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)

We describe an 89-year-old woman who presented with prominent plasmacytosis mimicking plasma cell leukemia. The apparent serum M-protein level of > 7 g/dL of gamma mobility was revealed to be a polyclonal increase of immunoglobulins. The plasma cells in the peripheral blood expressed polyclonal surface/cytoplasmic immunoglobulins as well as CD19, CD30, CD38, and CD138 antigens but lacked CD10, CD20, CD25, and CD56. The bone marrow plasma cells showed the CD45+, CD19+, CD56-, MPC-1(-/+), and CD49e- immunophenotype, which was in clear contrast with the immunophenotypes of the neoplastic myeloma cells. Abdominal lymphadenopathy, splenomegaly, and a high level of soluble interleukin 2 receptor may have been reflections of an underlying lymphoproliferative disorder, potentially leading to the polyclonal proliferation of plasma cells.
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PMID:Polyclonal proliferation of plasma cells associated with marked hypergammaglobulinemia in an elderly patient. 1571 91

CD5, CD10, and CD23 are cell surface antigens used to distinguish B-cell disorders. The expression of these antigens and their clinical significance in Waldenstrom's macroglobulinemia (WM), an uncommon B-cell disorder, remains to be clarified. We therefore determined expression of CD5, CD10, and CD23 by flow cytometric analysis on bone marrow lymphoplasmacytic cells (CD19+ k/l light chain restricted) for 171 serially biopsied patients with findings of the consensus panel definition of WM. Importantly, we also correlated laboratory and clinical data, as well as existence of a familial history of a B-cell disorder in view of reports suggesting familial predisposition in WM. These studies demonstrated tumor cell expression of CD5, CD10, and CD23 in 15 of 171 patients (9%), 11 of 161 patients (7%), and 37 of 105 patients (35%), respectively. Coexpression of CD23 with CD5 or CD10 was common. Tumor Lymphoplasmacytic from 10 of 15 (66%) and 3 of 11 (27%) patients with WM that expressed CD5 and CD10, respectively, also showed expression of CD23 (P = 0.01 and P = 0.08, respectively). Among patients with CD23 expression, increased serum immunoglobulin (Ig) M levels were observed compared with patients without CD23 expression (P = 0.05). No differences in age at diagnosis; presence of adenopathy and/or splenomegaly; bone marrow involvement; serum IgA, IgB, and b2 macroglobulin levels; hematocrit; platelet count; or familial history of WM or a related B-cell disorder were observed among patients with and without CD5, CD10, and CD23 expression. These studies demonstrate that CD5, CD10, and CD23 are commonly found in WM and that their expression should not exclude the diagnosis of WM. Moreover, expression of CD23 may define a clinically distinct subset of patients with WM.
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PMID:CD5, CD10, and CD23 expression in Waldenstrom's macroglobulinemia. 1579 57

A 61-year-old man with no subjective symptom was admitted to our hospital for further examination of the causes of anemia (hemoglobin, 9.5 g/dL) and thrombocytopenia (platelets, 9.2 x 10(4)/microL), which had been pointed out in a medical checkup half a year previously. A bone marrow examination showed 73% lymphoid cells. Immunophenotyping of these cells were CD19+CD20+CD3-CD5-CD10-CD23-, and light chain restriction (kappa) was positive by fluorescence-activated cell sorting analysis. A computed tomography scan showed mild splenomegaly. To confirm the diagnosis histologically, we performed a splenectomy. Finally, we diagnosed the patient's disease as nonvillous splenic marginal zone lymphoma (SMZL). A month after the splenectomy, the white blood cell count was remarkably increased to 7 x 10(4)/microL with the blastic transformation of lymphoid cells. We first treated the patient with fludarabine and then with the CHOP regimen (cyclophosphamide, hydroxydaunomycin, vincristine [Oncovin], and prednisone), but the disease was so refractory that the patient died of the disease 13 months after the splenectomy. Immunohistochemical staining and a molecular examination for p53 were carried out with specimens from the splenectomy. We found overexpression of the p53 protein in lymphoid cells and a point missense mutation in codon 280 at exon 8 that changed AGA (Arg) to AGT (Ser). This case may indicate the existence of a more aggressive subset of SMZL, suggesting a reconsideration of the roles of splenectomy and p53 overexpression in the diagnostic and therapeutic approaches to patients with SMZL.
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PMID:Blastic transformation after splenectomy in a patient with nonvillous splenic marginal zone lymphoma with p53 overexpression: a case report. 1615 23

A 70-year-old Japanese man presented to our hospital with a 1-month history of progressive general fatigue and anorexia. A physical examination revealed severe anemic condition, mild persistent splenomegaly, and no palpable surface lymph nodes. He had pleural effusion and ascites, though no malignant cells were detected in the effusion. He eventually died without any diagnosis of his disease. Immunohistochemical staining of his tumor after autopsy showed atypical cells that were negative for epithelial membrane antigen (EMA), keratin (AE1/3), keratin-20, vimentin, factor VIII, leukocyte common antigen (LCA/T200; CD45), myeloperoxidase (MPO), terminal deoxynucleotidyl transferase (TdT), lysozyme, CD1a, CD3, CD4, CD10, CD15, CD20 (L26), CD21, CD23, CD34, CD43, CD56, CD68, CD79a, CD138, and EBER-1 in situ. Only a few scattered cells expressed CD30, but they showed no staining for anaplastic large-cell lymphoma kinase (ALK). A few scattered cells expressed S-100 antigen and the majority of cells dominantly expressed dendritic cell-associated antigens (CD35, FDC, Ki-M1p). In conclusion, we found this unknown primary tumor to be consistent with a follicular dendritic cell tumor with anaplastic features.
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PMID:Follicular dendritic cell tumor as an unknown primary tumor. 1738 Apr 43

Immunophenotyping in leukemia offers a precise delineation of the hematopoietic lineage and differentiation stage of the malignant cell. In this study, we used flow cytometry to determine the frequency of the immunologic types of acute lymphoblastic leukemia (ALL) in Moroccan children. We analyzed 100 samples from ALL patients within an age ranging from 6 months to 16 years presented over a 4-year period (1996 to 2000). Immunophenotyping allowed classification into 2 major categories: T-ALL (37%) and B-ALL (63%), with a higher percentage of males (69%). Comparison of the clinical characteristics showed that the frequency of splenomegaly was similar in B-ALL and T-ALL patients (53% and 47%, respectively). Hepatomegaly and mediastinal masses were more often associated with T-ALL (62% and 71%, respectively). Splenomegaly, hepatomegaly, and mediastinal masses were more frequent in immature than mature B-ALL, whereas the reverse was observed for T-ALL. Complete remission was obtained in 88% and 84% of B-ALL and T-ALL, respectively and relapse after 1 year occurred in 30% and 37% of cases, respectively. CD10 expressing B-ALL showed a slightly higher complete remission rate, whereas the reverse was observed for CD10 expressing T-ALL. The overall 5-year survival rate of ALL was 38%, whereas patients with B-ALL showed better survival than children with T-ALL.
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PMID:Immunologic profile and outcome of childhood acute lymphoblastic leukemia (ALL) in Morocco. 1776 1

A 72-year-old woman was referred to our hospital for evaluation of leukocytosis revealed by a medical examination. Her physical examination demonstrated no splenomegaly and no palpable lymph nodes. Her white cell count was 10,900/microl with atypical lymphocytosis (84.5%). Her hemoglobin concentration was 10.4 g/dl, and platelet count 151,000/microl. On peripheral blood smears, the atypical lymphocytes had a hairy cell-like appearance, and phase-contrast microscopic and transmission electron microscopic findings revealed the lymphocytes had many long surface microvilli. Flowcytometric analysis of peripheral blood lymphocytes identified expanded B-lymphocytes as having the IgG+, CD5- CD10- CD11c+ CD19+ CD20+ CD23- CD25- and CD103- cell surface phenotype. Serum electrophoresis disclosed polyclonal elevation of IgG and IgM (2620 mg/dl and 840 mg/dl, respectively). No light-chain restriction and a polyclonal VH gene rearrangement pattern indicated the polyclonal proliferation of B cells. The patient was a nonsmoker and had HLA-DR4, as in previous reports which have suggested an association between hairy B-cell lymphoproliferative disorder (HBLD) and HLA-DR4. No chromosome 3 abnormality was observed. These findings were consistent with the characteristics of HBLD, but differed in some respects from those of persistent polyclonal B-cell lymphocytosis (PPBL). Therefore, we diagnosed this patient as having HBLD.
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PMID:[Polyclonal B-cell lymphocytosis with hairy cell appearance: hairy B-cell lymphoproliferative disorder]. 1786 2

Intravascular lymphoma is an extremely rare, disseminated, and aggressive extranodal CD20+ non-Hodgkin's lymphoma characterized by the presence of lymphoma cells only in the lumina of small vessels. We report a 72-year-old woman with a diagnosis of intravascular lymphoma presented with splenomegaly and leukemic appearance in the peripheral blood smear. Her clinical course was rapidly deteriorated before the initiation of specific chemotherapy and finally died due to multiorgan insufficiency. Bone marrow biopsy revealed a characteristic infiltration of CD5, CD10 B-cell lymphoma. To our knowledge, this is the first reported case of a CD5, CD10 intravascular B-cell lymphoma with leukemic presentation in peripheral blood with multiple cytogenetic aberrations.
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PMID:Intravascular B-cell lymphoma with leukemic presentation: case report and literature review. 1807 38

CD5-negative chronic B cell lymphoproliferative disorders in leukemic phase (B-CLPD) are heterogeneous and relatively uncommon pathologies that often lack a histopathological definition because of the absence of accessible pathological tissue. We describe the clinical features and evolution-related variables of 156 patients with CD5/CD10-negative B-CLPD (median age 66 years, range 25-86). The median follow-up was 51 months (range 6-216), and overall 3- and 5-year survival was respectively 87 and 76%; 50 patients needed therapy at diagnosis, 56 during follow-up, and 50 remained untreated until the last control. A combined clinical, histological, cytomorphological, immunophenotypical, and cytogenetic diagnostic approach allowed the complete classification of only a minority of patients as being affected by splenic marginal zone or lymphoplasmacytic lymphoma; the majority of cases remained unclassifiable. Multivariate analysis showed that the clinicohematological variables adversely related to overall survival were serum LDH levels and age, whereas high serum LDH levels, hemoglobin levels of <11 g/dl, and splenomegaly related to treatment-free time (in "wait and see" cases); only splenomegaly related to time to progression (in treated patients). In conclusion, our retrospective study describes the clinical features and variables related to evolution in a large group of patients with CD5/CD10-negative chronic B-cell lymphoid leukemias and underlines the fact that a probable lymphoplasmacytic or marginal zone normal cell origin can be supposed in such leukemic forms, but never surely demonstrated.
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PMID:A multicenter retrospective clinical study of CD5/CD10-negative chronic B cell leukemias. 1838 27

A 48-year-old woman was referred to our hospital because of fever and general fatigue. Peripheral blood analysis showed a hemoglobin level of 82 g/l, a white blood cell count of 1.95 x 10(9)/l and a platelet count of 80 x 10(9)/l. There were 9% CD5-positive B-cells in peripheral blood and 35% CD10-positive B-cells in bone marrow. The patient had a high serum soluble interleukin-2 receptor (SIL-2R) level of 5,185 U/ml and splenomegaly. Lymphoproliferative disease was suspected, however monoclonal rearranged band of immunoglobulin heavy chain was not detected. She also showed hyperthyroidism, Graves' disease and then treatment with thiamazole started. However, the treatment was stopped because of agranulocytosis and she received subtotal thyroidectomy. After treatment for hyperthyroidism, serum SIL-2R level decreased to 504 U/ml and pancytopenia gradually improved. Fifteen months postoperatively, the percentage of CD5-positive B-cells in peripheral blood and CD10-positive B-cells in bone marrow decreased to 8% and 2%, respectively. This clinical course suggests that polyclonal B-cell proliferation was caused by hyperthyroidism.
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PMID:[Graves' disease with splenomegaly and pancytopenia, mimicking B-cell lymphoproliferative disease]. 1834 Oct 41

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


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