Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Diffuse large B-cell lymphoma is the most common form of lymphoma. It usually begins in the lymph nodes; up to 40% may have an extranodal presentation. According to a definition of primary extranodal lymphoma with presentation only in extranodal sites, there are reports of large B-cell lymphomas limited to liver or spleen as separate entities, and to date there have been only three documented cases of primary hepatosplenic presentation. This paper reports a fourth case. Due to a review of the literature and the clinical course of the case reported, we conclude that primary hepatosplenic large B-cell lymphoma has been found predominantly in females older than 60 years. The patients reported had <2 months of evolution prior to diagnosis, prominent B symptoms, splenomegaly in three and hepatomegaly in two, none with lymph node involvement. All had thrombocytopenia and abnormal liver function tests; three had anemia and elevated serum lactic dehydrogenase levels, two with hemophagocytosis in bone marrow. Because of the previously mentioned data, it can be stated that primary hepatosplenic lymphoma is an uncommon and aggressive form of disease that requires immediate recognition and treatment.
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PMID:Primary hepatosplenic large B-cell lymphoma: a rare aggressive tumor. 2149 Aug 48

Hemophagocytic syndrome (HPS) may be provoked by infections, malignancies and autoimmune diseases. We report on a 56-year-old woman with long-lasting systemic lupus erythematosus (SLE) who presented with malar rash, inflammatory livedo reticularis, fever, weight loss, pancytopenia and mild splenomegaly with cervical lymphadenopathy. She had criteria for SLE flare-up (malar rash, high antinuclear antibody titer, complement consumption, pathological urinary sediment, and retinal vasculitis). Despite high-dose glucocorticoid therapy, pancytopenia and fever worsened. Important elevations of triglycerides and ferritin were also found. Bone marrow aspirate demonstrated hemophagocytosis, which confirmed the coexistence of HPS and SLE. The treatment with glucocorticoids, immunoglobulins, cyclophosphamide, filgrastim and antimicrobial therapy was unsuccessful. After one month, the patient developed Pneumocystis jirovecii pneumonia with fatal outcome. Bone marrow biopsy, taken 5 days before death, showed high grade diffuse large B-cell (CD20+, Ki-67+) non-Hodgkin's lymphoma (DLBCL). We are the first to report the association of both SLE and non-Hodgkin's lymphoma complicated by HPS. We showed that, based on clinical and laboratory data, it was difficult to distinguish the early phase of HPS from SLE flare-up and new-onset DLBCL. Therapy of such a complex case of HPS has not been standardized, and opportunistic infections remain a difficult issue.
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PMID:Systemic lupus erythematosus progressing to non-Hodgkin's lymphoma complicated by fatal hemophagocytic syndrome: case report. 2250 70

Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (EBV+ DLBCL) of the elderly is an aggressive B-cell neoplasm related to age-associated impaired immunity. We report such a case in a 59-year-old woman with a catastrophic disease course. The patient initially presented with fever, fatigue, malaise and weakness over one-week period. Despite empirical treatment with antibiotics and antiviral agents, she subsequently developed multi-organ failure and coagulopathy. Radiographic imaging revealed hepatomegaly, splenomegaly, pleural effusion, and ascites. Her complete blood cell count showed marked leukocytosis, anemia and thrombocytopenia. Morphologic examination of blood smear demonstrated many abnormal plasmacytoid lymphocytes, and flow cytometric analysis detected an intermediate-large mature B-cell population (69%) without detectable surface immunoglobulin. High copy numbers of EBV genome were detected in the blood by PCR. A diagnosis of EBV+ DLBCL, leukemic phase, was made. Despite aggressive treatment and supportive care, the patient succumbed to multi-organ failure one week after initial presentation. Autopsy demonstrated EBV+ DLBCL infiltration in all the organs examined. This case describes an unusual presentation of EBV+ DLBCL and highlights the necessity of pertinent ancillary tests to avoid delay in the diagnosis and treatment.
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PMID:An Epstein-Barr virus-positive diffuse large B-cell lymphoma presenting as multi-organ failure: a catastrophic lymphomatosis with fulminant visceral organ dissemination resulting in a precipitous death in a 59-year-old female with no identifiable etiology for immunodeficiency. 2412 Jul 31