Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Exchange of information occurs between cells of neuroendocrine and immune systems. Neuroendocrine hormones may modulate lymphoid cell activities, including proliferation and mitogenesis, and immune cells may produce neuropeptides as well. Neuropetide Y is synthesized in B-cell leukaemia lymphoblasts, while substance P immunoreactivity has been detected in neoplastic haematological samples of different types of leukaemias. The presence of receptors for neuropeptides on different animal and human lymphoid cell lines, as well as in several types of animal and human lymphoproliferative diseases has been demonstrated. Species variability in receptor distribution has been shown as well. Receptor expression in immune cells may be regulated by changes in microenvironmental conditions, it may also be related to the activation and/ or proliferation state of cells. Vasoactive intestinal peptides receptors have been detected in myeloma cells, while somatostatin receptors have been first detected in vitro on resting lymphocytes and cells of the monocyte/macrophage lineage, and later on human activated lymphocytes and on lymphoblastic leukaemia cells. Somatostatin receptors have been found in biopsies from patients with malignant lymphomas. Tumor localization in non-Hodgkin lymphomas and Hodgkin's disease can be visualized by in vivo somatostatin receptor scintigraphy, contributing to establish the diagnosis and the stage of the disease. Recently. somatostatin receptors have been in vivo and in vitro detected in human thymic tumors. Although treatment of lymphoproliferative diseases with somatostatin analogs is a little explored field, partial remission was found in patients with low-grade non-Hodgkin lymphoma and cutaneous T-cell lymphoma, and a successful treatment with octreotide has been reported in patients with thymoma. Specific somatostatin receptors present in progenitors of immune cells are not expressed in the mature phenotype, while they can be detected in transformed cell lines. The possibility that this phenomenon is caused by oncogene expression cannot be ruled out. Moreover, preliminary data showed a developmental expression of somatostatin receptors in lymphoid cells, suggesting a potential role for neuropeptide receptors as differentiation markers. Although controlled studies are warranted to investigate the efficacy of the currently available analogs, somatostatinergic compounds may be of interest in the treatment of lymphoproliferative malignancies. A promising approach in refractory patients with somatostatin receptor positive malignant lymphomas may be radionuclide-targeted and cytotoxic analog therapy. These concepts increase the possibility of a wider antitumor treatment with ligands for neuroepeptide receptors than in established 'classic' neuroendocrine tumors.
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PMID:Neuroendocrine aspects of immunolymphoproliferative diseases. 1176 38

Three male patients, aged 56, 40 and 52 years, presented with recurrent infections and clinically suspected immunodeficiency, which was confirmed by the presence of hypogammaglobulinaemia. In one patient, no obvious underlying disease was identifiable, and primary immunodeficiency syndromes were considered in the differential diagnosis. A thorough diagnostic work-up revealed the presence of non-Hodgkin lymphoma in an iliac crest biopsy specimen. The second patient was found to have a thymoma. In the third patient, the immunodeficiency was the key to the ultimate diagnosis of multiple myeloma, which was still asymptomatic at that stage. Establishing the underlying condition had important therapeutic and prognostic consequences in these patients.
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PMID:[Immunodeficiency in adult patients as the first sign of a primary underlying condition]. 1257 66

As the thymus is composed of heterogeneous admixture of lymphoid and epithelial elements, tumors originating in the thymus may be of varied histologic types. Thymomas are the most common thymic tumor in adults. Thymoma classification has historically been controversial, but a system put forth by the World Health Organization (WHO) in 2004 has been generally accepted as a reproducible and clinically relevant classification. In addition to histologic subtype, tumor stage and resection status are important factors in determining outcome in thymomas. Thymic lymphomas typically occur in younger patients than thymomas. The most common thymic lymphomas are precursor T-lymphoblastic lymphoma, Hodgkin lymphoma, and primary mediastinal large B-cell lymphoma. Thorough histologic sampling and, in some cases, the appropriate use of ancillary studies such as immunohistochemistry, flow cytometry, and molecular studies, are important in proper pathologic evaluation of thymic tumors.
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PMID:Pathology of thymic tumors. 1610 55

Determining the appropriate surgery-based treatment for complicated anterior mediastinal malignancies (CAMM), especially those invading the superior vena cava (SVC) and its branches, remains a challenge for general thoracic surgeons. In this report, we summarize our experience and lessons regarding this issue in order to discuss a reasonable strategy for diagnosis and treatment of CAMM. Between January 2001 and April 2003, 15 patients with CAMM invading the SVC and/or its branches with or without invasion of other neighboring organs were surgically treated in our institution by a single surgeon team. We collected clinical data from the medical charts and from surgeons' specific notes for complicated cases, and performed a comprehensive analysis. There were 9 patients with malignant thymoma. Thymic carcinoma, teratoma, embryonal carcinoma, Hodgkin's lymphoma, non-Hodgkin's lymphoma, and mixed teratoma with thymoma were diagnosed in 1 patient each. All procedures were performed via median sternotomy. Some angioplasty techniques were successfully used to resect and reconstruct the SVC. Ten of the 15 patients also underwent pulmonary resection due to involvement of pulmonary parenchyma. Four of the patients underwent perioperative chemotherapy. There were no perioperative deaths. Two patients suffered prolonged ventilation after surgery, and there were no other severe complications related to surgery. One patient died 10 months after surgery. The remaining 14 patients were still living and their progress is still monitored. As of August 2004, the median follow-up duration for all patients was 35 months, and the disease-free survival duration was 10-43 months. CAMM can be safely and completely resected via a median sternotomy, even if it has invaded other mediastinal structures. CAMM should be pathologically identified before initial treatment. A good outcome for patients with CAMM is possible if a suitable strategy combining accurate diagnosis and appropriate treatment, especially surgical resection, is established.
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PMID:Surgical treatment of complex malignant anterior mediastinal tumors invading the superior vena cava. 1642 72

Interpreting a fine needle aspiration biopsy (FNAB) from the mediastinum is challenging as this location may harbor many lesions, including primary and metastatic tumors. Image-guided transthoracic (percutaneous) FNAB is less invasive than mediastinoscopy or endoscopic-guided FNAB. The aim of this study was to determine the diagnostic accuracy of FNAB performed percutaneously for evaluating mediastinal lesions.A retrospective study of 157 consecutive CT-guided transthoracic FNAB of the mediastinum was performed (1988-2004). Direct smears (N = 145; average 13 slides/case), ThinPrep slides (N = 25), and adequate cell blocks (N = 131) were prepared from procured cytologic material. When needed, ancillary studies included immunocytochemistry (N = 53) and flow cytometry (N = 8). Subsequent histologic tissue diagnoses available for 68 cases were also reviewed. Patients were of average age 57 yr (range 1-88 yr), including 75 males and 82 females. A definitive diagnosis was rendered in 128 (82%) cases. Primary neoplasms (N = 38) included 24 lymphomas (6 Hodgkin and 18 non-Hodgkin), 7 thymomas, 1 thymic carcinoma, and 6 peripheral nerve sheath tumors. Metastases (N = 72) were mainly carcinomas (N = 71) and 1 melanoma. There were 4 non-neoplastic lesions (1 granulomatous process; 2 bronchogenic and 1 pericardial cyst), 1 case of undifferentiated malignant large cell neoplasm, 13 cases negative for malignancy, and 29 (18%) that were indeterminate, due largely to insufficient cellularity. Subsequent histologic diagnoses were concordant with FNAB diagnoses in 53/68 cases (78%). Nine FNAB were inadequate/nondiagnostic. There were 6 discordant cases, including 5 FNAB that were of adequate cellularity but interpreted as negative for malignant cells (on subsequent histology 2 turned out to be Hodgkin lymphoma, 2 carcinomas, and 1 diffuse large cell lymphoma), and 1 diagnosed as thymoma that on histologic evaluation was a thymic large cell lymphoma. Adequate diagnostic cytologic material was obtained by image-guided percutaneous FNAB of mediastinal lesions in 82% of our cases. Sufficient material was available to make cell blocks and perform ancillary studies when necessary. These data also show a high proportion of agreement (78%) between FNAB and subsequent histologic diagnoses for a wide variety of mediastinal lesions. The majority of discordant cases were primarily interpretive, with a final cytologic diagnosis negative for malignancy. Only one problematic case misdiagnosed on FNAB as thymoma was found on subsequent surgical excision to be a thymic large B cell lymphoma. Cases with nondefinitive FNAB diagnoses were largely due to sampling error and/or insufficient cellularity. Therefore, percutaneous FNAB of the mediastinum is a diagnostically helpful, minimally invasive procedure that can be performed in patients of all ages as part of the evaluation of a mediastinal mass lesion.
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PMID:Diagnostic accuracy of image-guided percutaneous fine needle aspiration biopsy of the mediastinum. 1792 8

There are several processes implicated as uncommon causes of temporal lobe epilepsy. Trauma is the leading cause of epilepsy in young adults, intracerebral blood collection being the most consistent risk factor of seizures, especially subdural hematomas and brain contusions. Infarction is the entity most commonly related to epilepsy in the elderly population. Seizures usually present as complex seizures with high recurrence between 6 months and 2 years after stroke. There are some radiological characteristics of the affectation associated with high risk of early and late seizures. Noninfectious limbic encephalitis is a syndrome characterized by seizures, memory loss, and confusion. It includes paraneoplasic and non-paraneoplasic limbic encephalitis, both presenting as hyperintense lesion affecting temporobasal regions more evident with fluid-attenuated inversion recovery sequences. Paraneoplasic limbic encephalitis is associated with several types of tumor-induced autoimmunity against the nervous system. The tumors most frequently implicated are the lungs, testis, and breast, including Hodgkin's lymphoma, teratoma, and thymoma in young patients. Once a tumor is excluded, non-paraneoplasic limbic encephalitis has to be considered by investigating the presence of antibodies against voltage-gated potassium channels. It is associated with hyponatremia and responds to regimens of steroids, plasma exchange, and intravenous immunoglobulins. Finally, herpetic limbic encephalitis is also associated with seizures, accompanied by fever and neurologic symptoms. It presents characteristic findings and distribution on magnetic resonance imaging, which shows abnormalities in more than 90% of patients with proven Herpes simplex virus type 1.
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PMID:Uncommon epiloptogenic lesions affecting the temporal lobe. 1838 7

The anterior mediastinum is a common site of tumors with abundant lymphoid elements. Flow cytometry is a useful complementary technique to analyze this type of tumors, which provides qualitative and quantitative information. A differential diagnosis can be sometimes made between thymoma and precursor T-lymphoblastic lymphoma (T-LBL). Correct identification is of utmost importance for patient treatment. A total of 38 mediastinal tumors were analyzed, and samples were separated for flow cytometry. Flow cytometry data from thymomas and normal thymic tissue were compared with 42 cases of T-LBL from other anatomical locations. Among 38 mediastinal tumors, we found 6 benign lesions, 9 diffuse large B-cell lymphomas (DLBCL), 10 Hodgkin lymphomas (HL), 11 thymomas and 2 T-LL. Flow cytometry provided positive information in 24 cases, and defined lymphoid neoplastic cells immunophenotype or the typical lymphocytes accompanying thymomas. Flow cytometry helped differentiate 10 cases of HL and 4 benign lesions from other lymphomas (DLBCL, T-LBL, etc.). CD3, CD4 and CD8 expressions were most useful for the differential diagnosis of thymomas and T-LL. To conclude, flow cytometry is a valid complementary technique, which promptly provides information on mediastinal lesions, requiring small quantities of tissue for both early diagnosis and follow up of these diseases.
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PMID:[Flow cytometry for the study of mediastinal tumors with abundant lymphoid elements]. 1841 19

Pure red cell aplasia (PRCA) is a rare haematological disorder characterised by selective inhibition of red cell precursors in the bone marrow. We conducted a retrospective analysis of nine cases of PRCA seen in our adult haematology clinic from January 2000 to December 2003. All patients had baseline hemogram, bone marrow examination, CT scan of chest, autoimmune and infectious disease markers. The diagnosis of PRCA was made on bone marrow examination showing normal granulocytic and megakaryocytic series with erythroblastopenia. The age range was 14-68 years (median: 40 years) and a male to female ratio of 3 : 1. In five patients, the aetiology of PRCA could not be identified with available investigations and were labelled as primary PRCA. The secondary causes of PRCA included three cases of thymoma and one case of non-Hodgkin lymphoma. Out of nine patients, three patients had died and two lost to follow-up. At the last available follow-up till December 2006, three patients (one secondary and two primary PRCA) are maintaining normal haemoglobin and one patient still has active disease. The retrospective review shows that causes and outcome of PRCA in the developing world are different to those seen in the West.
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PMID:Spectrum of pure red cell aplasia in adult population of north-west India. 1861 74

Myasthenia gravis (MG) is an autoimmune neuromuscular junction disorder that is frequently associated with other autoimmune diseases. It has been only rarely reported in association with thymic Hodgkin's lymphoma. We report a case of a 22-year-old man who presented with thoracic symptoms. Clinical examination was normal. Simple chest radiography showed a heterogeneous mediastinal mass. Thoracic computed tomography revealed an antero-superior mediastinal mass measuring 6 cm and invading the upper lobe of the left lung with parenchymal micronodules of the left lung and an involvement of mediastinal nodes. In order to consider a diagnosis of thymoma, electromyography was performed despite the fact that the patient was asymptomatic. This exam demonstrated signs specific of MG. After stabilising the MG with symptomatic therapies, surgical intervention was performed. Histological and immunohistochemical findings led to the diagnosis of thymic Hodgkin's lymphoma. The MG regressed completely after surgical removal of the lesion. The patient is currently receiving complementary chemotherapy. An association between asymptomatic MG and thymic Hodgkin's lymphoma has not been documented in the literature, and the present case appears to be the first reported. The pathogenesis of this association remains unknown. Some authors support a genetic origin, while others propose a hypothesis based on immunological studies. The treatment of thymic Hodgkin's lymphoma is based on Cotswold staging system. Nonetheless, MG generally regresses after surgical removal of the thymic lesion.
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PMID:A primary mediastinal Hodgkin's lymphoma with asymptomatic myasthenia gravis: a rare association. 1884 21

Stiff-person syndrome (SPS) is an immunologically mediated central nervous system disorder that is infrequently associated with malignancy. We report a 31-year-old woman who developed SPS after autologous bone marrow transplantation and subsequent interferon treatment for multiple myeloma. Anti-glutamic acid decarboxylase (anti-GAD) antibody serology was positive. The myeloma remains in remission 10 years posttransplant. SPS has been described in association with lung cancer and breast cancer, thymoma, and Hodgkin lymphoma. This is the second report of SPS in a patient with myeloma and the first description of SPS following autologous transplantation. Aberrant immune reconstitution is known to occur following hematopoietic progenitor cell transplantation. Interferon is also known to augment the immune response posttransplant. We speculate that an aberrant posttransplant immune response may have caused both the SPS and an autologous graft-versus-myeloma effect, resulting in prolonged remission posttransplant.
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PMID:Stiff-person syndrome associated with multiple myeloma following autologous bone marrow transplantation. 1893 4


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