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

Composite lymphoma (CL) is a rare disease with 2 distinct lymphomas concurrently arising in a single patient with an estimated incidence of 1-4.7% of newly diagnosed lymphomas per year. CL most commonly involves 2 B-cell non-Hodgkin lymphomas (NHL) or a B-cell NHL with a Hodgkin lymphoma. Our case is unique in that it was a bilineage CL with both a T-cell and B-cell NHL, which has only been reported in a few case reports. A 49-year-old woman presented with several months of progressive cough, weight loss, dyspnea, and supraclavicular lymphadenopathy. Computed tomographic imaging done upon admission to the hospital found that she had extensive anterior and middle mediastinal lymphadenopathy as well as bilateral supraclavicular lymphadenopathy. The patient underwent an excisional biopsy on the supraclavicular lymph node and was found to have a composite lymphoma involving both a T-cell and B-cell NHL. Her final pathological diagnosis was peripheral T-cell lymphoma and lymphoplasmacytic lymphoma. The patient was found to have stage IIIB disease. Her HIV, hepatitis panel, and tuberculosis tests were all negative. She then underwent chemotherapy with dose-adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab). The patient showed a complete response and was then referred to a bone marrow transplant center for an autologous hematopoietic stem cell transplant. CL is a rare disease composed of at least 2 distinct lymphomas concurrently arising in a single patient. Due to the complexity in having to treat multiple types of lymphoma simultaneously CL presents challenges with treatment and assessing prognosis.
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PMID:A Patient with Supraclavicular Lymphadenopathy and Anterior Mediastinal Mass Presenting as a Rare Case of Composite Lymphoma: A Case Report and Literature Review. 2820 78

Hepatic Hodgkin lymphoma is a rare disease, characterized by the presence of abundant granulofibrous stroma, and its radiological features have rarely been described. We report a 67-year-old man, who presented with liver masses that showed apparent delayed enhancement, along with systemic lymphadenopathy and musculoskeletal lesions. Repeated percutaneous needle biopsy, however, failed to confirm the diagnosis, and surgical biopsy finally revealed small amount of Hodgkin cells and Reed-Sternberg cells. In this report, the radiological features of hepatic Hodgkin lymphoma will be presented and discussed, in correlation with its histological findings.
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PMID:Hepatic Hodgkin lymphoma with delayed enhancement on CT and MRI. 2822 77

The gastrointestinal tract is the most common site of extra-nodal non-Hodgkin lymphoma. However, the incidence of primary rectal lymphoma is extremely rare. Among the primary gastrointestinal lymphomas, follicular lymphoma has been described as a rare disease. It is difficult to diagnose rectal lymphoma due to its variable growth patterns and inadequate biopsies. Majority of patients with rectal lymphoma have non-specific symptoms or negative biopsies, often delaying the diagnosis. Our patient is a 62-year-old female. Two sessile and smooth subepithelial lesions with a yellowish normal mucosa were found on a screening colonoscopy. The initial mucosal biopsy finding was chronic inflammation, but we were highly suspicion of malignancy; we performed an endoscopic mucosal resection. Herein, we present a rare case of rectal follicular lymphoma diagnosed by endoscopic mucosal resection with a literature review.
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PMID:Primary Follicular Lymphoma in the Rectum Incidentally Found on Screening Colonoscopy. 2823 83

Primary pancreatic lymphoma (PPL) is an extremely rare disease, with only a few cases reported in the literature. Clinical manifestations of PPL are often nonspecific and may mimic other pancreatic diseases. Because of the limited experience of PPL, clinicopathological features, differential diagnosis, optimal therapy, and outcomes are not well defined. We described two cases diagnosed as PPL and confirmed by histological examination and immunohistochemical analysis. Case 1 was a young man with obstructive jaundice and upper abdominal malaise mimicking a pancreatic adenocarcinoma. A computed tomography (CT) scan revealed a diffuse heterogeneous mass in the head of the pancreas along with dilated bile ducts, no dilated pancreatic duct, no liver or splenic involvement, or evident retroperitoneal adenopathies. The patient underwent a pancreatico-duodenectomy, and the postoperative histopathology confirmed diffuse large B-cell non-Hodgkin lymphoma. Postoperatively, he received six courses of the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone). Case 2 was an older man with left flank pain. A CT confirmed a mass with irregular margins at the tail of the pancreas and the hilum of the spleen. The mass was heterogeneous, with no clear boundary between lesions, spleen, stomach, and pancreas, with nearby blood vessels wrapped around it, and multiple enlarged lymph nodes in the abdominal cavity. A CT-guided biopsy was performed. The immunohistological findings of the specimen revealed a diffuse large B-cell lymphoma. The size of the tumor was significantly reduced after four cycles of the CHOP chemotherapy regimen. These two cases were different in clinical manifestation, location, and treatment. We reviewed the literature and discussed the clinicopathological features, differential diagnosis, optimal therapy, and outcomes of this neoplasm.
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PMID:Primary pancreatic lymphoma: two case reports and a literature review. 2835 55

Breast implant-associated (BIA) anaplastic large-cell lymphoma (ALCL) is a rare disease, comprising a small percentage of all non-Hodgkin lymphomas (NHLs), reportedly 2-3%. There is currently no established standard approach to the treatment of BIA ALCL. The first case on the development of ALCL in the presence of a breast implant was reported in 1997 and the association was first identified by the Food and Drug Administration in 2011. We herein describe a case of BIA ALCL in a patient with a previous history of breast cancer and breast reconstruction who presented with hardening of her breast implant. The patient underwent capsulectomy and the findings of the pathological examination were consistent with ALCL. The patient completed three cycles of combination chemotherapy consisting of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP regimen) followed by radiation consolidation therapy, and has maintained a complete remission ever since. The aim of the present study was to review the treatment options for BIA ALCL and suggest an investigation of the CD30-directed antibody-drug conjugate, brentuximab vedotin, as a potential treatment option for BIA ALCL.
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PMID:Breast implant-associated anaplastic large-cell lymphoma and the role of brentuximab vedotin (SGN-35) therapy: A case report and review of the literature. 2841 63

Nodular lymphocyte Hodgkin lymphoma (NLPHL) is a rare disease for which the optimal therapy is unknown. We hypothesized that rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) could decrease rates of relapse and transformation. We retrospectively reviewed patients with NLPHL diagnosed between 1995 and 2015 confirmed by central pathologic review. Fifty-nine had sufficient treatment and follow-up data for analysis. We described progression-free survival (PFS), overall survival (OS), and histologic transformation according to treatment strategy and explored prognostic factors for PFS and OS. The median age at diagnosis was 41 years; 75% were male, and 61% had a typical growth pattern. Twenty-seven patients were treated with R-CHOP with an overall response rate of 100% (complete responses 89%). The median follow-up was 6.7 years, and the estimated 5- and 10-year PFS rates for patients treated with R-CHOP were 88.5% (95% confidence interval [CI], 68.4% to 96.1%) and 59.3 (95% CI, 25.3% to 89.1%), respectively. Excluding patients with histologic transformation at diagnosis, the 5-year cumulative incidence of histologic transformation was 2% (95% CI, 87% to 100%). No patient treated with R-CHOP experienced transformation. A high-risk score from the German Hodgkin Study Group was adversely prognostic for OS (P = .036), whereas male sex and splenic involvement were adversely prognostic for PFS (P = .006 and .002, respectively) but not OS. Our data support a potential role for R-CHOP in patients with NLPHL. Larger prospective trials are needed to define the optimal chemotherapy regimen.
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PMID:Encouraging activity for R-CHOP in advanced stage nodular lymphocyte-predominant Hodgkin lymphoma. 2875 54

Composite lymphoma (CL) is a rare disorder defined as the coexistence of two or more distinct lymphoma subtypes at a single anatomic site. Discordant lymphoma (DL), which is the simultaneous occurrence of two or more distinct lymphoma subtypes at different sites, is also rare. CL complicated with DL involving three distinct subtypes of lymphoma in the same patient is an extremely rare disease. Clonal relationships in CL and DL are commonly investigated by molecular analysis using mutational status with t(14;18)BCL2/IgH translocation and immunoglobulin heavy chain variable-region (IgVH) gene rearrangement. A 73-year-old woman was admitted to our hospital with systemic lymphadenopathy and was initially diagnosed with diffuse large B-cell lymphoma based on pathological features of the biopsied esophageal tumor. However, the results of inguinal lymph node biopsy led to a revised pathological diagnosis CL consisting of Hodgkin lymphoma and follicular lymphoma. Three distinct coexisting lymphomas were identified in this individual patient. Molecular analysis revealed CL derived from common germinal center B-cell precursors, while clonal relationship between CL and DL was not clarified. This case suggests a mechanism underlying B-cell lymphoma pathogenesis involving two pivotal somatic mutations, t(14;18)BCL2/IgH translocation and IgVH rearrangement.
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PMID:Three coexisting lymphomas in a single patient: composite lymphoma derived from a common germinal center B-cell precursor and unrelated discordant lymphoma. 2917 42

Extramarginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) is a non-Hodgkin lymphoma of low-grade malignancy. The most common localization is the stomach, and the common nongastric sites are salivary glands, the skin, orbits, the conjunctiva, the lung, breasts, upper airways, other gastrointestinal sites, and the liver. Primary hepatic MALT lymphoma is a rare disease and the diagnostic can be challenging. The clinical presentation is nonspecific and may range from no symptoms to end-stage liver disease. The radiological aspect of hepatic lymphoma may indicate this diagnosis; however, the final diagnosis is made by hepatic biopsy. We report the case of a 47-year-old woman with no chronic liver disease, incidentally found with a focal liver mass at ultrasound examination. The only clinical symptom was fatigue. The blood tests were normal and tumoral markers were negative. Computed tomography and magnetic resonance imaging were performed. However, because the hepatic lesion was first described as a benign entity and, at second opinion, the suspicion of lymphoma was raised, the patient decided to undergo surgery first, without prior biopsy. The histopathologic analysis confirmed the diagnosis: hepatic MALT lymphoma positive for CD 20 and negative for CD 5, BCL6, cyclin D1, and CD 23. No lymph node involvement was noted and follow-up imaging with positron emission tomography-computed tomography did not show any other site of disease, thus confirming the diagnosis of primary hepatic MALT lymphoma. The aim of this paper was to highlight the imagistic features of primary hepatic lymphoma to contribute to the early diagnosis of this rare disease entity.
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PMID:Primary hepatic mucosa-associated lymphoid tissue lymphoma in a patient with no chronic liver disease: Case report. 2948 56

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare disease that is frequently underdiagnosed due to clinical features that are similar to those of non-Hodgkin lymphomas, systemic lupus erythematosus (SLE), or infectious reactive lymphadenopathy. An excisional biopsy is required. We report a young Caucasian female diagnosed with KFD with skin lesions, complicating with SLE. The clinical course, laboratory, and CT findings are described, as are histopathologic features, for a better recognition of this rare disorder in clinical practice.
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PMID:A Challenging Case of Kikuchi-Fujimoto Disease Associated with Systemic Lupus Erythematosus and Review of the Literature. 2978 10

Angioedema due to acquired deficiency of the inhibitor of the first component of complement (C1-INH) is a rare disease known as acquired angioedema (AAE). About 70% of patients with AEE display autoantibodies to C1-INH, the remaining patients have no antibodies to C1-INH. The clinical features of C1-INH deficiency include recurrent, self-limiting local swellings involving the skin, the gastrointestinal tract, and the upper respiratory tract. Swelling is due to accumulation of bradykinin released from high molecular weight kininogen. Patients with angioedema due to acquired C1 inhibitor deficiency (AEE) often have an associated lymphoproliferative disease including Non-Hodgkin Lymphomas (NHL). Among AAE patients with NHL, splenic marginal zone lymphoma (SMZL) has a higher prevalence (66%) compared to general population (2%) In the present study, we focused on patients with SMZL in AAE. We found 24 AAE patients with NHL and, among them 15 SMZL (62.5% of all NHL). We found NOTCH 2 activation in 4 /15 patients (26.6%) with SMZL, while no patients carried MYD 88 or BIRC3 mutations. Restricted immunoglobulin gene repertoire analysis showed that the IGHV1-2*04 allele was found to be over-represented in the group of patients with or without lymphoproliferative disease presenting with autoantibodies to C1-INH (41 of 55 (75%) of patients; p value 0.011) when compared to the control group of patients with AEE without antibodies to C1-INH, (7 of 27 (26%) of patients). Immunophenotyping failed to demonstrate the presence of autoreactive clones against C1-inhibitor. Taken together, these findings suggest a role for antigenic stimulation in the pathogenesis of lymphomas associated with AEE.
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PMID:Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization. 3007 22


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