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)

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

A 54-year-old woman with leukocytosis, was referred to our clinic in February 1982. Based on findings of pancytosis, high NAP score, high serum vitamin B12, increase in total red cell volume and splenomegaly, she was diagnosed as having polycythemia vera (PV). Since then, she has been treated with pipobroman, hydroxycarbamide and phlebotomy. Leukocytosis with increase in blastic cells and thrombocytopenia was noted in August 1995, and she was admitted to our hospital. Since the blastic cells were CD10(+)19(+)20(+), she was diagnosed as having acute lymphoblastic leukemia and treated with vincristine and prednisolone, resulting in remission. This case suggests that PV is a disease of multipotent stem cells including those with a lymphoid lineage.
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PMID:[Polycythemia vera progressing to acute lymphoblastic leukemia after 13 years]. 899 30

We report the case of a 78-year-old man in whom routine physical examination revealed cervical adenopathy and splenomegaly. Peripheral blood showed a normal white blood cell count with an absolute lymphocytosis, which included a population with slightly indented nuclei. Lymph node biopsy showed morphology compatible with mantle cell lymphoma. Bone marrow biopsy showed replacement by a lymphoid proliferation composed of lymphocytes with features similar to those found in the peripheral blood. Immunophenotypic analysis of both peripheral blood and lymph node showed positivity for CD19, CD20 and CD22, with lambda light chain restriction. Tests for CD5 and CD10 were negative. Cytogenetic analysis and polymerase chain reaction studies confirmed the presence of t(11,14) supporting a diagnosis of mantle cell lymphoma. This unusual case of CD5-negative mantle cell lymphoma exemplifies the importance of combined molecular, cytogenetic, and morphologic evaluation when confronted with a lymphoma having an atypical phenotype.
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PMID:CD5 negative diffuse mantle cell lymphoma with splenomegaly and bone marrow involvement. 963 25

A novel human EBV-negative B-cell line, designated DOBIL-6, was established from a patient with non-secretary myeloma. The DOBIL-6 cell has cytoplasmic gamma protein and expresses CD19, 20, 38, 45RO, VLA-4 and PCA-1 antigens, but lacks CD10, 45RA and VLA5 antigens. Chromosome analysis showed that DOBIL-6 cells had many complex structural abnormalities, including t(11;4) (q13;q32), which were consistent with that of the fresh tumour cells. Interestingly, abundant interleukin-6 (IL-6) and parathyroid hormone-related protein (PTHrP) accumulated in the culture supernatant of DOBIL-6 cells. Hypercalcaemia and splenomegaly associated with plasma cell proliferations which resulted in the expansion of the light zones in the follicles were observed in DOBIL-6 transplanted nude mice. RT-PCR analysis detected mRNA for PTHrP, and IL-6 as well as its receptor (GP80) in DOBIL-6 cells. Treatment of the DOBIL-6 cells with neutralizing anti-IL-6 antibody inhibited their growth in a dose-dependent manner, whereas the addition of exogenous IL-6 stimulated it in serum-depleted conditions. These findings suggest that both IL-6 and PTHrP are produced in DOBIL-6 cells, and that IL-6 promotes its growth by an autocrine mechanism. Since IL-6 is known to stimulate not only the growth of B-cell neoplasms but also osteoclastic bone resorption by cooperating with PTHrP, this simultaneous production of IL-6 and PTHrP might be synergistically linked and play a role in the development of hypercalcaemia of the patient. The DOBIL-6 cell is a useful tool to clarify the mechanism of hypercalcaemia associated with mature B-cell neoplasms.
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PMID:A novel mature B-cell line (DOBIL-6) producing both parathyroid hormone-related protein and interleukin-6 from a myeloma patient presenting with hypercalcaemia. 967 42

A 49-year-old man was admitted to our hospital for investigation of splenomegaly and lymphocytosis. He had no significant past history and was not a smoker. Physical examination revealed massive splenomegaly and no palpable superficial lymph nodes. Hematological examination showed a hemoglobin concentration of 10.5g/dl, a platelet count of 9.8 x 10(4)/microliter, and a leukocyte count of 21.2 x 10(3)/microliter with 70% abnormal lymphocytes. In May-Giemsa stained blood films, the abnormal lymphocytes had round nuclei, abundant, pale cytoplasm, and slightly serrated edges. Phase-contrast microscopic and scanning electron microscopic examinations revealed many long surface villi. Tartrate-resistant acid phosphatase activity in these cells was negative. The abnormal lymphocytes had a CD5-, CD10-, CD11a+, CD11c+, CD19+, CD20+, CD22+ phenotype. These features were similar to those described for a variant form of hairy cell leukemia (HCL-Japanese variant). However, studies of Ig gene rearrangement and expression of sIg revealed a polyclonal proliferation of B cells. On the basis of these findings, this case was diagnosed as hairy B-cell lymphoproliferative disorder, a recently described condition characterized by polyclonal B-cell lymphocytosis and features resembling HCL-Japanese variant. Serological assays for antibodies against Epstein-Barr virus suggested a past infection. Splenectomy alleviated the anemia and thrombocytopenia, but not the lymphocytosis.
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PMID:[Polyclonal B-cell lymphocytosis with clinical and hematological features resembling hairy cell leukemia]. 975 Apr 56

A 72-year-old man was referred to our hospital because of lymphadenopathy, splenomegaly, and leukocytosis. His WBC count was 54,300/microliter, with 89.6% atypical lymphocytes two to three times the diameter of red blood cells, cleaved nuclei, and one or two nucleoli. A lymph node specimen revealed a vaguely nodular pattern, and the diagnosis of mantle cell lymphoma (MCL) was made. The lymphoma cells appeared smaller and more mature than the leukemic cells. The phenotype of the peripheral blood and the lymph node cells was CD5+ CD10- CD19+ CD20+ and the same rearranged JH bands were detected, suggesting that their lymphocytes were of the same origin. In addition, the phenotype of the leukemic cells was CD23+ CD38+ CD43- CD44+ FMC-7+ micro+ chi+. Cytogenetic analysis revealed complex anomalies but not t(11; 14). Cyclin D1 protein was not detected. Because the lymphocyte morphology of the peripheral blood and lymph nodes was discordant, we speculated that variant large cells had proliferated mainly in the peripheral blood. The patient achieved a partial response after 6 courses of CHOP regimen, and was then placed on a COP regimen. He seemed to have MCL, but the following findings were unusual: marked lymphocytosis at initial presentation, discordant morphology, CD5+ CD10- CD23+ CD43- phenotype with neither t(11; 14) or cyclin D1 over-expression.
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PMID:[Mantle cell lymphoma with marked lymphocytosis at the presentation and with discordant morphology between the lymph node and peripheral blood]. 979 5

We report a case of mantle cell lymphoma in leukemic phase, which was diagnosed by a bone marrow biopsy performed as part of a workup for chronic anemia in a patient without lymphadenopathy. The patient, a 79-year-old man with diabetes mellitus, hypertension, chronic renal failure, congestive heart failure, and atherosclerosis, presented with claudication. On admission, he also had an 8-month history of anemia, during which time he experienced a 18-kg weight loss. On presentation, the patient had normal vital signs, anemia, leukocytosis (as well as an absolute lymphocytosis), and splenomegaly; as mentioned, lymphadenopathy was absent. A bone marrow biopsy showed an increase in small to intermediate-sized, slightly irregular lymphocytes in interstitial nodules. Flow cytometric immunophenotyping of the bone marrow identified a monoclonal population of cells, representing 25% of cells within the bone marrow, with expression of CD19, CD20, immunoglobulin M/D, lambda light chain, HLA-DR, and CD5; reactions for CD10 and CD23 were absent. Based on morphologic and immunophenotypic analysis of the bone marrow, as well as morphologic review of the peripheral blood smear, a diagnosis of mantle cell lymphoma involving the bone marrow and in leukemic phase was made. Subsequent polymerase chain reaction analysis of DNA from peripheral blood identified a population of cells with the bcl-1 rearrangement. This case is unique in that the diagnosis of mantle cell lymphoma was made without lymph node or spleen analysis and the patient, although exhibiting bone marrow and peripheral blood involvement by mantle cell lymphoma at presentation, did not have lymphadenopathy.
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PMID:Leukemic phase of mantle cell lymphoma presenting as anemia: diagnosis by combining flow cytometry and cytomorphology. 982 32

An 88-year-old Japanese woman with splenomegaly, but without lymphadenopathy, was admitted because of epigastric distress. Laboratory data disclosed an RBC of 310 x 10(4)/microliter, Hb of 10.1 g/dl, Ht of 30.6%, Plt count of 9.8 x 10(4)/microliter, and WBC of 4,470/microliter with 38% abnormal lymphocytes. Peripheral blood films revealed lymphocytes with thin, short cytoplasmic villi, condensed nuclear chromatin, and small nucleoli. The lymphocytes stained negative for tartrate-resistant acid phosphatase. Also, immunophenotyping was positive for expression of the cell surface markers CD19, CD20, IgG, kappa and HLA-DR, but not for CD5, CD10, CD11c, CD23, CD25, CD38, or CD103 antigens. Chromosomal analysis of peripheral blood cells disclosed the 46, XX, del(7), (q32) aberration. A splenectomy was performed simultaneously with partial colon resection because of a mucinous carcinoma found in the transverse colon. Histologic examination of resected spleen tissues revealed a distinctive pattern of white pulp infiltration by lymphoma cells. The histologic findings and clinical data were consistent with the features of splenic lymphoma with circulating villous lymphocytes. Our patient exhibited a relatively benign clinical course, and was being followed on an outpatient basis with no additional therapy.
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PMID:[Splenic lymphoma with villous lymphocytes expressing chromosomal abnormalities]. 1035 43

In order to assess the age-related changes in CD10 and CD19 fluorescence intensity (FI) the present study analyzed by flow cytometry 56 sternal biopsies from 'normal' infants, children and adults undergoing cardiac surgery. The CD10(+weak) subset was predominant in all age groups, representing approximately 50% of the bone marrow (BM) lymphoid cells in children younger than 4 years. Both CD10+ subsets significantly decreased with age but their ratio did not differ significantly. Moreover, the intensity of CD10 and CD19 fluorescence in the strong and weak subsets did not vary with age. The CD19 intensity was significantly higher in CD10(+weak) than in CD10(+strong) cells. In addition, we classified as CD10(+weak) or CD10(+strong) the leukemic cells from BM aspirates of 117 patients with common acute lymphoblastic leukemia (cALL) (78 children and 39 adults). A higher frequency of cases expressing the CD19+ CD10(+strong) phenotype was observed both in children and adults. Children of the CD10(+weak) group tended to be older than those of the CD10(+strong) group (median = 7 vs. 4 years, P = 0.07), and presented a significantly higher frequency of splenomegaly (93.7 vs. 55%, P = 0.04), which was massive in about 60% of these cases. Among adults, a significantly higher frequency of cases expressing the CD10(+weak) phenotype was observed in females. No other clinical or biological difference was detected between the two groups either for children or adults. Concerning the treatment outcome, we did not observe significant differences in complete remission rate (CRR) or in disease free survival (DFS) among the 32 children and 28 adults analyzed. Finally, we compared the CD10 and CD19 intensity in normal and leukemic BM. Overexpression of either or both antigens in leukemic cells was observed in 42.4% of the cALL cases. In these cases, using cut off values of 110 afu for the CD10 FI and of 100 afu for the CD19 FI, the detection of leukemic cells was possible at levels of 0.2% based on CD10 analysis, of 0.6% based on CD19, and 0.02% when both antigens were overexpressed. In conclusion, we demonstrated that the heterogeneity of CD10 and CD19 fluorescence intensity is of no clinical relevance in cALL, although its study may be helpful for the diagnosis and the detection of minimal residual disease.
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PMID:CD10 and CD19 fluorescence intensity of B-cell precursors in normal and leukemic bone marrow. Clinical characterization of CD10(+strong) and CD10(+weak) common acute lymphoblastic leukemia. 1037 58

We present the clinicopathologic findings and survival data on 10 patients with acute lymphoblastic leukemia (ALL) and a rare t(8;14)(q11.2;q32). There were five male and five female patients, nine Caucasians and one Black, aged 4-17 (median 10.9) years. Three had Down syndrome. Eight (80%) patients had a white blood cell (WBC) count <50 x 109/l at presentation. No patient had central nervous system involvement or a mediastinal mass. Two patients had concurrent splenomegaly and hepatomegaly. Adenopathy was absent in four, minimal in three, moderate in one and prominent in two patients. All eight cases where immunophenotyping was performed by flow cytometry showed a B-precursor phenotype with expression of CD10 (CALLA). Only one case exhibited t(8;14)(q11.2;q32) as the sole karyotypic abnormality. Three patients were classified as standard-risk and seven high-risk by NCI (National Cancer Institute) consensus risk group categories. All patients achieved complete remission and seven patients were in complete continuous remission (CCR) after chemotherapy designed for B-precursor ALL. Three patients relapsed after 23.5, 31.3 and 32.1 months of EFS; the first patient also had t(9;22)(q34;q11), the second had a WBC count of 126 x 109/l at presentation while the third patient had no high risk features except for age 10 years. Thus, from our data, the t(8;14)(q11.2;q32) does not appear to confer an increased risk of relapse. Further observations are needed to confirm this conclusion.
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PMID:Acute lymphoblastic leukemia with an unusual t(8;14)(q11.2;q32): a Pediatric Oncology Group Study. 1148 May 76


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