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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Asian variant of intravascular large B-cell lymphoma (IVL) is characterized by hemophagocytic syndrome, i.e. the clinical features include pancytopenia, hepatosplenomegaly, and rarely mass formation. It usually lacks any neurological abnormality or skin lesions, which are typical features of classical IVL. Sixty-seven cases of IVL reported in Japan since 1990 were classified into two groups, and their clinicopathologic features were compared. Forty-five cases of IVL that met the clinical and laboratory criteria for the Asian variant were classified into Group A (average: 66 yr; male: 62%), and 22 cases that did not meet the criteria were classified into Group C (average: 65 yr; male: 73%). Hemophagocytosis was described only in Group A. The positivity rates for bone marrow invasion, fever, hyperbilirubinemia and elevated levels of LDH and CRP were significantly (p = 0.0037, p < 0.0001, p = 0.0428, p = 0.0108 and p = 0.0008, respectively) higher in Group A than in Group C. On the other hand, the positivity rates for neurological abnormality and skin lesions were significantly (p < 0.0001 and p = 0.0011, respectively) higher in Group C than in Group A. Our reassessment of the reported cases of IVL in Japan identified two major categories of IVL: the classical form and the Asian variant.
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PMID:[Clinicopathologic features of intravascular large B-cell lymphoma in Japan: review of the special reference to the Asian variant]. 1186 65

A patient is described who presented with pancytopenia, splenomegaly and excessively elevated lactate dehydrogenase levels in concurrence with signs of extramedullary hematopoiesis. Although initially considered in the differential diagnostic spectrum, a highly aggressive lymphoma could not be identified before the patient died, 6 weeks after admission. Even an intensive diagnostic work-up including splenectomy and repeated bone marrow biopsies was inconclusive. Finally, the diagnosis of an intravascular large B-cell lymphoma, a highly aggressive clinical subtype of a diffuse large B-cell lymphoma, spreading within vascular structures of multiple organs was established by autopsy. Intravascular large B-cell lymphoma is often not diagnosed before death due to the exclusive intravascular growth pattern of the tumor cells and a fulminant clinical course. The heterogeneous clinical features of this lymphoma subtype are discussed.
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PMID:Intravascular large B-cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem. 1219 78

A 75-yr-old male simultaneously having lymphoplasmacytic lymphoma (LPL) and diffuse large B-cell lymphoma (DLBCL) is presented. He had manifested with lumbago, high-grade fever, and confusion. Physical examination on admission showed disorientation and severe back pain. There were neither lymphadenopathy nor hepatosplenomegaly. Routine laboratory tests showed moderate pancytopenia, hypercalcemia (serum calcium, 15.9 mg/dL), IgM lambda-type monoclonal gammopathy (IgG, 405 mg/dL; IgA, 42 mg/dL; and IgM, 2023 mg/dL), and lambda-type Bence-Jones protein in the urine (0.8 g/d). Bone marrow biopsy showed the clusters of surface lambda-positive small-sized mature-appearing lymphoplasmacytoid cells. Bone survey and computed tomographic scan showed multiple osteolytic lesions and a tumor involving the third lumbar spine (L3). An open biopsy of the L3 tumor showed diffuse proliferation of CD20- and lambda-positive large cells. We thus diagnosed the patient as simultaneously having LPL and DLBCL. Although the combination chemotherapy was at least partially effective, he died of bacteremia and organ failure after three courses of chemotherapy. To clarify the clonal relatedness between LPL and DLBCL, we analyzed the sequences of the complementarity-determining region 3 in immunoglobulin heavy-chain genes. The data showed that LPL and DLBCL in the present patient originated from two independent clones.
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PMID:Simultaneous development of lymphoplasmacytic lymphoma and diffuse large B-cell lymphoma--analyses of the clonal relatedness by sequencing CDR3 in immunoglobulin heavy chain genes. 1258 Nov 94

A 57-year-old man was admitted with fever and epigastralgia, and presented with splenomegaly and pancytopenia. A CT scan revealed splenic infarctions. There were no lymphadenopathies, skin lesions, or neurological abnormalities. A splenectomy was performed. Bone marrow involvement with hemophagocytosis was noted. The diagnosis of Asian variant of intravascular diffuse large B-cell lymphoma was based on intravascular and sinusoidal distribution of large CD5+ B cells. The patient died of the disease 11 months after onset. To our knowledge, this is the first report of AIVL that presented with splenic infarction. This distinct lymphoma should be included in the differential diagnosis of splenic infarction.
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PMID:Asian variant of CD5+ intravascular large B-cell lymphoma with splenic infarction. 1258 30

Bone marrow involvement is infrequent at presentation in cases of diffuse large B-cell lymphoma. We report four adult patients with diffuse large B-cell lymphoma in whom bone marrow involvement with hematologic manifestations was the predominant clinical feature at presentation. Three patients presented with a leukoerythroblastic blood picture and one with pancytopenia. In each case, the unusual hematologic manifestations, with bone marrow replacement and the presence of immature forms in the peripheral blood, led to consideration of alternative hematologic diagnoses, including acute granulocytic leukemia in three cases and a myelodysplastic syndrome in one. The correct diagnoses were established by immunohistochemistry on formalin-fixed, paraffin-embedded bone marrow for two cases and by flow cytometry on aspirated bone marrow or peripheral blood lymphocytes for the other two. Diffuse large B-cell lymphoma should be considered in the differential diagnosis of unusual hematologic presentations, particularly in the elderly.
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PMID:Primary bone marrow B-cell lymphoma: report of four cases. 1263 6

A 56 year-old-man was admitted due to upper abdominal tumor and was diagnosed as having stage IVb diffuse B-cell malignant lymphoma that originally developed in the terminal ileum. The first and the second administrations of CHOP (cyclophosphamide, 750 mg/m(2); adriamycin, 50 mg/m(2); vincristine, 1.4 mg/m(2); and prednisolone, 100 mg/day) therapy were effective; however, the third course of therapy was postponed because of an episode of massive hematochezia. After this episode, lymph nodes began to enlarge and progressive pancytopenia occurred. Bone marrow smear showed the proliferation of reactive histiocytic cells which phagocytized red blood cells, white blood cells, and platelets. B-cell lymphoma-associated hemophagocytic syndrome (B-LAHS) was diagnosed. This case is extremely rare because: (1) LAHS occurred in an ileum-origin B-cell lymphoma, and (2) LAHS developed during an interval after chemotherapy.
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PMID:Hemophagocytic syndrome in ileum-origin B-cell lymphoma. 1461 9

A 31-yr-old man presented with a 1-wk history of fever, chills, weakness, headaches, and a significant 20-lb weight loss over the preceding 2 months. His past medical history was relevant for liver amebiasis during childhood. Two days before admission, the patient noticed jaundice. He denied abdominal pain or other GI symptoms, and there was no history of alcohol intake, medications, or illicit drugs. His physical examination revealed generalized jaundice, hepatosplenomegaly, and bilateral leg edema. Neurologically, the patient was agitated, with periods of disorientation, and he had bilateral flapping. His blood tests revealed pancytopenia, renal failure, liver failure, and coagulopathy. Because the patient had a fever, hepatosplenomegaly, and pancytopenia, a further workup also included a bone marrow and liver biopsy. No conclusive diagnosis could be made from the above tests, and the patient died 5 days after admission. Postmortem evaluation, including flow cytometry and gene rearrangement in the tissue obtained from the liver, revealed large B cell lymphoma. This case illustrates an unusual presentation of hepatic non-Hodgkin's lymphoma. Current information regarding this entity is scant, mainly owing to its rarity. We present a review of the literature, including the incidence, presentation, treatment, and prognosis of primary hepatic lymphoma.
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PMID:Primary hepatic non-Hodgkin's lymphomas: case report and review of the literature. 1468 34

A 70-year-old man presented with pancytopenia in August 2000, and the results of a bone marrow examination performed in January 2001 confirmed the diagnosis of refractory anemia. He was treated with cyclosporine (CsA) at 3.3 mg/kg per day, and the pancytopenia improved. The patient complained of epigastralgia 21 months later, and a gastric endoscopic examination showed an ulcer with a cleaved bank. A biopsy revealed diffuse large B-cell lymphoma. In situ hybridization analysis detected no Epstein-Barr virus (EBV) in the lymphoma. CsA treatment was discontinued, and a gastrectomy was performed 31 days later. A detailed histologic examination revealed no infiltration of abnormal B-cells in the resected stomach. Although EBV-positive lymphoma is a known complication of immunosuppressive therapy, no causal association between immunosuppressants and EBV-negative lymphoma has been established. The spontaneous remission observed after the withdrawal of CsA treatment suggests that immunosuppressive therapy can be a pathogenic factor in a subset of EBV-negative lymphomas.
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PMID:Spontaneous remission of Epstein-Barr virus-negative non-Hodgkin's lymphoma after withdrawal of cyclosporine in a patient with refractory anemia. 1500 45

We describe the case of a patient treated with 2-chloro-2'-deoxyadenosine, CdA or Cladribine for hairy cell leukemia who subsequently developed an Epstein Barr virus (EBV)-positive polymorphous large B-cell lymphoma (p-LBCL). The time interval between Cladribine therapy and development of p-BCL was 11 months and morphologically resembled an EBV-positive post transplant lymphoproliferative disorder (PTLD). Molecular genetic studies for EBV-clonality by Southern blot hybridization showed a clonal population of infected cells, implying that this was an EBV induced lesion. The chronology of events suggest that Cladribine, a purine analog which has been previously described to induce long-lasting immunodeficiency, can, in some cases, weaken the host defense mechanism to a level at which an innocuous EBV infection may transform the normal lymphoid cells into an aggressive neoplasm. Unlike most methotrexate-related lymphoproliferative disorders (LPDs), which undergo spontaneous remission after discontinuation of therapy, LPDs secondary to purine analogs often fails to resolve after discontinuation of therapy and requires additional therapy. Our patient was treated with rituximab following the diagnosis of p-LBCL, with the goal of improving the pancytopenia to permit chemotherapy. However, the patient failed to show any dramatic improvements in counts, developed systemic symptoms and progressive ascites. He expired 3 weeks after a second dose of rituximab. Cladribine is a potent immunosuppressive agent and should be included with the list of immunosuppressive agents that may be associated with EBV-related B-cell lymphoproliferative disorders.
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PMID:Epstein-Barr virus positive large B-cell lymphoma arising in a patient previously treated with Cladribine for hairy cell leukemia. 1529 65

A frail 78-year-old man presented with lethargy, fever, splenomegaly and pancytopenia. Bone marrow aspirate showed marked haemophagocytosis. A diagnosis of haemophagocytic syndrome secondary to diffuse splenic large B-cell lymphoma was eventually made. Treatment with laparascopic splenectomy was successful. Secondary haemophagocytic syndrome is a rare complication of many common conditions, and is fatal if untreated. A brief literature review is included.
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PMID:Non-fatal haemophagocytic syndrome in an elderly patient. 1610 62


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