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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The haemophagocytic syndrome (HPS) is clinically characterized by fever, pancytopenia and hepatosplenomegaly. Usually it takes an acute course with a high mortality. The pathogenetic basis is inadequate activation of the immune system--in particular Th1-lymphocytes with subsequent overproduction of cytokines and extreme activation of macrophages with haemophagocytosis. The activated cells infiltrate organs, cause tissue damage and clinical manifestations of the syndrome. From the etiological aspect two forms exist: primary (familial) with autosomal recessive inheritance and the secondary form which forms a heterogeneous sub-group, caused as a rule by infection and/or a tumour. The prognosis seems somewhat more favourable in secondary forms. In treatment which is essentially the same for both forms, chemotherapy combined with immunosuppression proves useful, in more aggressive forms chemotherapy as used in the treatment of non-Hodgkin lymphomas. The only curative method is transplantation of haematopoietic stem cells which is also the treatment of first choice in the familial form of haemophagocytosis. In the submitted paper the authors present a review of contemporary knowledge on this treacherous and relatively rare entity. The haemopgagocytic syndrome should be always taken into account in the differential diagnosis of fever with an obscure etiology. The authors assume that the haemophagocytic syndrome is rarely considered in practice and therefore is usually inadequately diagnosed and thus not treated in time. In the conclusion the authors describe the case-records of a 26-year-old female patient with haemophagocytic syndrome which developed during pregnancy.
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PMID:[Hemophagocytic syndrome (case report and literature review)]. 1272 83

We describe a fatal case of human herpesvirus 8-associated bone marrow failure in a patient who had received intense treatment for Hodgkin lymphoma and was undergoing bone marrow transplantation. Bone marrow failure was resistant to antiviral treatment and a second infusion of autologous stem cells. Human herpesvirus 8 infection continues to be a major concern in transplant recipients in critical condition.
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PMID:Bone marrow failure associated with herpesvirus 8 infection in a patient undergoing autologous peripheral blood stem cell transplantation. 1313 Apr 19

Actinomycin C was administered to 35 patients with a variety of malignant states. There was no evidence of any toxic reaction. Of 11 patients with Hodgkins disease, four had brief objective remission ranging from two to four months. Individual patients with chronic myelogenous leukemia, multiple myeloma and neuroblastoma had short periods of clinical improvement. The usefulness of actinomycin C appears to be limited. It may best be used in instances of Hodgkin's disease where pancytopenia prevents the use of other agents.
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PMID:The carcinolytic effect of actinomycin C; a report of clinical studies. 1342 97

Relapse after anthracycline based combination chemotherapy is frequently seen in patients with aggressive non Hodgkin's Lymphomas (NHL), whereas complications such as secondary leukemia or solid tumor rarely occur. We report a patient with diffuse large cell (DLC) NHL and concurrent renal cancer, who developed acute myelofibrosis (AMF) later in the course of her disease. This 60-year-old female patient presented with pancytopenia and a right sided renal mass. Diagnostic work up revealed severe bone marrow infiltration by DLC NHL and renal cancer T1N0M0G2. Cytogenetic and molecular evaluation of bone marow cells showed three distinct clones, (a normal 46XX karyotype, a ringed chromosome 7 and a third clone with an enlarged chromosome 2 as well as several fragments). The patient underwent nephrectomy and eventually received 6 cycles of CHOP 14 chemotherapy. Anemia persisted followed by severe granulocytopenia and thrombocytopenia 6 weeks later. Repeated bone marrow biopsy showed absence of lymphoma and/or cancer metastasis, but massive myelofibrosis with an increased number of atypical megakaryocytes. Considering the short clinical course and the absence of hepatosplenomegaly AMF was diagnosed. The concurrence of three distinctneoplasms within a short period of time as well as the complex cytogenetic aberrations found in her bone marrow cells reflect a strong individual susceptibility to malignant disease in this patient.
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PMID:Acute myelofibrosis in a patient with diffuse large cell non Hodgkin's lymphoma and renal cancer. 1456 65

The risk of Hodgkin's lymphoma is increased in HIV-infected patients. In these patients Hodgkin's lymphoma is an aggressive disease with poor clinical outcome. Complete remission and survival rates are far below that reported in HIV-uninfected population. Since the widespread use of highly active antiretroviral therapy, malignancies are the major cause of mortality in HIV-infected patients. We report a case of a 29-year-old HIV-positive male who presented with fever of unknown origin, pancytopenia, and hemophagocytic syndrome. The diagnosis of Hodgkin's lymphoma with bone marrow involvement was made on bone marrow biopsy, 5 months after the onset of fever. The patient was treated with chemotherapy and achieved a complete remission. Relapse occurred 28 months later and he died 31 months after initial diagnosis. Prolonged fever is frequently observed in HIV-infected patients and could represent a diagnostic challenge. Infectious diseases are the most common causes, however lymphomas are the third most common cause of fever of unknown origin in HIV disease. This case illustrates the difficulties in accurately diagnosing Hodgkin's lymphoma in patients presenting with prolonged, unexplained fever, and emphasizes the use of bone marrow biopsy to confirm Hodgkin's lymphoma, particularly if cytopenia and hemophagocytic syndrome are present.
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PMID:Hodgkin's lymphoma as a cause of fever of unknown origin in HIV infection. 1458 89

Haemophagocytic syndrome is a serious disorder, often related to Epstein-Barr virus (EBV) or other infectious agents. Frequently an underlying immune abnormality or a T-cell lymphoma is present. The combination of haemophagocytosis and Hodgkin's lymphoma seems to be rare. A 70-year-old female with rheumatoid arthritis was admitted with constitutional symptoms, persistent fever, pancytopenia, deranged liver enzymes, lymphadenopathy and splenomegaly. A fatal coagulopathy supervened. The clinical picture and the bone marrow findings indicated a haemophagocytic syndrome and a lymph node biopsy disclosed an EBV-positive Hodgkin's lymphoma. EBV serology pointed at viral reactivation and a high EBV DNA content was detected in serum by real-time quantitative PCR analysis (5.5 x 10(6) copies per mL). The case history is presented and the literature is reviewed.
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PMID:Hodgkin's lymphoma, Epstein-Barr virus reactivation and fatal haemophagocytic syndrome. 1474 67

During treatment for inflammatory bowel disease (IBD) 2 men with ulcerative colitis, aged 52 and 38 years, and a 37-year-old man with Crohn's disease developed Epstein-Barr virus (EBV)-related non-Hodgkin's B-cell lymphoma. The first 2 patients underwent proctocolectomy and the use of immunosuppressive agents was discontinued, after which the lymphoma disappeared. The third patient had icterus, hepatosplenomegaly and pancytopenia; he died from multiple organ failure. Azathioprine and 6-mercaptopurine are first choice therapy in the treatment of steroid-refractory IBD. These immunomodulating agents are associated with the development of EBV-positive lymphomas in the setting of solid organ transplantation. This type of lymphoma is a rare complication in IBD, although the incidence in referral centres appears to be increasing. Since azathioprine is an important drug in IBD, there is a need for identification of IBD patients at risk of developing a lymphoma. EBV-DNA in plasma or in faeces may be a candidate tumour marker.
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PMID:[Epstein-Barr virus-related lymphomas in patients with inflammatory bowel disease]. 1612 85

Before the mid-1980s, haemophilia often was unknowingly treated with contaminated plasma products, resulting in high rates of human immunodeficiency virus (HIV-1), hepatitis C virus (HCV) and hepatitis B virus (HBV) infections. To estimate the impact of these infections, a new cohort was established. All HCV-seropositive patients, age 13-88 years, at 52 comprehensive haemophilia treatment centres were eligible. Cross-sectional data collected during April 2001 to January 2004 (median June 2002) were analysed. Plasma HIV-1 and HCV RNA were quantified by polymerase chain reaction. Highly active antiretroviral therapy (HAART) was defined as use of at least three recommended medications. Among 2069 participants, 620 (30%) had HIV-1. Of 1955 with known HBV status, 814 (42%) had resolved HBV and 90 (4.6%) were HBV carriers. Although 80% of the HIV-1-positive participants had > or = 200 CD4+ cells microL(-1), only 59% were on HAART. HIV-1 RNA was undetectable in 23% of those not taking antiretroviral medications. Most (72%) participants had received no anti-HCV therapy. HCV RNA was detected less frequently (59%) among participants treated with standard interferon plus ribavirin (P = 0.0001) and more frequently among HIV-1-positive than HIV-1-negative participants (85% vs. 70%, P < 0.0001). HIV-1-positive participants were more likely to have pancytopenia and subclinical hepatic abnormalities, as well as persistent jaundice, hepatomegaly, splenomegaly and ascites. HAART recipients did not differ from HIV-negative participants in the prevalence of ascites. The clinical abnormalities were more prevalent with older age but were not confounded by HBV status or self-reported alcohol consumption. Eleven participants presented with or previously had hepatocellular carcinoma or non-Hodgkin lymphoma. Although prospective analysis is needed, our data reveal the scale of hepatic and haematological disease that is likely to manifest in the adult haemophilic population during the coming years unless most of them are successfully treated for HIV-1, HCV or both.
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PMID:Prevalence of conditions associated with human immunodeficiency and hepatitis virus infections among persons with haemophilia, 2001-2003. 1612 97

We report a case of spontaneous regression of Epstein-Barr virus (EBV)-negative methotrexate-associated lymphadenopathy occurring with Hodgkin's lymphoma in the bone marrow of a 48-year-old woman with rheumatoid arthritis. Following 10 years of treatment with low-dose methotrexate, the patient developed pancytopenia, hypercalcemia, and elevated levels of liver enzymes over the course of 2 months. A computed tomography scan of the abdomen revealed splenomegaly and enlarged abdominal lymph nodes. A bone marrow biopsy demonstrated cellular marrow with 2 paratrabecular granuloma-like lesions composed of histiocytes, fibroblasts, small lymphocytes, a few plasma cells, and scattered CD30(+)CD15(+) Hodgkin's cells, including a classic Reed-Sternberg cell. The results of EBV studies of the bone marrow were negative. Within a month from withdrawal of methotrexate treatment, the patient's symptoms and the abnormalities in the laboratory results had regressed completely. A positron emission tomography scan failed to detect lymphadenopathy. Twelve months later, the patient remains free of symptoms.
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PMID:Reversible Epstein-Barr virus-negative lymphadenopathy and bone marrow involved by Hodgkin's lymphoma in a rheumatoid arthritis patient undergoing long-term treatment with low-dose methotrexate: a case report and review of the literature. 1644 52

Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of extranodal non-Hodgkin lymphoma derived from cytotoxic T-cells, usually manifesting by sinusoidal infiltration of spleen, liver and bone marrow. In 1997 World Health Organization classified malignant lymphomas and placed HSTCL among peripheral T-cell neoplasms. The course of the diseases is usually very agressive with a median survival time of 8 to 16 moths despite multiagent chemotherapy. We present a case of a 48-year-old male patient whose initial symptoms were fatigue, weight loss and night sweats, which were followed by splenomegaly and pancytopenia. After clinical examination we suspected him to have HSTCL which was proved pathohistologically upon splenectomy and it is the first case of this lymphoma diagnosed in "Merkur" Clinical Hospital. As a first line of lymphoma therapy we decided to apply FED course (fludarabine, cyclophosphamide, prednisone), being aware of the published poor results the standard CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisolone) yields. As far as we know, the results of this chemotherapy course in the therapy of this tumor have never been published. The patient underwent 6 courses of FED therapy, which he tolerated well and was in good clinical condition. Upon the completion of the 6th course of therapy he was diagnosed with lung anaplastic microcellular carcinoma and was treated with 3 course of PE therapy (cisplatin, etoposide).
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PMID:[Microcellular lung carcinoma in patient with hepatosplenic T-cell lymphoma: a case report]. 1680 95


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