Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a 12-year-old black male who presented with cervical lymphadenopathy, hepatosplenomegaly of 3 months duration, and ataxia and incoordination of recent onset. Hodgkin's disease, stage IVB, was diagnosed. An MRI of the head demonstrated a nonenhancing, well-defined pontine lesion. The pontine lesion and the associated neurologic symptoms were consistent with central pontine myelinolysis. Treatment of Hodgkin's disease resulted in complete remission and complete disappearance of the pontine abnormality.
 ...
PMID:Hodgkin's disease associated with central pontine myelinolysis. 768 85

A 30-year-old man complained of high fever, resistant to antibiotics, and progressive loss of strength for five weeks. The peripheral blood showed pancytopenia (leucocytes 2200/microliters, platelets 45,000/microliters, haemoglobin 10.7 g/dl). There was also hepatosplenomegaly, abdominal lymph node enlargement, pleural and pericardial effusions and slight excess of lymphocytes in the cerebrospinal fluid. Histological examination of the bone marrow suggested a small-cell pleomorphic T-cell lymphoma, but Hodgkin's disease was also considered. Splenectomy was performed to confirm the diagnosis and treat the pancytopenia. The blood count rapidly returned to normal and the suggestion of Hodgkin's disease was excluded. Intensive chemotherapy (BMFT-ALL protocol) was followed by regression of nearly all the abnormalities, but marrow infiltration persisted. This treatment was discontinued after phase II of induction. After an intercurrent cytomegalovirus infection maintenance treatment with alpha-interferon (up to 5 million units daily) was started. The partial remission remained stable for 22 months. The lymphoma then relapsed but was held in check by further chemotherapy (Dexa-PAMB protocol). For the past 11 months the patient has remained in stable partial remission on treatment with alpha-interferon (3 million units every other day). Frequent infections (sinusitis, diarrhoea, abscess) require close supervision.
 ...
PMID:[Pleomorphic T-cell lymphoma. The diagnostic problems, therapeutic possibilities and infection-induced complications]. 768 64

We examined bone marrow specimens from 19 patients with malignant histiocytosis (MH) and/or malignant lymphoma (ML) with concurrent hemophagocytic syndrome (HS) who suffered from high fever, hepatosplenomegaly, liver dysfunction, profound cytopenia, and erythrophagocytosis. There was little lymph-node enlargement or no tumor formation. The neoplastic cells in 3 patients exhibited histiocytes/macrophages phenotype with positive reactions for fluoride-sensitive nonspecific esterase, lysozyme and CD68 (KP1). Twelve other patients showed a T-cell (CD3) phenotype, in which 5 patients expressed CD30 (BerH2) as well. B-cell characteristics with CD20 (L26), CIg. nu lambda and gamma kappa were manifest in 2 patients, but indeterminate markers were found in the 2 remaining patients. Eighteen patients showed an infiltration of large neoplastic cells mainly with noncohesive interstitial growth pattern, ranging from 1.7% to 74.2% of the nucleated cells in the bone marrow. A large number of histiocytes/macrophages and dendritic cells was diffusely observed in 15 patients. Severely decreased hematopoiesis in all three series of hematopoietic cells was found in 16 patients. Bone marrow infiltration by the neoplastic cells and numerous reactive cells with erythrophagocytosis appears to be an important factor of profound cytopenia in patients of MH and/or ML with HS. The infiltrating pattern of the neoplastic and reactive cells in the bone marrow of MH and/or ML with HS was different from that of other types of peripheral T-cell ML, B-cell ML in high grade malignancy, and Hodgkin's disease. Cell characteristics and lineage of the neoplastic cells in MH and/or ML with HS are also discussed in this study.
 ...
PMID:Bone marrow findings in malignant histiocytosis and/or malignant lymphoma with concurrent hemophagocytic syndrome. 816 38

A 58-year-old man was admitted in May 1988, because of high fever, skin, pigmentation and body weight loss. Abdominal ultrasonography and generalized computed tomography examinations showed swelling of general lymph nodes and bilateral adrenal glands, splenomegaly, and lesion in the liver. Serum cortisol, urinary 17-OHCS and 17-KS level were within the normal range, while the ACTH level was elevated (189.9 pg/ml). ACTH overload test showed a non-reactive pattern, leading to a diagnosis of partial Addison's disease. He was also diagnosed as non-Hodgkin lymphoma, diffuse, large cell type (B) by a biopsy of the left supraclavicular lymph node. After combination chemotherapy, swelling of the supraclavicular lymph node diminished, followed by normalization of ACTH level and improvement of symptoms. Abdominal lymphadenopathy, hepatosplenomegaly and swelling of adrenal glands also decreased in size. Although involvement of tumor cells in adrenal glands sometimes occurs in malignant lymphoma, it is reported that more than 90% destruction of adrenal gland tissue is necessary to develop Addison's disease. It was suggested that the involvement of many lymphoma cells in both adrenal glands resulted in the development of partial Addison's disease in this case.
 ...
PMID:[B cell malignant lymphoma complicated with partial Addison's disease, report of a case]. 823 Jul 44

We report the fine-needle aspiration (FNA) cytomorphologic features of six cases of primary mediastinal large-cell lymphoma with sclerosis. The series consisted of three men and three women with a median age of 36 yr. All the patients presented with a large anterior or superior mediastinal mass with no evidence of peripheral lymphadenopathy or hepatosplenomegaly. Two of the cases showed typical findings of lymphoma, characterized by hypercellular specimens with numerous individually scattered markedly atypical lymphoid cells present, demonstrating nuclear irregularity with the presence of nucleoli and surrounding scant to slight amount of cytoplasm. Numerous lymphoglandular bodies were seen in the background. Both cases were correctly diagnosed as representing non-Hodgkin's large-cell lymphomas. Two other cases had slight cellularity with the presence of a few scattered atypical lymphoid cells. Although a definite diagnosis was not rendered in either case, the possibility of malignant lymphoma was considered. Two other cases consisted predominantly of microtissue fragments with some associated scattered individual atypical cells. Within the microtissue fragments, the cells were distorted with a tendency to elongate and spindle in a prominent fibrous matrix. Our experience demonstrates that FNA cytology of primary mediastinal diffuse large cell lymphoma with sclerosis can be challenging, with a potential for a false-negative diagnosis due to limited cellularity secondary to the sclerosis or a misdiagnosis as a spindle cell neoplasm due to distortion of these cells by the fibrous matrix. To the best of our knowledge, we believe this is the first FNA series of primary mediastinal diffuse large cell lymphoma with sclerosis.
 ...
PMID:Fine-needle aspiration cytology of primary large-cell lymphoma of the mediastinum: cytomorphologic findings with potential pitfalls in diagnosis. 851 16

A case of a healthy 23-year-old woman is reported with cytomegalovirus mononucleosis as a result of infection of cytomegalovirus probably primary. The patient presented with symptoms of generalized adenopathy, migratory arthralgias and arthritis, hepatosplenomegaly, long lasting rash as well as complications of pneumonia and myocarditis. Because on histopathological examination of lymph node the Hodgkin-Reed-Sternberg-like cells were found a misdiagnosis of Hodgkin's disease was initially made. After about 8 weeks period there was a complete recovery. The current problems related to cytomegalovirus infection are presented.
 ...
PMID:[Difficulties in diagnosis of cytomegalovirus mononucleosis syndrome]. 852 1

We studied 14 adult patients presenting with fever and cytopenia of the peripheral blood and histiocytic hyperplasia with hemophagocytosis (HHH) in the bone marrow regarding an association of cytomegalovirus (CMV) and Epstein-Barr virus (EBV) by using in situ hybridization (ISH) and also evaluated the clinical and laboratory findings according to the encountered organisms. ISH using a CMV RNA probe demonstrated infected cells in 6 out of 14 cases (43%), and ISH using an EBV EBER RNA probe demonstrated infected nuclei in 5 out of the same 14 cases (36%) of HHH. No cases showed a positive reaction with both probes. Three cases showed a negative reaction with both probes. The mean age of all patients was 29 years; and that of the CMV-positive patients was 27 years and that of the EBV-positive patients was 36 years. Organomegaly was found in 3 out of 6 CMV-positive patients (1 hepatomegaly, 1 splenomegaly, 1 hepatosplenomegaly), and 4 out of 5 EBV-positive patients (lymphadenopathy in all 4 cases, hepatosplenomegaly in 2 cases). One of the CMV-positive case had acute myeloblastic leukemia, and 2 EBV-positive cases had underlying malignancy (1 Hodgkin's disease, 1 non-Hodgkin's lymphoma). Seven out of the 14 HHH cases (50%) died within several months after diagnosis. Nucleic acid hybridization methods can be used for the routine examination of the association of CMV or EBV.
 ...
PMID:In situ hybridization studies of cytomegalovirus and Epstein-Barr virus in reactive histiocytic hyperplasia with hemophagocytosis. 887 10

A 62-year-old man was admitted to our hospital because of severe jaundice and fever. Physical examination demonstrated hepatosplenomegaly. The laboratory data revealed elevated serum bilirubin, alkaline phosphatase, lactate dehydrogenase, aspartate aminotransferase and alanine aminotransferase, and the reduced hepaplastin test (Normotest). Computed tomography showed hepatosplenomegaly and swelling of the paraaortic lymph nodes. Although he was treated with antibiotics and steroids, he died of hepatic failure 22 days after admission. At autopsy, his liver weighed 1910 grams, and a histological examination of the liver revealed marked infiltration of CD30 (Ki-1) positive lymphoma cells. He was diagnosed as having non-Hodgkin lymphoma, large cell anaplastic type, Ki-1 lymphoma. We herein report our findings of this very rare case of Ki-1 lymphoma associated with hepatic failure.
 ...
PMID:An autopsy case of Ki-1 lymphoma associated with hepatic failure. 944 89

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder characterized by diffuse lymphadenopathy, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia. Morphologically, the involved lymph nodes demonstrate complete effacement of the normal architecture, prominent neovascularization and infiltration by immunoblasts and plasma cells. Other terms that have been used to describe this entity include diffuse plasmacytic sarcomatosis, immunoblastic lymphadenopathy, lymphogranulomatosis X, and immunologic aberrations in idiopathic reticulosis. Initially, AILD was thought to be a disease of B-cell origin that represented reactive immune response to unknown stimulus and high potential for malignant transformation. It is now evident that AILD in 80% of cases follows an aggressive course with short median survival, especially, if complete response with chemotherapy is not achieved. Immunologic and molecular studies have demonstrated that the majority of AILD cases are T-cell clonal disorders. Despite the numerous reports on the role of Epstein-Barr virus in this disorder, it is unknown whether the presence of this virus is associated with the immune defect that accompanies AILD, or whether it is a pathogenetic factor. In contrast to non-Hodgkin's lymphomas, a stage is not usually assigned to the patient since the disease is systemic in nature, subsequently, parameters such as extent of disease and tumor bulk used to identify high-risk patients with non-Hodgkin's lymphomas, do not appear to correlate with disease activity or prognosis in AILD. Treatment of AILD has been unsatisfactory, with approximately 25% of patients achieving complete and sustained remission when combined chemotherapy agents are used. This article is devoted to a discussion of the different manifestations, suggested pathogenesis, and treatment of AILD.
 ...
PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment. 985 98

Penicillium marneffei is rarely pathogenic in humans. Most previously reported cases of P. marneffei infection were from Southeast Asia where patients were usually in an immunocompromised state due to human immunodeficiency virus (HIV) infection. The majority of the patients reported in Western countries were immunocompromised by malignancy, especially Hodgkin's lymphoma. In Taiwan, the first case of P. marneffei infection was reported in 1994 and involved an adult with HIV infection. We report a case of systemic P. marneffei infection in a child with common variable immunodeficiency (CVID). The patient, a 4-year, 5-month-old boy, had a 1-year history of oligoarthritis resembling juvenile rheumatoid arthritis (JRA). He developed a low grade fever (38 degrees C) and hepatosplenomegaly 1 month before admission to the hospital. Although cultures of synovial fluid obtained at the time of onset of oligoarthritis did not grow any organisms, cultures of blood, bone marrow, synovial fluid, and lymph node biopsy samples taken during this admission were positive for P. marneffei. Further immunologic studies revealed a profile characteristic of CVID. The fungal infection was finally eradicated by combined therapy with amphotericin B, fluconazole, itraconazole, and regular immunoglobulin replacement. This case reminds us that JRA or JRA-like arthritis should be differentiated from septic arthritis caused by rare pathogens in immunocompromised patients.
 ...
PMID:Systemic Penicillium marneffei infection in a child with common variable immunodeficiency. 987 36


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>