Cases reported "Lymphatic Diseases"

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1/22. Two cases of bites by the black-bellied swamp snake (Hemiaspis signata).

    We report two cases of envenomation by the black bellied swamp snake, Hemiaspis signata, with expert identification of the snakes. In the first case a 12 year old boy, who after the removal of the pressure immobilisation bandage, developed decreased fibrinogen levels and positive cross-linked fibrinogen degradation products (XDPs), but normal prothrombin time and activated partial prothrombin time. These changes resolved over 8h with no treatment. In the second case a 7 year old boy had local pain, swelling and axillary lymphadenopathy following the bite. These cases indicate the potential for coagulopathy and local symptoms following bites by large specimens of H. signata in young children.
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2/22. Pigmented mesenteric lymphadenopathy in familial adenomatous polyposis - an unusual cause of intraoperative abandonment of ileo-anal pouch.

    Familial adenomatous polyposis (FAP) is an autosomal dominant condition with near complete penetrance, characterised by the presence of numerous adenomatous polyps of the colon and rectum. melanosis coli describes the brownish-black discolouration of the colon resulting from the accumulation of a granular pigment in the phagosomes of macrophages in the colonic lamina propria. The presence of melanosis pigment in pericolonic lymph nodes has been reported in patients with coincidental melanosis coli, following segmental colonic resection. We report a unique case of FAP with melanosis pigment in lymph nodes in the small bowel mesentery that initially prevented a restorative proctocolectomy but that resolved following a colectomy, subsequently facilitating formation of an ileo-anal pouch.
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3/22. phenytoin hypersensitivity syndrome: a case report.

    We report a life-threatening case of phenytoin hypersensitivity syndrome, a rare reaction primarily characterized by fever, skin eruptions, lymphadenopathy, eosinophilia, and hepatotoxicity. The reaction developed in a 27-year-old black man with surgically corrected tetralogy of fallot who was treated with phenytoin for one month for exercise-induced sustained ventricular tachycardia. phenytoin therapy was discontinued and the patient was treated with intravenous methylprednisolone; the therapy was later converted to oral prednisone. Clinical improvement was noted five days after admission and the patient was discharged on hospital day 14. This adverse reaction usually develops within six weeks of starting phenytoin therapy and may be fatal in up to 40 percent of the patients who develop hepatotoxicity. Early recognition of this relatively rare reaction is essential to prevent serious and potentially fatal complications.
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4/22. Vascular transformation of lymph node sinuses. A process displaying a spectrum of histologic features.

    Nonneoplastic vascular lesions in lymph nodes have been infrequently reported. These lesions are believed to be rare and display histologic features ranging from minimal changes associated with vasodilation to vascular proliferative lesions resembling Kaposi's sarcoma. The spectrum of histologic features observed in these cases appears to be the result of the extent and duration of regional lymphatic and/or venous obstruction. We describe two such cases, one presenting in a 66-year-old black woman, after undergoing radical mastectomy for treatment of invasive ductal carcinoma of the breast following a breast biopsy; the other presenting in a 66-year-old black man with obstruction of the subclavian vein. We consider these cases worthy of review, both from a diagnostic standpoint and in the differential diagnosis of vascular neoplasms of lymph nodes, especially Kaposi's sarcoma.
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5/22. Systemic lupus erythematosus with deficiency of the T4 epitope on T helper/inducer cells.

    Three black Jamaicans with systemic lupus erythematosus (SLE) were identified whose T helper/inducer cells lacked the T4 epitope (T4 epitope-deficient phenotype). All three patients had lymphadenopathy as part of their syndromes. The asymptomatic and otherwise healthy T4 epitope-deficient brother of one of these patients also had lymphadenopathy in a distribution identical to that of his sister with SLE. family studies pointed to an autosomal codominant mode of inheritance not linked to the HLA locus for the T4 epitope phenotype. Cultures of peripheral-blood mononuclear cells revealed impaired B-cell differentiation upon stimulation with pokeweed mitogen in cells originating from the T4 epitope-deficient family members as compared with those originating from their T4 epitope-intermediate relatives. Ratios of T helper/inducer cells to T suppressor/cytotoxic cells, the presence of various autoantibodies, and proliferation in response to mitogens and in the mixed lymphocyte reactions did not correlate with T4 epitope phenotype. We suggest that SLE in association with the T4 epitope-deficient phenotype may represent a unique subset of patients with SLE that has distinct clinical and immunologic properties.
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6/22. Sinus histiocytosis massive lymphadenopathy syndrome: histogenesis of the hepatic lesion.

    Sinus histiocytosis massive lymphadenopathy (SHML) syndrome with hepatic involvement, occurring in a seven-year-old black female, is reported. Morphologic characterization of the hepatic lesion is accomplished utilizing conventional light, fluorescent and electron microscopy, and histochemical techniques and by comparing and contrasting the findings with those in cases of familial erythrophagocytic lymphohistiocytosis (FEL) and virus-associated hemophagocytic syndrome (VAHS). The histiocytic proliferation in the liver in SHML differs by showing: (a) an intralobular distribution with portal sparing; (b) marked steatosis; and (c) lipofuscinosis. The aforesaid intralobular distribution and the accompanying hypertrophy and hyperplasia of kupffer cells, as well as commonalities of steatosis and lipofuscinosis, and, to a lesser extent, erythrophagocytosis and siderosis, suggest a histogenesis from kupffer cells. The histochemical finding of fatty acid peroxides and both fluorescent microscopic and histochemical evidence of lipofuscin inclusions, a by-product of lipid peroxidation, in kupffer cells provide at least a theoretical basis for both erythrophagocytosis and proliferation eventuating in intralobular histiocytosis.
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7/22. phenytoin-induced hypersensitivity reactions.

    A case of phenytoin-induced hepatitis with mononucleosis is reported, and syndromes associated with phenytoin hypersensitivity reactions are discussed. A 23-year-old black woman with a two-month history of seizure disorder was admitted to a hospital with nausea, vomiting, fever, lymphadenopathy, diffuse maculopapular rash, left-upper-quadrant tenderness, and hepatomegaly. She was receiving phenytoin sodium 300 mg/day; carbamazepine 200 mg four times daily had been discontinued four days before admission because of leukopenia. phenytoin was discontinued after admission; however, phenytoin 1 g i.v. was given for a tonic-clonic seizure two days after admission, after which swelling of the face and legs and pruritus developed. Over the next few days, signs and symptoms of hepatotoxicity progressed, and she became comatose. seizures were treated with diazepam. She began to recover after 10 days of supportive therapy and was discharged several weeks later on primidone therapy. Serious phenytoin hypersensitivity reactions may appear as dermatologic, lymphoid, or hepatic syndromes. fever, rash, and lymphadenopathy often accompany hepatic injury. Encephalopathy and death may occur. Proposed mechanisms for phenytoin hypersensitivity include antigen-antibody reactions, alteration of lymphocyte function, and an enzyme abnormality causing the production of toxic metabolites. Treatment is supportive; phenobarbital and carbamazepine may be used with caution as alternate anticonvulsant therapy. The possibility of phenytoin hypersensitivity reactions should be considered when patients receiving phenytoin have unusual symptoms, particularly fever, rash, and lymphadenopathy.
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8/22. giant lymph node hyperplasia with osteoblastic bone lesions and the POEMS (Takatsuki's) syndrome.

    A 38-year-old black man with giant lymph node hyperplasia (GLH), osteoblastic lesions, and the poems syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) was treated at the University of chicago hospitals. The patient had hepatosplenomegaly and generalized peripheral lymphadenopathy. Endocrinologic abnormalities included decreased testosterone with elevated luteinizing hormone and follicle-stimulating hormone, as well as hyperprolactinemia and possible hypothyroidism. biopsy of a right femoral lymph node revealed GLH, and an osteoblastic pelvic lesion showed a marked lymphoplasmacytic infiltrate. By immunohistochemical techniques, plasma cells in the lymph node and osteoblastic lesion were polyclonal. A polyclonal hypergammaglobulinemia was present. The lymph node T-lymphocyte population showed a decreased helper-to-suppressor cell ratio. Other findings included thickening of the skin, finger clubbing, and anasarca. A severe sensory-motor polyneuropathy was the major factor contributing to the patient's death. The association of GLH, osteoblastic bone lesions, and the poems syndrome has been noted previously in japan; however, the authors are unaware of reports on Western patients who had this combination of clinical and laboratory findings.
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9/22. Eosinophilic cholangitis, lymphadenopathy, and peripheral eosinophilia: a case report.

    A 32-year-old black man had nonspecific abdominal complaints and was found to have obstructive jaundice, lymphadenopathy, and eosinophilia. Eosinophilic infiltration of the cystic duct, gallbladder, lymph nodes, and bone marrow was demonstrated. The patient's symptoms, lymphadenopathy, and eosinophilia resolved spontaneously, and there was no evidence of allergy, parasitic infestation, or hypereosinophilic syndrome. A comparison of this case to other diseases in which eosinophilia and eosinophilic infiltration of one or more gastrointestinal organs occurs is presented.
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10/22. Congenital thymic cyst in the neck.

    Cervical thymic cysts are rare. Thirty-four cases are reported in the English literature. Persistence of a part of thymopharyngeal duct may lead to the cyst formation in the neck. The majority of the cysts are seen in children under 10 years of age. There is marked variation in the size of the cysts. The majority of them are multilocular and the color of the fluid varies widely. The epithelial lining is variable; the majority of the cysts show stratified squamous epithelium. The characteristic histological features are Hassal's corpuscles, cholesterol crystals and aggregates of lymphocytes. Our patient was a four and a half year old female; she presented with a four month history of a painless swelling in the neck. The multilocular bluish-black cyst excised from the left side of the neck had all the histological features of a thymic cyst. A pre-operative diagnosis of such a cyst can be made only by bearing in mind the possibility of thymic remnants in the neck.
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