microangiopathy. The usual etiological work-up is negative, but the search for steroidal anti-hormones antibodies by radioimmuno assay is positive. The evolution is favorable with hemodialysis, perfusion of fresh plasma during the sessions, calciparin, dipyridamol and anti-hypertensive drugs with, eight months later, a creatinin clearance of 45 ml/min and important persisting ischemic lesions in a new renal biopsy. The hemolytic and uremic syndrome following oral contraceptives remains rare (about thirty cases reported in the literature) and its prognosis remains severe despite a well adjusted treatment. Prevention includes the detection of risk patients and the search for steroidal anti-hormones antibodies could certainly play a role which remains to be evaluated.
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Chronic renal failure, rarely seen in tuberous sclerosis (TS), is usually related to renal hamartomas and/or cysts, but has only been very rarely linked to glomerular changes. In a patient with TS, we have performed repeated renal biopsies at early and advanced stages of renal failure. We have correlated the renal function deterioration with the evolution of focal and segmental glomerulosclerosis. Renal insufficiency would be in relation not only with renal involvement by multiple tumors, but also with the progression of focal and segmental glomerulosclerosis. This would be caused by hemodynamic changes in remnant glomeruli, after reduction in renal mass induced by renal tumors, and would be a new example of renal injury due to hyperfiltration.
A case of auto-immune thrombocytopenic purpura in a woman with benign cystic leiomyoma in Retzius' space is reported. The platelet Coombs' test was positive on platelets; the indirect Coombs' test was negative on serum but strongly positive on cyst fluid. Following excision of the leiomyoma thrombocytopenia rapidly subsided and the serological findings became negative, which strongly suggests that the antibody was produced by the tumour.
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