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)

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease. Although an increased number of PPFE cases have been reported recently, the characteristics of this condition have not been well described. The present study reports on the case of a 34-year-old male patient who presented with unilateral lung abnormalities. The patient was admitted due to a 9-year history of progressive cough and exertional dyspnea, as well as a history of Hodgkin's lymphoma treated by autologous hematopoietic stem cell transplantation (HSCT). The patient had been initially diagnosed with tuberculosis and received regular anti-tuberculosis therapy for 18 months; however, the symptoms progressed. Serial chest computed tomography scans indicated a gradually worsening diffuse pleural thickening, dense subpleural opacification and volume loss, associated with evidence of fibrosis in the right lung. On physical examination the patient was cachectic, with a body mass index of 18.5 kg/m2, and he had a flattened thoracic cage. Arterial blood gas analysis revealed hypoxia. Pulmonary function tests revealed restrictive ventilation dysfunction and decreased diffusion capacity. The microbiological and cytological examinations were negative. Lung biopsy revealed a thickened pleura consisting of large amounts of collagen and elastic fibers, coexisting with subpleural intra-alveolar fibrosis with alveolar septal elastosis, without inflammatory infiltrates. The patient was diagnosed with PPFE secondary to HSCT and eventually succumbed to respiratory failure and infection while waiting for a lung transplant. Physicians should be aware of the typical and atypical characteristics of this rare disease, as its clinical and radiological characteristics may lead to misdiagnosis, particularly as chronic infections. The prognosis remains poor without effective long-term treatment.
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PMID:Pleuroparenchymal fibroelastosis secondary to autologous hematopoietic stem cell transplantation: A case report. 3090 45

Palisaded neutrophilic granulomatous dermatitis (PNGD) presents as skin-colored to erythematous papules symmetrically distributed on extensor extremities. Histologically, interstitial and/or palisaded granulomatous infiltrates are present with neutrophils and leukocytoclasis. Together with interstitial granulomatous dermatitis (IGD), PNGD is considered a reactive granulomatous dermatitis to a systemic trigger including connective tissue disease, infection, medication reaction, and less commonly malignancy. We present the second case of PNGD leading to diagnosis of Hodgkin lymphoma. A 47-year-old woman presented with a new asymptomatic eruption of erythematous, nonscaly papules and thin plaques with variable annular configuration, symmetrically distributed on extremities. Biopsies revealed mid-dermal focal degeneration of collagen and leukocytoclasis in association with a palisaded mixed inflammatory infiltrate of neutrophils, lymphocytes, and histiocytes, some forming small granulomas. She had associated fevers, night sweats, weight loss, arthralgias and malaise. Imaging revealed retroperitoneal adenopathy. Excisional lymph node biopsy led to diagnosis of Hodgkin lymphoma. Paraneoplastic PNGD is rare with only 9 reported cases. In all cases, PNGD presented concurrently or before presentation of clinical signs/symptoms of underlying neoplasm. We reviewed a total of 37 cases of paraneoplastic PNGD/IGD, summarizing clinical presentation, histology, associated neoplasm, and response of the skin lesions to treatment. The most commonly associated neoplasia is hematologic, with myelodysplastic syndrome (9 of the 37 cases or 24%) being the most frequent. Seven cases of solid tumor malignancies (prostate, esophageal, hypopharyngeal, breast, endometrial, and lung cancers) have been reported. Recognition of paraneoplastic PNGD/IGD may allow dermatopathologists, pathologists, and dermatologists to direct further workup that reveals underlying malignancy.
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PMID:Palisaded Neutrophilic Granulomatous Dermatitis Leading to Diagnosis of Hodgkin Lymphoma: Report of Rare Case and Literature Review of Paraneoplastic Granulomatous Dermatitides. 3092 Oct 8

This is a case report of a 68-year-old male with stage III right lower extremity lymphedema following right inguinal lymph node dissection and adjuvant chemoradiotherapy for Hodgkin's lymphoma. He developed peripheral neuropathy and radiation-induced right femoral artery thrombosis, treated with saphenous vein graft. He underwent three vascularized lymph node transfers (VLNTs) to the upper medial thigh, posterior calf, and ankle with placement of nanofibrillar collagen scaffolds. Three months after surgery, he had volume reduction, less neuropathic pain, and improved ambulation.
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PMID:Successful treatment of lymphedema in a vasculopath and neuropathic patient. 3122 51

Anaplastic large cell lymphoma (ALCL) and classical Hodgkin lymphoma (cHL) are lymphomas that contain CD30-expressing tumor cells and have numerous pathological similarities. Whereas ALCL is usually diagnosed at an advanced stage, cHL more frequently presents with localized disease. The aim of the present study was to elucidate the mechanisms underlying the different clinical presentation of ALCL and cHL. Chemokine and chemokine receptor expression were similar in primary ALCL and cHL cases apart from the known overexpression of the chemokines CCL17 and CCL22 in the Hodgkin and Reed-Sternberg (HRS) cells of cHL. Consistent with the overexpression of these chemokines, primary cHL cases encountered a significantly denser T cell microenvironment than ALCL. Additionally to differences in the interaction with their microenvironment, cHL cell lines presented a lower and less efficient intrinsic cell motility than ALCL cell lines, as assessed by time-lapse microscopy in a collagen gel and transwell migration assays. We thus propose that the combination of impaired basal cell motility and differences in the interaction with the microenvironment hamper the dissemination of HRS cells in cHL when compared with the tumor cells of ALCL.
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PMID:Migration Properties Distinguish Tumor Cells of Classical Hodgkin Lymphoma from Anaplastic Large Cell Lymphoma Cells. 3158 76

Classical Hodgkin lymphoma (cHL) is one of the most common malignant lymphomas in Western Europe. The nodular sclerosing subtype of cHL (NS cHL) is characterized by a proliferation of fibroblasts in the tumor microenvironment, leading to fibrotic bands surrounding the lymphoma infiltrate. Several studies have described a crosstalk between the tumour cells of cHL, the Hodgkin- and Reed-Sternberg (HRS) cells, and cancer-associated fibroblasts. However, to date a deep molecular characterization of these fibroblasts is lacking. Thus, the aim of the present study is a comprehensive characterization of these fibroblasts. Gene expression profiling and methylation profiles of fibroblasts isolated from primary lymph node suspensions revealed persistent differences between fibroblasts obtained from NS cHL and lymphadenitis. NS cHL derived fibroblasts exhibit a myofibroblastic phenotype characterized by myocardin (MYOCD) expression. Moreover, TIMP3, an inhibitor of matrix metalloproteinases, was strongly upregulated in NS cHL fibroblasts, likely contributing to the accumulation of collagen in sclerotic bands of NS cHL. As previously shown for other types of cancer-associated fibroblasts, treatment by luteolin could reverse this fibroblast phenotype and decrease TIMP3 secretion. NS cHL fibroblasts showed enhanced proliferation when they were exposed to soluble factors released from HRS cells. For HRS cells, soluble factors from fibroblasts were not sufficient to protect them from Brentuximab-Vedotin induced cell death. However, HRS cells adherent to fibroblasts were protected from Brentuximab-Vedotin induced injury. In summary, we confirm the importance of fibroblasts for HRS cell survival and identify TIMP3 which probably contributes as a major factor to the typical fibrosis observed in NS cHL.
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PMID:Fibroblasts in Nodular Sclerosing Classical Hodgkin Lymphoma Are Defined by a Specific Phenotype and Protect Tumor Cells from Brentuximab-Vedotin Induced Injury. 3167 43

Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+LBCL) is a rare non-Hodgkin lymphoma that exhibits a characteristic immunoblastic/plasmablastic morphology and is frequently expressing Clathrin-anaplastic lymphoma kinase fusion protein. Since being negative for T- and B- linage markers, this tumor is easy to be misdiagnosed, especially when it has unusual morphology and immunophenotype. Here, we report an ALK+LBCL with a diagnostic pitfall of carcinoma. The patient was a 28-year-old man with enlargement of a right submandibular lymph node. Morphologically, the lymph node had an unusual nodular growth pattern, with nodules surrounded by collagen bands. The neoplastic cells expressed epithelial membrane antigen, CD138, CD38, Mum-1, but negative for T- and B- linage markers, and showed a strong granular cytoplasmic Anaplastic Lymphoma Kinase staining pattern. Some tumor cells had the expressing of Cytokeratin.
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PMID:Anaplastic lymphoma kinase-positive large B-cell lymphoma with a diagnostic pitfall of carcinoma: a case report. 3196 73


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