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)

Blood mononuclear cells from 540 newly diagnosed, unselected patients with B-cell chronic lymphocytic leukemia (CLL) were examined by immunofluorescence flow cytometry for a panel of surface membrane markers, including IgM and IgD, the monoclonal antibodies anti-CD3, -5, -20, -21, -22, -FMC7, and, for the final 125 patients, anti-CD23. There were 503 CD5+ and 37 CD5- cases. In the CD5+ cases, the cells typically expressed IgM, IgD, CD20, CD21, CD22, and CD23. In univariate analysis, age, clinical stage, IgM-fluorescence intensity, CD23, and FMC7 had significant prognostic importance, with high IgM-fluorescence intensity, high FMC7, and low CD23 expression being associated with a short survival. There was no significant difference in survival between 351 cases expressing IgMD and 55 cases expressing IgM without IgD, or between kappa and lambda light chain monoclonal cases. CD20, CD21, and CD22 had no prognostic importance. In Cox multiple regression analyses, age, CD23, IgM-fluorescence intensity, and clinical stage (International Workshop System) had independent prognostic importance. Thus, besides clinical variables, CD23 and IgM intensity might be useful prognostic markers in the management of CD5+, B-cell CLL. The survival of CD5- patients was on the borderline of being significantly shorter than that of CD5+ patients. The majority of the CD5- cases were FMC7+, CD23-, had strong IgM fluorescence, and had splenomegaly.
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PMID:Prognostic importance of flow cytometric immunophenotyping of 540 consecutive patients with B-cell chronic lymphocytic leukemia. 171 71

Cell surface structures related to differentiation, activation and "homing" were identified on the leukemic cell clone in blood of 64 patients with a monoclonal B-cell lymphoproliferative disorder. Patients were selected with regard to clinical signs and symptoms of the disease. 39 patients had progressive chronic lymphocytic leukemia of B-cell type (B-CLL): 16 with lymph node enlargement and 14 with progressive lymphocytosis as the most prominent symptom, respectively. 1 patient had an isolated splenomegaly and 8 had symptoms from enlarged lymph nodes, lymphocytosis and/or splenomegaly. 25 patients had an isolated monoclonal B-cell lymphocytosis in blood and bone-marrow but no other signs or symptoms of the disease. The lymphocytosis in these patients was considered to be of "undetermined significance" and the term B-cell lymphocytosis of undetermined significance (B-MLUS) was used. Patients with a prominent lymphadenopathy and/or splenomegaly had CD22+ leukemic cells while in patients with a progressive lymphocytosis the B-cell clone expressed Leu-8. Thus, CD22 might be related to the homing capacity of B lymphocytes for lymphnodes and spleen, while Leu-8 might define a circulating B-cell subset. In B-MLUS about 50% of the monoclonal B cells co-expressed Leu8 which is consistent with a more differentiated phenotype compared to B-CLL with progressive lymphocytosis. The CD22 expression was mostly low in B-MLUS although a few patients showed high values. The expression of receptors for growth factors (CD23, CD25, CD71) was higher in B-CLL compared to B-MLUS patients (p less than 0.001), which is consistent with a difference in lymphocyte activation stage and/or response to growth factors.
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PMID:Clonal cell surface structures related to differentiation, activation and homing in B-cell chronic lymphocytic leukemia and monoclonal lymphocytosis of undetermined significance. 226 61

Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are differentiated B-cell leukemias with well-described clinical, morphologic, and immunologic characteristics. We encountered two patients with indolent chronic B-cell leukemia showing overlapping features of these malignancies. The patients had progressive splenomegaly, minimal lymphadenopathy, and abnormal lymphoid cells with abundant cytoplasm and villi, which were strongly positive for surface antigens CD22 and CD11c, features associated with HCL. However, blood counts showed lymphocytosis without neutropenia and monocytopenia, and the bone marrow biopsies demonstrated tightly aggregated nodules of lymphocytes. In addition, the lymphoid cells were dual positive for CD19 and CD5, displaying weak-to-moderately positive monoclonal surface immunoglobulin, findings strongly suggestive of CLL. One patient failed to respond to therapy with chlorambucil and prednisone. The second patient showed a partial response to treatment with 2-chlorodeoxyadenosine. We compare our patients with similar variants of differentiated B-cell leukemias reported in the literature, including disorders described as hairy cell variant (HCL-V) or splenic lymphoma with villous lymphocytes (SLVL).
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PMID:CD5+ chronic B-cell leukemia with features intermediate to chronic lymphocytic leukemia and hairy cell leukemia. 752 21

We present the clinical and immunological features of a rare case of chronic lymphoid leukaemia with lymphoplasmacytoid morphology. The patient was first admitted suffering from weakness, pallor, dyspnoea, marked splenomegaly, hepatomegaly and systemic lymphadenopathy and panhypogammaglobulinaemia. White blood cell count revealed important leukocytosis (220 x 10(9) WBC/l) with 2% neutrophils and 98% lymphoid cells showing lymphoplasmacytoid features, while lymphoid cells of identical morphology severely infiltrated the bone marrow and lymph nodes. The disease, initially controlled by non aggressive chemotherapy over a period of 30 months, later evolved to a clinical and haematological picture suggestive of Richter's syndrome. Immunophenotyping of the leukaemic cells demonstrated a monoclonal expansion of B-cells bearing surface markers of typical CLL (CD5, CD19, CD20, CD21, CD22, CD23, CD24, CD40 and low density IgM+IgD/kappa) and also the CD11c and CD38 antigens. A proportion of these cells expressed activation markers (CD25, CD69 and CD71). Following in vitro activation with TPA or PWM, the cells responded by weak incorporation of 3H-TdR but failed to secrete immunoglobulins. These findings confirm the broad morphological, phenotypical and clinical spectrum of chronic lymphoid leukaemias.
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PMID:Monoclonal expansion of immunoglobulin not-secreting CD5+ CD11c+ CD38+ B-cells in a rare case of chronic lymphoplasmacytoid leukaemia. 797 Dec 44

The recently described monocytoid B-cell lymphoma is a low-grade lymphoma presenting most frequently in elderly women and commonly associated with autoimmune diseases. Leukaemic expression of this disease has been reported in advanced stages. A case of monocytoid lymphocytosis without lymph node enlargement is presented herein. A 60-year old woman complaining of easy bruises was found to have a 2-cm splenomegaly. Her laboratory data included the following: haemoglobin, 125 g/L; haematocrit, 0.35 L/L; white cell count, 29 x 10(9)/L with 32% PMN, 3% stabs, 2% myelocytes, 1% metamyelocytes, 30% lymphocytes and 32% atypical mononucleated cells showing wide, pale cytoplasm neatly contoured and oval nucleus with monocytoid features. The basal coagulation study showed prothrombin 50%, APTT 40 seconds, fibrinogen 68 mg/dL and FDP between 80 and 160 ng/dL. Splenomegaly without lymph-node enlargement was found on CT scan. The bone-marrow biopsy showed a 68% monocytoid lymphocytic infiltration, acid-phosphatase positive and tartrate-sensitive, without fibrosis. Bone-marrow and peripheral immunophenotype showed those cells to be CD22, CD 19 and CD11 positive, while T and CD25 markers were absent. The patient was treated with alpha-2b interferon at a dose of 3MU three times a week for 6 months, with general improvement and regression of the leukaemic expression. Eleven months after diagnosis she died of a central nervous system haemorrhage. The morphological, immunological and cytochemical features of the monocytoid lymphocytes in this case are commented, along with their variable behaviour. A review of the literature is also carried out, attention being laid on the onset and the response to therapy of B-cell monocytoid lymphomas as the singularity of this case lies on its exclusively leukaemic onset. It is concluded that an interrelationship between monocytoid B-lymphocytic leukaemia and B-cell monocytoid lymphoma might possibly exist, such as that between chronic lymphocytic leukaemia and diffuse lymphocytic lymphoma.
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PMID:[Monocytoid B-lymphocytic leukemia]. 805 92

Twenty-six patients, whose B-cell lymphoma had relapsed after conventional therapies, were treated in a phase I dose escalation study with an immunotoxin consisting of a mouse CD22 monoclonal antibody (RFB4:IgG1K) coupled to chemically deglycosylated ricin A chain (dgA). Two to 12 doses of the immunotoxin were infused intravenously at 48-hour intervals. The peak serum concentration and half-life (T1/2) did not correlate directly with the dose and averaged 3.8 micrograms/mL and 7.8 hours, respectively. The main dose-limiting toxicity was caused by the vascular leak syndrome (VLS) consisting of weight gain, edema, serum albumin decrease, and critically by pulmonary edema. Myalgia occurred frequently and was only dose limiting in one patient who developed rhabdomyolysis. The presence of lymphoma cells in the blood (> or = 10(10)/L) and clinically detectable splenomegaly were associated with reduced toxicity and a shorter T1/2. Nine of 24 evaluable patients (37.5%) made antibody to either mouse Ig or dgA. There were five partial responses (PR) and one complete response (CR) lasting 30 to 78 days. High peak concentrations of immunotoxin in the serum, a long T1/2, and large areas under the curve (AUC) correlated with both clinical response and toxicity. None of three patients with CD5+ lymphomas (including two CLL patients) had more than mild toxicity or responded to the immunotoxin.
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PMID:A phase I study of an anti-CD22-deglycosylated ricin A chain immunotoxin in the treatment of B-cell lymphomas resistant to conventional therapy. 821 17

An unusual case of low-grade B-cell lymphoproliferative disorder with peripheral lymphocytosis and splenomegaly followed for 4 1/2 years is reported. During this period, the phenotype of the tumor cells in the blood changed from that of hairy cell leukemia (HCL)/chronic lymphocyte leukemia (CLL) to HCL/prolymphocytic leukemia (PLL), to PLL. The lymphoid population in the blood showed a mixture of hairy cells, villous lymphocytes, small lymphocytes, and prolymphocytes, corresponding to the phenotypes at various stages. Although relatively specific markers for CLL, HCL, and PLL, such as CD5, CD11c, CD22, CD25, and FMC-7, were positive at various stages, all these markers have also been demonstrated in a large study series of splenic lymphoma with villous lymphocytes (SLVL). In addition, the histologic pattern of the bone marrow biopsy and splenectomy specimen were not typical for HCL. This case can therefore be classified either as HCL variant or as SLVL. As SLVL assumes various cytologic and histologic patterns, which overlap with different lymphoproliferative disorders, especially HCL variants, this entity appears to represent a heterogeneous group of lymphomas/leukemias that may evolve into each other. The absence of activation of c-myc and bc1-2 oncogenes as well as mutation of p53 tumor suppressor gene, together with the presence of only one single rearranged band for both heavy chain and kappa light chain genes in our case suggest that these morphologically different lymphoid tumors may belong to the same family.
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PMID:Relationship between hairy cell leukemia variant and splenic lymphoma with villous lymphocytes: presentation of a new concept. 860 28

Observations are described using a combination of two bispecific F(ab')2 antibodies (BsAb) to deliver the ribosome-inactivating protein, saporin, in the treatment of low-grade, end-stage, B-cell lymphoma. Two BsAb were used, each having one arm directed at saporin and one at the CD22 on target B cells. The BsAb, however, recognized different, non-overlapping epitopes on each molecule, a strategy which permits high-avidity double attachment of saporin to the target. The BsAb and saporin were pre-mixed at a molar ratio of 3:1 24 h before treatment and infused intravenously over a period of 1 h. Five patients have been treated, mostly with weekly doses of between 2 and 4 mg of saporin for a period of up to 6 weeks. Toxicity was minimal. Three complained of weakness and myalgia for 1 to 2 days after treatment, without objective neurological deficit or rise in serum creatine kinase. One patient produced an anti-mouse Fab' and an anti-saporin response. All patients showed a rapid and beneficial response to treatment. When present, circulating tumor cells were cleared (4/4 patients), ascitic and pleural effusions were eliminated (2/2 patients) and one patient with splenomegaly showed a marked reduction in tumor bulk. Malignant lymph nodes showed significant, but partial, shrinkage in all patients and finally marrow responded well with tumor clearance in biopsy material and impressive resolution of pancytopenia in some patients. While these responses were mainly short-lived, with tumor progression once the treatment was stopped, their speed and magnitude, and the relative lack of associated toxicity warrants further study of this treatment to determine maximum tolerated doses and therapeutic utility.
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PMID:Response of B-cell lymphoma to a combination of bispecific antibodies and saporin. 879 95

The paper presents new findings in favor of recognition of splenic lymphocytoma (SLC). This disease was characterized by A. I. Vorob'ev and M. D. Brilliant in 1982 in terms of detailed clinicomorphological features, prognosis and optimal treatment policy. The study included 52 patients (mean age 53 years) of which 36 were females and 16 males. They were followed up for 5.7 years, on the average. SLC manifested clinically by splenomegaly with minimally enlarged lymph nodes, morphologically by nodular lymphocytic proliferates in the spleen, bone marrow and liver, diffuse or diffuse-nodular proliferation in the lymph node. Peripheral blood contained middle-size lymphoid cells with round nuclei. SLC immunophenotype exhibits moderate or marked expression of CD22 and membrane immunoglobulins, the absence of CD5, CD23 and EM receptor, combination of CR1-/ CR2+. Paraprotein secretion was recorded in 49% of cases. There were frequent autoimmune reactions, especially against erythroid cells and platelets (42%). Optimal therapeutic policy is expectation and eventual splenectomy producing a persistent clinical effect in 94% of patients. In progressive disease long-term therapy with cyclophosphamide is recommended. Thus, SLC is a mature-cell lymphatic tumor growing as a rule in the spleen. Its prognosis in valid therapy is favourable.
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PMID:[Lymphocytoma of the spleen--a separate nosological form requiring a specific management procedure]. 892 72

Lymphomas of the marginal spleen zone are an entity recently considered as separate by the International Lymphoma Study Group. There are B-cell non Hodgkin's lymphomas (NHL) of low grade malignancy with a characteristic phenotype that allows to differentiate from mantle lymphomas and other B-cell lymphoproliferative syndromes. The case of a 69-year-old female patient admitted for abdominal pain due to large splenomegaly is reported. Pancytopenia and the presence of atypical large-sized lymphocytes with extensive cytoplasm and a rounded nucleus with indentations, reticulated appearing chromatin and one or several nucleoli were of note in the hemogram. Microscopic examination of the bone marrow demonstrated moderate-degree lymphocytary infiltration with grade I reticulin fibrosis. Laparotomy with splenectomy was performed. White pulp invasion with multifocal infiltration of the red pulp by lymphocytes of the same characteristics as those observed in the peripheral blood and bone marrow were observed on microscopic bone marrow examination. Immunophenotypic study of these lymphocytes was positive for CD19, CD20 and CD22 while being negative for CD5, CD10, CD23, CD25, CD11c and FMC7, the phenotype belonging to the lymphocytes of marginal spleen zone. Following splenectomy the patient recovered hemoperipheral counts and did not undergo additional treatment. The patient died due to septic shock of respiratory origin 4 months later. The clinical, morphologic and immunophenotypic features of marginal spleen zone lymphomas are reported with emphasis on the differences with other B-cell non Hodgkin's lymphomas of low malignancy.
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PMID:[Lymphoma of the marginal zone of the spleen. A case study]. 923 20


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