Cases reported "Lymphatic Diseases"

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1/112. granuloma inguinale (donovanosis) presenting as a neck mass in an infant.

    A case of granuloma inguinale (GI) presenting as a lateral neck mass in a 4-month-old, hiv-positive infant is described. The histological features of the mass were typical of GI, with numerous macrophages containing intracellular organisms with a "closed-safety-pin" appearance. This is a rare occurrence, and the mode of transmission of infection is discussed. An awareness of GI in infants by both clinicians and pathologists is important to prevent morbidity and allow for prompt institution of appropriate treatment.
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keywords = macrophage
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2/112. Destructive bone disease in early syphilis.

    Although destructive bone disease is a well-known complication of tertiary syphilis, osteitis or osteomyelitis are not commonly recognized as complications of early (primary or secondary) syphillis. A patient with secondary syphilis characterized by generalized lymphadenopathy, perianal condyloma lata, and positive rapid plasma reagin (RPR) and fluorescent treponemal antibody-absorption (FTA-ABS) tests also complained of headache, right should pain, and right anterior chest pain and swelling. Roentgenograms showed mottled osteolytic lesions consistent with previously described luetic bone disease. biopsy confirmed the diagnosis of syphilitic osteomyelitis, and treatment with penicillin resulted in prompt resolution of symptoms.
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ranking = 0.027628176423176
keywords = bone
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3/112. association of reticular dysgenesis (thymic alymphoplasia and congenital aleukocytosis) with bilateral sensorineural deafness.

    Reticular dysgenesis is a rare congenital disorder characterized by severe combined immunodeficiency and profound neutropenia, curable to date, only by bone marrow transplantation. This report describes the association of bilateral sensorineural deafness with this disease.
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ranking = 0.0046046960705293
keywords = bone
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4/112. Punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis: report of four new cases.

    Four new cases of punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis (LCH) are added to the four previously reported cases. All cases were shown on CT scans; plain films were rarely diagnostic. Pathologic correlation remains elusive since the usual biopsies of the LCH have been on skin or bone biopsies. A single prior pathologic study of the thymus in untreated LCH showed microscopic calcospherites. The thymic punctate calcific densities in patients with LCH may represent further accretion so that the calcospherites become macroscopic. The finding of such punctate calcific densities in an enlarged thymus of an infant with skin or bone or lung disease is strongly suggestive of LCH.
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ranking = 0.0092093921410585
keywords = bone
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5/112. Identification of new Fas mutations in a patient with autoimmune lymphoproliferative syndrome (ALPS) and eosinophilia.

    autoimmune lymphoproliferative syndrome (ALPS) is a rare, newly recognized, chronic lymphoproliferative disorder in children and is characterized by lymphadenopathy, splenomegaly, pancytopenia, autoimmune phenomena and expansion of double-negative (DN) T lymphocytes (TCR alpha beta , CD4-, CD8-). Defective lymphocyte apoptosis caused by mutations of the Fas (CD95) gene has been linked in the pathogenesis of ALPS, as binding of Fas-ligand to Fas can trigger apoptosis. Of the ALPS cases reported to date, point mutations, frameshifts and silent mutations in Fas all have been identified. We report two new point mutations in Fas in a child with ALPS and eosinophilia; studies on other family members established the pattern of inheritance for these mutations. Flow cytometric analysis of blood and tissues (spleen, lymph node, bone marrow) revealed abnormally expanded populations of DN T lymphocytes. Furthermore, activated lymphocytes and IFN gamma-activated eosinophils were resistant to Fas-mediated apoptosis. Eosinophil resistance to Fas-mediated apoptosis has not been previously described in ALPS. Sequencing of Fas revealed two separate mutations not previously reported. One mutation, a C to T change at base 836, was a silent mutation inherited from the mother, while the second mutation, a C to A change at base 916, caused a non-conservative amino acid substitution in the death domain of Fas, changing a threonine to a lysine. This mutation is associated with a predicted change in the structure of a part of the death domain from a beta-pleated sheet to an alpha-helix. We speculate that the mutation in the death domain prevents the interaction of Fas with intracellular mediators of apoptosis and is responsible for the autoimmune manifestations of ALPS and the abnormal lymphocytosis and eosinophilia in this patient.
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ranking = 0.0046046960705293
keywords = bone
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6/112. Immunoglobulin related amyloidosis presenting as isolated lymph node and pulmonary involvement.

    Here we present an unusual case of a 53-year old patient presenting AL-kappa amyloidosis with diffuse-type amyloidosis of lungs, lymph nodes and pleura. The underlying pathology was a B-cell immunoglobulin-secreting non-Hodgkin lymphoma, as proven by the presence of a monoclonal B-cell population in the bone marrow. Diffuse parenchymal infiltration of the lungs is extremely rare in non-systemic amyloidosis, with only 4 previous cases having been reported in the English literature.
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ranking = 0.0046046960705293
keywords = bone
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7/112. An unusual case of mediastinal lymphadenopathy caused by amyloidosis.

    A 71-year-old Japanese female Nagasaki-atomic-bomb survivor was admitted for evaluation of a mediastinal mass. She was infected with human T-cell leukemia virus type I. Histological examination of the biopsy specimen, obtained thoracoscopically from the mass, revealed amyloid lymphadenopathy confirmed by congo-red staining and electron-microscopic examination. Amyloid deposits and the serum monoclonal peak consisted of immunoglobulin lambda light chains. No atypical cells were detected in bone marrow. The case was diagnosed as an unusual amyloidosis affecting the mediastinal lymph node. No other sites were found to be affected by amyloidosis, although systemic involvement could not be conclusively ruled out.
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ranking = 0.0046046960705293
keywords = bone
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8/112. High-resolution CT findings of macrophage activation syndrome: a case report.

    macrophage activation syndrome (MAS) is a rare disorder characterised by benign, reactive, excessive, well-differentiated macrophage proliferation, secondary to an immune dysregulation in response to some triggering agents such as viral infection. We report a 3-year-old girl with MAS and pulmonary involvement. This is the first radiographic description of MAS on high-resolution CT.
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ranking = 5
keywords = macrophage
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9/112. mycobacterium genavense infection in a patient with long-standing chronic lymphocytic leukaemia.

    We describe the first case of disseminated infection with mycobacterium genavense in an hiv-seronegative patient with a chronic haematological disorder. Our patient, an 80-year-old woman, had been under long-term treatment with chlorambucil (partially in combination with prednisone) for B-cell chronic lymphocytic leukaemia (B-CLL). When she developed general fatigue and progressive anaemia, as well as progressive lymphadenopathy and splenomegaly, bone marrow biopsy revealed granulomas with acid-fast bacilli, and cultures of both bone marrow and blood grew M. genavense. The patient's CD4 cell count was approximately 100 microL(-1). Treatment with clarithromycin, ethambutol and rifabutin resulted in improvement of anaemia and general health as well as in regression of lymphadenopathy and splenomegaly.
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ranking = 0.0092093921410585
keywords = bone
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10/112. Metal-containing lymph nodes following prosthetic replacement of osseous malignancy: potential role of MR imaging in characterisation.

    The identification of regional lymphadenopathy in patients with bone malignancy treated by excision and insertion of a prosthesis usually indicates metastatic disease. We present two cases in which the lymphadenopathy was due to an uncommon but well-recognized foreign body reaction. This is secondary to the lymphatic uptake of metal debris shed by the prosthesis. In one case the metal within the excised lymph node could be demonstrated on in vitro MR imaging and in retrospect on the original in vivo scans. This condition should be considered when undertaking an MR examination in patients with bone malignancy treated by prosthetic replacement in whom there is a clinical suspicion of metastatic spread to the regional lymph nodes.
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ranking = 0.0092093921410585
keywords = bone
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