Cases reported "Lymphatic Diseases"

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1/54. Hilar adenopathy in allergic bronchopulmonary aspergillosis.

    BACKGROUND: A 20-year-old male student developed allergic bronchopulmonary aspergillosis (ABPA). Computed tomography (CT) of the thorax done to detect central bronchiectasis (CB) for confirmation of diagnosis revealed, in addition, right hilar lymphadenopathy. Hilar adenopathy is thought to be rare in ABPA and has been documented only once before. Because of the finding of hilar adenopathy, the earlier reported patient had to undergo an invasive surgical procedure. OBJECTIVE: To report a case of true hilar adenopathy in ABPA. methods: This is a single case report. Contrast enhanced CT of the thorax was done. serum precipitating antibodies against aspergillus fumigatus were tested using gel diffusion technique, and intradermal testing with antigens of Aspergillus species was performed. Specific IgG antibodies against A. fumigatus and total IgE levels were measured by ELISA. RESULTS: A review of serial chest radiographs over a period of 3 years demonstrated transient pulmonary infiltrates and right hilar prominence. Computed tomography of the thorax revealed right hilar lymphadenopathy along with bilateral central bronchiectasis and patchy infiltrates. Strong bands of precipitins were detected against A. fumigatus. Intradermal testing with antigens of Aspergillus species elicited strong type I (immediate) and type III (Arthus-type) hypersensitivity reactions to A. fumigatus and A. niger. Specific IgG antibodies against A. fumigatus was positive and total IgE level was significantly elevated. Peripheral blood eosinophilia was also detected. CONCLUSIONS: Although extremely rare, ABPA should be considered in the differential diagnosis of hilar adenopathy.
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2/54. CD138-positive and Kaposi's sarcoma-associated herpesvirus (KSHV)-negative B-cell lymphoma with serosal spreading of the body cavity and lymphadenopathy: an autopsy case.

    CD138-positive and Kaposi's sarcoma-associated herpes virus (KSHV)-negative B cell lymphoma with serosal spreading of the body cavity and lymphadenopathy is presented. Our lymphoma cells showed pleomorphic morphology and a clonal immunoglobulin gene rearrangement. Immunophenotypically, they lacked B- and T-cell-associated antigens but expressed strong membranous CD138 antigen along the serosa. Although our case was not conventional primary effusion lymphoma (PEL) because of the absence of KSHV and the presence of lymphadenopathy, its unique phenotype and serosal spreading were consistent with those of PEL. Our case suggests that, irrespective of KSHV infection, some pleomorphic B cell lymphomas with membranous CD138 expression show a peculiar serosal spreading.
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3/54. Chronic T-cell lymphoproliferative disease expressing natural killer cell receptors: clinicopathological and molecular features.

    The frequency and clinicopathological significance of the expression of natural killer cell receptors (NKRs) in T-cell malignancies remain undefined. A 71-year-old man presented with leukocytosis, generalized lymphoadenopathy, and hepatosplenomegaly. bone marrow and lymph node biopsies showed a T-cell lymphoproliferative disease expressing NKRs (CD2( ), CD3( ), CD4( ), CD5( ), CD7( ), CD8(-), CD56(-), CD94( ), CD158a( ), CD158b( ), CD161(-), p70(-), TCRalphabeta(1), TCRgammadelta(2), TIA-1(-)). An abnormal clone, 46,Y,add(X)(p14),der(1)t(1;6)(p33;p21),t(7;12)(p10;q10), was found on conventional karyotyping. comparative genomic hybridization confirmed these findings, and showed a deletion of 12p that was not apparent on karyotyping. Clinically, the disease remained indolent and responded transiently to purine analogs but not to intensive chemotherapy. Peripheral T-cell lymphoproliferative disease of CD4( )alphabeta(1)NKR( ) phenotype is hitherto undescribed. The issues of whether this case was derived from transformation of a rare T-cell subtype or represented aberrant T-cell expression of NK-cell antigens, and the clinicopathologic significance of these T-cell neoplasms warrant further studies.
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4/54. PET and PLAP in suspected testicular cancer relapse: beware sarcoidosis.

    A 31-year-old man previously treated with chemotherapy for metastatic testicular cancer presented with new mediastinal lymphadenopathy and peripheral lung opacities. serum tumour markers were not elevated and a PET (positron emission tomography) scan revealed increased FDG (fluoro-deoxyglucose) uptake in the lungs and mediastinum consistent with testis cancer relapse. A biopsy of a mediastinal lymph node was performed and the pathology was that of sarcoidosis. immunohistochemistry however was positive for PLAP (placental alkaline phosphatase) and negative for EMA (epithelial membrane antigen). This immunohistochemical profile raised concerns that the observed pathology represented a sarcoid reaction to micro-metastatic testicular cancer relapse. We performed immunohistochemical pathology analysis on four known cases of sarcoidosis and found the same immunohistochemical-staining pattern. This case highlights the problem of specificity when interpreting the significance of PET scans and immunohistochemical analysis in this situation. sarcoidosis, a condition that has been associated with testicular cancer, should always be considered in the differential diagnosis.
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5/54. lymphangioma involving the mandible: immunohistochemical expressions for the lymphatic proliferation.

    We report a case of lymphangioma involving oral mucosa and mandible of an elderly female. The surgical and radiological examinations indicated that the lymphangioma was mainly distributed in the labial mucosa tissue, but had gradually extended into the periosteum and intrabony space of mandible. Immunohistochemical staining was also performed using antiseras of alpha-smooth muscle actin (alpha-SMA), von willebrand factor (vWF), angiogenin, vascular endothelial growth factor (VEGF), and proliferating cell nuclear antigen (PCNA) to elucidate the pathogenetic implications of the intraosseous lymphangioma. The present case of lymphangioma showed strong immunohistochemical reactivity of angiogenin and vWF, while it showed weak reactions of VEGF and PCNA. The immunostaining of alpha-SMA disclosed an abnormally thinned and discontinuous smooth muscle layer in the lymphatics. Both the x-rays and histological examination showed that the lymphangioma lesion was gradually extending into the adjacent osteoporotic marrow space of mandible. Therefore, we believe that the present case of intraosseous lymphangioma, which showed the harmatomatous growth of the lymphatics into the marrow space of mandible, is closely related to osteoporotic changes of old age.
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6/54. Generalized lymphadenopathy: a rare presentation of disseminated prostate cancer.

    Prostate cancer most often metastases to regional lymph nodes and bones by hematogenous or lymphatic spread. Metastases to the supradiaphragmatic nodes are rare. A 56-yr-old male smoker with generalized lymphadenopathy was referred to our center with the complaints of weight loss of 15 kg and severe back and leg pain. On computed tomography of the thorax and abdomen, massive mediastinal, intra-abdominal, retroperitoneal, and inguinal lymphadenopathies with hydroureteronephrosis of the left kidney were noted. Excisional biopsy of left cervical lymph node revealed metastasis of prostatic adenocarcinoma and transrectal biopsy of the prostate disclosed poorly differentiated adenocarcinoma. bone marrow aspiration biopsy, done for the differential diagnosis of anemia, also showed infiltration with prostate-specific antigen positive neoplastic cells. Supradiaphragmatic spread of prostate cancer has been postulated to be by a hematogenous route via the vertebral venous system, or Batson's plexus, accessible via direct extension from the primary cancer site.
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7/54. Lymph node infarction. An immunohistochemical study of 11 cases.

    CONTEXT: The etiology of lymph node infarction may be difficult or impossible to determine by histologic examination. Lymph node infarction is followed by malignant lymphoma in some but not all patients. The role of immunohistochemistry in the evaluation of lymph node infarction is not well defined. Although it is widely believed that necrotic tissue is not suitable for immunohistochemical study, this view may be inaccurate. OBJECTIVE: To determine whether lymphoid antigens are preserved in infarcted lymph nodes and to determine the utility of immunohistochemical staining in the evaluation of lymph node infarction. DESIGN: Retrospective immunohistochemical study of infarcted lymph nodes using archival formalin-fixed, paraffin-embedded tissue. SETTING: Academic medical center. patients: Eleven adult patients with lymph node infarction retrieved from pathology files. MAIN OUTCOMES MEASURES: Results of immunohistochemistry, diagnosis of lymphoma. RESULTS: Preservation of lymphoid antigens was observed in 4 of 6 cases of lymph node infarction associated with malignant lymphoma, including 3 of 5 cases of diffuse large B-cell lymphoma and 1 case of peripheral T-cell lymphoma. Nonspecific staining was not encountered. In 1 case, in which an infarcted lymph node showed a benign pattern of lymphoid antigen expression, lymphoma has not developed after 5 years. CONCLUSION: Lymphoid antigens are frequently preserved in cases of lymph node infarction, and immunohistochemical study of infarcted lymph nodes may provide clinically useful information.
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8/54. Angio-immunoblastic lymphadenopathy. A report of 3 cases.

    Angio-immunoblastic lymphadenopathy (AILD) was diagnosed in 3 Black patients, AILD is a non-malignant disorder which resembles a malignant lymphoma clinically and morphologically. It is thought to represent an abnormal response of B lymphocytes to antigenic stimuli which are often therapeutic agents. The disorder usually affects adults of the older age group and the clinical course can be rapidly fatal, particularly if vigorous chemotherapy is given. Of the 3 patients with AILD, 2 died from complicating infections within 6 months after the initial diagnosis. The third patient was completely cured after short courses of prednisone and vincristine. It appears that in some patients the disorder may be completely reversible but whether these patients are at a greater risk of eventually developing a lymphoma is uncertain at the present time, and remains to be ascertained by their long-term follow-up.
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9/54. Lymphadenopathy, oligoclonal T cell receptor rearrangement and systemic lupus erythematosus.

    To investigate the cause of generalized lymphadenopathy in case of systemic lupus erythematosus (SLE), we performed a molecular genetic analysis of lymph node, peripheral blood mononuclear cell and bone marrow specimens with T cell receptor and immunoglobulin gene probes. Oligoclonal T cell receptor rearrangements were detected in the lymph node cells. The oligoclonal T cell expansion observed is the first such example reported in SLE, and may be indicative of an immune response to specific antigenic challenge. Alternatively, these changes may represent the earliest phases of a malignant process. Molecular genetic investigations in autoimmune disease such as SLE can provide opportunities to enhance our understanding of the underlying condition, or reveal unexpected abnormalities requiring further assessment.
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10/54. Sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease). Clinico-pathological analysis of a paediatric case.

    Histochemical and immunohistochemical studies performed in only a few cases of sinus histiocytosis with massive lymphoadenopathy (SHML) indicated that SHML cells belong to the macrophage--histiocyte system, though their exact origin is still uncertain. We analyzed the morphological, antigenic and enzymatic characteristics of the histiocyte-like cells in one paediatric case of SHML (also named Rosai-Dorfman disease). The SHML cells expressed the S-100 protein, lectins concanavalin a, peanut agglutinin and monocyte-macrophage related antigens CD 11c, CD 14, CD 33, CD 68 and LN 5. Reactivity with other anti-macrophage antibodies (MAC387, lysozyme, alpha-1 anti-chymotrypsin) was variable. The CD1a antigen was present only in scattered cells, whereas HLA-DR and the HLA-DR associated invariant chain were absent. Cytochemistry demonstrated an intense activity of acid phosphatase and non specific esterase of SHML cells. A large amount of medium sized mononuclear cells were located in the sinuses and intersinusoidal tissue. Our findings suggest that SHML cells have intermediate features between phagocytes and langerhans cells/interdigitating reticulum cells. The heterogeneity of marker expression on SHML cells might be related to the local content of factors (e.g., cytokines), capable of modulating the phenotype of monocyted and derived cells.
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