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

In the past decade, rituximab in combination with polychemotherapy has become the standard approach in most patients with advanced CD20-positive B-cell lymphoma. In mantle cell lymphoma (MCL), follicular lymphoma and diffuse large B-cell lymphoma, rituximab has been used as monotherapy and in combination with various chemotherapy regimens in different treatment situations. Routinely, rituximab is given intravenously, but other routes of administration have also been described. Here, we report a 64-yr-old woman who was previously treated with three different chemotherapy regimens for stage IV MCL. No sustained clinical and radiological response could be achieved. The patient's general status declined and she developed massive ascites as the dominant clinical problem. Local, intraperitoneal administration of rituximab was initiated as an experimental treatment approach. After 11 doses of rituximab, the general status of the patient improved significantly, ascites resolved completely and computed tomography (CT) scans demonstrated a partial remission of intra-abdominal lymph nodes and splenomegaly. Furthermore, we observed a regression of mediastinal lymph nodes, pleural effusion and centrocytes in peripheral blood as well as improvement of anaemia. The response to the experimental treatment has maintained for more than 6 months. In summary, we observed a sustained local and systemic response to intraperitoneal administration of rituximab in a patient with heavily pretreated advanced MCL.
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PMID:Intraperitoneal application of rituximab in refractory mantle cell lymphoma with massive ascites resulting in local and systemic response. 1790 14

A 78-year-old woman was admitted to our hospital because for pancytopenia and splenomegaly. The bone marrow examination revealed hairy cells in 11.6% of all nucleated bone marrow cells. Hairy cells were positive for tartaricresistance acid phosphatase (TRAP) staining, CD2, CD11c, CD19, CD20, sIgD, CD25, CD103 and k chain. In addition, abdominal enhanced computed tomography (CT) demonstrated splenomegaly and multiple nodular early stains in the spleen. She was diagnosed as having typical hairy cell leukemia (HCL). The patient was treated with cladribine infused continuously for 7 days at a dose of 0.09 mg/kg/day, which induced partial remission without severe adverse effects. Purine analogs such as cladribine and pentostatine are considered first-line agents for patients with HCL. Treatment with cladribine achieved a high overall response rate for patients with HCL and was safe even in elderly patients like ours. It is necessary to observe carefully the complications of severe infections, second malignancies and relapse of HCL after treatment with cladribine.
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PMID:[Successful use of cladribine in the treatment of an elderly woman with typical hairy cell leukemia]. 1803 31

Castleman disease, also known as angiofollicular lymph node hyperplasia, is an uncommon tumor involving lymph node tissues. The most common location is in the thorax presenting as a mediastinal mass. In the abdomen, most of the lesions are located in the pelvis, mesentery, and retroperitoneum. Only nine other cases of Castleman disease involving the pancreas have been described. We report a case of Castleman disease (hyaline vascular type) presenting as a pancreatic mass and an enlarged spleen. A 23-year-old woman presented with a 2-month history of abdominal pain localized to the left upper quadrant. Ultrasound and CT demonstrated a large retrogastric pancreatic mass and an enlarged spleen. Distal pancreatectomy and splenectomy were performed. Histopathologic examination revealed a markedly enlarged lymph node showing germinal centers with hyalinization and prominent follicular dendritic cells meshwork surrounded by concentric layers of small lymphocytes. Within the interfollicular area were regions of increased vascularity. BCL2, CD20, and CD79a show expanded mantle zones with a concentric targetoid arrangement of small lymphocytes. This is a case of unicentric Castleman disease of the hyaline-vascular type, which presented with signs and symptoms of a primary pancreatic neoplasm.
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PMID:Rare case of Castleman disease involving the pancreas. 1818 91

Sjogren's syndrome (SS) is characterized by an increased risk of developing non-Hodgkin's lymphoma (NHL). Optimal treatment for NHL-complicating SS is not clearly established. NHL, which expresses the CD20 antigen on tumor cell surfaces, is a disease entity candidate to treatment with anti-CD20 monoclonal antibodies. We report clinical and immunological data of a patient with SS and NHL who was treated with a regimen consisting of cyclophosphamide/vincristine/prednisone (CVP) plus rituximab. A 68-year-old women had a 26-year history of SS and autoimmune thyroiditis. The clinical course of SS was complicated with severe splenomegaly. An increased percentage of CD19+ B cells (up to 30%) was detected in peripheral blood during follow-up. Clonal rearrangement of immunoglobulin heavy chain was detected. Low-grade B marginal zone lymphoma was diagnosed (peripheral blood immunophenotype: CD19+CD20+CD23+sIg+Kappa; bone marrow immunophenotype: 25% lymphocytes; CD19+CD20+CD79A/BCL2+). She received a total of six cycles of CVP plus rituximab (375 mg/m2). Therapy was well tolerated, and B lymphocytes were depleted from the peripheral blood. Splenomegaly normalized. No evidence of neoplastic infiltration was detected in bone marrow after completion of therapy, while certain symptoms of SS (sicca and arthralgia) improved with treatment. CVP plus rituximab proved effective in a patient with SS with NHL.
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PMID:Combined therapy with rituximab plus cyclophosphamide/vincristine/prednisone for Sjogren's syndrome-associated B-cell non-Hodgkin's lymphoma. 1827 Aug 61

Combination of the splenic marginal zone B-cell lymphoma (SMZL) and classical Hodgkin lymphoma (cHL) is extremely rare. We report a unique case with concurrent SMZL and cHL. The patient was a 63-year-old man who presented with fatigue and anemia, showing a splenomegaly and retroperitoneal lymphadenopathy. A splenectomy revealed monotonous marginal zone lymphocytic infiltrates and numerous large Reed-Sternberg-like cells. Flow cytometry revealed a kappa light-chain-restricted CD5 (-), CD23 (-) B-cell population. DNA polymerase chain reaction analysis confirmed the presence of clonal rearrangement of the immunoglobulin heavy-chain gene. Immunohistochemical studies revealed that the large atypical cells were CD30 (+), CD15 (weakly +), CD20 (-), CD45 (-), Pax5 (weakly +), BOB.1 (-), and Oct2 (-), indicating the coexistence of SMZL with cHL. After the chemotherapy, the patient achieved a clinical/radiologic remission, whereas cHL was detected in liver and bone marrow subsequently. The case indicates that both components of lymphoma can present concurrently as a composite form of lymphoma and both need to be treated adequately.
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PMID:Classical Hodgkin lymphoma concurrently evolving in a patient with marginal zone B-cell lymphoma of the spleen. 1848 99

Rituximab is a chimeric monoclonal antibody directed against the phosphoprotein CD20. Because of its efficacy and acceptable toxicity profile, rituximab is now commonly used for the treatment of CD20-positive B-cell malignancies, including B-cell non-Hodgkin's lymphoma. However, rituximab-induced acute thrombocytopenia is an extremely rare side effect. We report a case of acute thrombocytopenia occurring immediately after rituximab infusion in a mantle cell lymphoma patient with bone marrow involvement and massive splenomegaly. Although the mechanism of thrombocytopenia is still unclear, it is possible that tumor burden, bone marrow involvement, the presence of infusion-related symptoms, and mantle cell histology are related to this rare complication of rituximab therapy. Hence, rituximab should be used with caution in patients who have these factors, and clinicians must be aware of this rare, but serious, side effect.
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PMID:Rituximab-induced acute thrombocytopenia: a case report and review of the literature. 1854 33

We report an unusual case of hairy cell leukemia (HCL) in a 55-year-old male who presented with fatigue, increased bruising, leukocytosis, anemia, thrombocytopenia and moderate splenomegaly without lymphadenopathy. Microscopically, a monomorphic population of small to medium-sized lymphoid cells with bean-shaped nuclei, ground glass chromatin and fine cytoplasmic projections was identified in the peripheral blood and bone marrow. Flow cytometric immunophenotyping demonstrated a monoclonal population of mature B cells with coexpression of CD25, CD11c and CD103. The clonal B-cells all exhibited homogenous expression of CD20 and uniform light scatter characteristics. However, CD103 expression was present in only half of the clonal B-cells. Flow cytometric cell cycle analysis using DRAQ5 DNA dye in intact live cells showed that both the CD103-positive and CD103-negative cell subsets exhibited a low S-phase fraction with no significant difference between the two subpopulations. Clinical remission was achieved by treatment with 2-chlorodeoxyadenosine. Variant and atypical cases of HCL have been described with varying intensity of CD11c, loss of CD25, aberrant expression of CD10, and lack of CD103 expression. However, the lack of CD103 in only a subset of the malignant cells in our case is an immunophenotypic aberrance that, to our knowledge, has not been previously reported.
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PMID:Hairy cell leukemia with unusual loss of CD103 in a subset of the neoplastic population: immunophenotypic and cell cycle analysis by flow cytometry. 1878 8

Hairy cell leukaemia (HCL) is a B-cell malignancy with a late developmental arrest. This report describes a patient that presented with leucocytosis and splenomegaly. The abnormal leucocytes showed typical morphology and expressed CD103, CD11c, CD19 and CD20 but not CD25 by immunophenotyping. The patient failed to respond to splenectomy and then developed lytic bone lesions and pathological fractures, which progressed despite a single course of cladribine chemotherapy. Review of the pathology of the bone reamings showed nonsecretory myeloma of the same kappa-light chain isotype. He went on to receive induction chemotherapy in preparation for an autologous stem-cell transplant but failed to mobilize sufficient numbers of stem cells. He has had two localized relapses with bony lesions, one within 6 weeks of stopping chemotherapy for which he received localized radiotherapy and thalidomide consolidation. Sequential myeloma has been described in HCL. There is controversy whether this represents clonal evolution or a secondary malignancy. An increased rate of secondary malignancies has been reported by some, but not other, authors in long-term survivors of HCL. This case illustrates the value of a repeat pathological review in case of unexpected complications.
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PMID:A case of Mollitias and Fragilitas Ossium - unusual presentation of hairy cell leukaemia followed by the diagnosis of nonsecretory myeloma. 1904 17

We report a case of B-cell prolymphocytic leukemia (B-PLL) that was treated successfully with splenic irradiation (SI). An 86-year-old man underwent a medical examination for lumbago and general fatigue at another hospital in June 2007. A compressed lumbar fracture and splenomegaly were found using computed tomography (CT). Thereafter, the patient consulted our hospital because of leukocytosis. Peripheral blood showed hemoglobin level 9.8 g/dl and white blood cell count 38.1x10(9)/l with 91% atypical cells. Surface marker analysis demonstrated that atypical cells were positive for CD20, CD22, FMC7, surface IgM, surface IgD and kappa, but were negative for CD5, TdT and lambda. The morphology of these cells was compatible with prolymphocytes with prominent nucleoli and condensed nuclear chromatin. A diagnosis of B-PLL was made. SI (total dose 20 Gy) was chosen for the treatment and a single course of SI was very effective without causing any significant adverse events. This case demonstrates that SI may remain valuable for the treatment of B-PLL in an elderly patient.
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PMID:[Splenic irradiation as a successful treatment for an elderly patient with B-cell prolymphocytic leukemia]. 1911 May 24

Five cases of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly are reported; three were splenectomized. BCL2/IGH rearrangements were found in three cases; HLA-DRB1*07 in all. Bone marrow (BM) trephines showed a moderate lymphoid infiltrate with intrasinusoidal distribution resembling a splenic marginal-zone lymphoma. Splenic white pulp revealed an enlargement of the marginal-zone area; red pulp was infiltrated by the same lymphocytes engulfing the sinuses. Splenic and BM B-lymphocytes were CD79a(+)/CD20(+)/IgM(+)/IgD(+)/bcl-2(+)/CD27(+)/DBA.44(-)/CD31(-) and polyclonal by immunophenotype/polymerase chain reaction. PPBL features an expansion of splenic marginal-zone B-lymphocytes, which infiltrate BM sinusoids and circulate in the blood with no evidence of clonality, even in cases with progressive splenomegaly.
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PMID:Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly. 1913 77


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