Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.11 (CD10)
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We report the first case of primary mucosa-associated lymphoid tissue (MALT) lymphoma of the vagina, the diagnosis of which is supported by genetic and immunophenotypic studies. A 65-year-old, para 2 woman presented to our hospital in July 1997 with a history of prolonged vaginal discharge. Although cytologic examination suggested possible malignancy, a biopsy of the vaginal wall was diagnosed as chronic inflammation. In June 2000, she underwent gynecologic examination because of anuria. Excisional biopsy revealed subepithelial infiltration of atypical lymphoid cells that stained for CD20, CD79a, and BCL-2; stained weakly for IgM; and did not stain for CD3, CD5, CD7, CD10, CD56, CD23, and IgD, suggesting marginal zone B-cell lineage. Monoclonality was detected by Southern blot analysis, and this patient was finally diagnosed as having primary MALT lymphoma of the vagina. She received 3 cycles of chemotherapy (THP-COP) and concurrent radiation to the whole pelvis. The patient is alive and well 40 months after treatment. Because the vagina is one of the mucosa-associated tissues, MALT lymphoma, though rare, must be included in the differential diagnosis of the vaginal neoplasms.
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PMID:A case of primary mucosa-associated lymphoid tissue lymphoma of the vagina. 1534 21

Biphenotypic acute leukemias (BALs) are uncommon. Most are of myeloid-B-cell or myeloid-T-cell lineage. We report herein a 70-year-old man with an unusual acute leukemia where the blasts expressed both B- and T-lymphoid markers. He presented to us with an enlarging cutaneous tumor. The presenting peripheral blood and bone marrow aspirate showed 40% and 90% blasts, respectively, which were negative for the usual cytochemical stains. The flow cytometric analysis revealed that the blasts were positive for CD19, CD20, CD22, cytoplasmic (Cyt) CD79a, CD10, Cyt CD3, CD5, CD7, CD4, HLA-DR, TdT, and were negative for myeloid markers. According to the scoring system from the European Group for the Immunological Characterization of Acute Leukaemias (EGIL), this case was an unequivocal B-cell/T-cell BAL. Conventional cytogenetic analysis revealed 46XY [t(4;11)(q31;q13), add(8)(q24), der(9)del(9)(p21)del(9)(q32q34), -13, +mar] in all 25 metaphases analyzed. Fluorescence in situ hybridization (FISH) and polymerase chain reaction (PCR) for 11q23 rearrangements as well as t(9;22) were negative. PCR for both TCR-gamma and IgH gene analyses revealed polyclonal rearrangements. We postulate that this case of BAL might have arisen from the putative common lymphoid progenitor cell.
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PMID:Acute lymphoblastic leukemia with the phenotype of a putative B-cell/T-cell bipotential precursor. 1538 7

We describe two elderly patients with follicular lymphoma (FL) involving the skin and superficial soft tissues, with a striking proliferation of follicular dendritic cells (FDC). In addition, one patient had bone marrow involvement by FL. Histopathologically, the most remarkable feature in both cases seen at low magnification was a striking pallor of the constituent cells, which were arranged in fascicles, whorls, and round islands. The majority of the cells had the typical cytologic features of FDCs. They were intimately intermingled with centroblasts and centrocytes. A large amount of the clear cytoplasm and the pale nuclei of FDCs, which predominated in the tumors, caused the striking overall pallor of the lesions. Small reactive lymphocytes were scattered between the fascicles. A vague follicular growth pattern was seen only focally. The mantle zones were markedly reduced or absent so that the follicles were seen lying unseparated. The close intermixture of the FDCs and the germinal center cells was responsible for the FDCs appearing to be decorated with B-associated marker, and the germinal center cells seemed to be stained to some degree with FDC-markers. The tumor bulk demonstrated a diffuse and strong reaction with CD10, CD20, CD21, CD35, and stained weakly with CD79a. Fascin and CD23 showed only a weak and focal staining pattern. Bcl-2 decorated large centroblasts and small reactive T-cells. The tumor bulk was negative for actin, EMA, cytokeratins, vimentin, desmin, and factor XIIIa. The proliferative index was rather low; MIB-1 mainly decorated large centroblasts. No monoclonal rearrangement of IgH genes was detected. Epstein-Barr virus was not identified. Electron microscopy revealed typical features of FDCs intermingled with germinal center cells. Such cases may represent a diagnostic pitfall, as FDC overgrowth can mask FL and give the neoplasm the appearance of FDC sarcoma/tumor. We believe that, in both cases, the FDC proliferation had a reactive character.
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PMID:Follicular lymphoma of the skin and superficial soft tissues associated with a prominent follicular dendritic cell proliferation: an unusual pattern which may represent a diagnostic pitfall. 1546 4

We report a case of primary effusion lymphoma (PEL) in a 75-year-old woman without human immunodeficiency virus or hepatitis C virus, which presented as fever, chest pain, and pericardial effusion. The lymphoma cells were positive for CD20 and CD79a, and were negative for CD3 and CD10. Genomic human herpes virus 8 (HHV-8) and Epstein-Barr virus were not detected in the lymphoma cells. Cytogenetic analysis showed complex abnormalities by the G-banding technique, and spectral karyotyping (SKY) analysis provided more detailed characterization of the chromosomal aberrations, including t(1;22)(q21;q11) and t(14;17)(q32;q23). We did not detect C-MYC gene rearrangement or BCL-2 expression. She was treated successfully with six courses of the CHOP regimen. The present case demonstrated a rare category of PEL that is not associated with HHV-8 or C-MYC gene rearrangement. In addition, SKY analysis disclosed cryptic chromosomal abnormalities involving 1q21 and 17q23.
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PMID:Primary effusion lymphoma of the pericardial cavity carrying t(1;22)(q21;q11) and t(14;17)(q32;q23). 1558 55

Myeloid sarcoma (MS) is a localized extramedullary mass of immature granulocytic cells that usually occurs in patients with acute myeloid leukemia (AML) or myeloproliferative disorders. It may rarely precede peripheral blood or bone marrow involvement, presenting a diagnostic challenge. Although MS may be found in any location, an intraoral occurrence is rare. In this report we describe a rare case of a patient with nonleukemic MS of the maxillary gingiva. The histologic specimen was first interpreted as non-Hodgkin's lymphoma. The correct diagnosis was reached after extensive immunohistologic studies. The malignant cells were myeloperoxidase positive, lysozyme positive, CD45+, CD68+, CD3-, CD10-, CD19-, CD20-, CD30-, CD34-, CD56-, CD79a-, S100-, and chloroacetate esterase negative. Induction therapy with FLAND (fludarabine, Ara-C, mitoxantrone, and dexamethasone) was started, but the patient did not achieve a remission. Some weeks later, the patient presented pleural effusion and paralysis of the seventh cranial nerve on the left side. She died a few days later. The present case indicates the importance of a correct initial diagnosis for adequate therapy, which is often delayed because of a high misdiagnosis rate. If the MS is treated without intensive chemotherapy for AML as soon as possible, the prognosis will be poor.
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PMID:Myeloid sarcoma occurring in the maxillary gingiva: a case without leukemic manifestations. 1576 82

We report a unique case of de novo composite lymphoma in the tibia of a 35-year-old man who presented with increasingly frequent and intense pain in the right upper leg. He was otherwise healthy without significant medical history. A plain radiograph of the right leg showed a permeative lesion with alternating areas of radiolucency and radiodensity in the upper third of the tibia. Magnetic resonance imaging showed a large, heterogeneous enhancing lesion involving the medullary and cortical bone of the proximal tibia with cortical disruption and extension into the adjacent soft tissue. A biopsy showed sheets and clusters of large cells, punctuated by clusters of small, irregular lymphocytes. Flow cytometry and immunohistochemical analysis showed composite lymphoma: diffuse large B-cell lymphoma (DLBCL) and peripheral T-cell non-Hodgkin lymphoma with predominantly small cell morphologic features. The DLBCL expressed CD19, CD20, CD79a, CD5, CD10, CD23, CD38, CD117, bcl-2, and bcl-6, with monotypic expression of immunoglobulin kappa light chain. The T cells expressed CD2, CD3, CD5, CD7, and CD8, with partial loss of CD4. Clonal rearrangement of T-cell receptor gamma chain gene was found. Neither the large B cells nor the small T cells expressed Epstein-Barr virus-encoded RNA. Physical examination and radiologic studies showed no evidence of lymphadenopathy, organomegaly, or other mass lesions in the body. No peripheral lymphocytosis or bone marrow involvement was present.
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PMID:Composite B-cell and T-cell non-Hodgkin lymphoma of the tibia. 1584 45

To evaluate the sensitivity and specificity analysis of the lineage related antibodies in acute leukemia immunophenotyping by flow cytometry (FCM), immunophenotyping in 184 patients with acute leukemia was performed by FCM analysis. The results showed that in the lineage-related antibodies of acute myelocytic leukemia (AML), the sensitivity of CD13 and CD33 was higher (95.5% and 91.2%, respectively), the specificity of them was deficient (72.5% and 62.2%, respectively); the sensitivity of MPO was low (69.1%), but the specificity was high (100%); the sensitivity and specificity of CD117 were high (88.2% and 100%, respectively); the sensitivity of CD14 and CD15 was low (18.4% and 27.2%, respectively); the specificity of CD14 with monocytes was high. As the lineage-related antibodies of B-lineage ALL were concerned, CD19 showed high sensitivity and low specificity (100% vs 83.4%); the sensitivity and specificity of CD79a (96.4% vs 100%) and CD22 (100% vs 100%) were high; the sensitivity and specificity of CD10 (53.6% vs 82.5%) and CD20 (70.4% vs 87.5%) were low. In T-lineage ALL, the specificity of CD3 was high (97.5%), but the sensitivity was below the mark (80.0%); the sensitivity of CD7 was high (100%), but the specificity was low (77.9%); while the sensitivity and specificity of CD5, CD2 and CD1a were all deficient. In conclusion, the sensitivity and specificity analysis of the lineage-related antibodies in acute leukemia immunophenotyping are coincident with St Jude immunophenotyping project. It seems only that CD117 is superior to MPO in defining AML, but the sensitivity and specificity analysis of CD22 and CD79 are similar in defining B-lineage ALL, therefore, anyone of them may be selected as your need.
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PMID:[Sensitivity and specificity analysis of the lineage related antibodies in acute leukemia immunophenotyping by flow cytometry]. 1585 4

Histological, clinical and immunohistochemical analysis of 6 cases of primary liver lymphomas (PLL) are presented. PLL represents 4.3% of primary malignant liver tumors diagnosed in our department. The patients were relatively young people, who despite the presence of a large tumor, were in good general health status. There were no signs of scirrhosis, and cancer markers were normal. All lymphomas were CD20, CD79a, BAX positive, CD3, CD30, EMA, CD10, CD5, CD59, c-myc, Bcl2, EBV(LMP), CK negative. The proliferation index (Ki67) was high, ranging from 50-100%. In two cases positive staining for Bcl6 and in another one for cyclin D1 was obtained. The major histological type of the tumor was diffuse large B-cell lymphoma. Positive immunohistochemical results with BAX and the lack of Bcl2, c-myc and CD59 are associated with better prognosis. We have not confirmed the value of Bcl6 and CD10 stains as a predictor of poor outcome. Despite clinically advanced stage at the time of diagnosis, if treated appropriately, the primary lymphoma of the liver has relatively good prognosis (five of our patients are alive).
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PMID:Primary lymphoma of the liver -- morphological and clinical analysis of 6 cases. Success of aggressive treatment. 1587 91

The transcription factor PU.1 has been shown to be crucial for the early stages of B cell development but its function at later stages of B cell development is less well known. We observed previously that PU.1 is expressed uniformly throughout the mature pre-plasma cell B cell population, the only exception being a subpopulation of germinal centre (GC) cells which showed exceptionally high expression of PU.1. This suggested that PU.1 may also have a role in GC B cell biology. To test this hypothesis and to screen for possible genes regulated by PU.1, we first evaluated semi-quantitatively the possible co-expression of PU.1 with proteins known to be upregulated or downregulated during GC B cell development. Normal lymphoid tissues and 255 B cell non-Hodgkin lymphomas of putative GC B cell origin were evaluated. PU.1 expression was positively associated with CD10 (p < 0.0001), CD20 (p = 0.043), CD22 (p = 0.005), CD79a (p = 0.024) and Bcl-6 (p < 0.0001) and negatively associated with cytoplasmic immunoglobulin light-chain expression (p = 0.036) in diffuse large B cell lymphoma. Identical or nearly identical associations were found in follicular lymphoma. Since CD20 is known to be partly regulated by PU.1 and putative PU.1-binding sites have been described in the regulatory regions of the CD22, CD79a and CD10 genes, we looked for putative PU.1 binding sites in the BCL6 promotor. Four such putative PU.1 binding sites were identified. Further analysis by gel-shift electromobility essay showed that PU.1 protein binds to three of the four putative binding sites in the BCL6 promotor. PU.1 and Bcl-6 were also found to be upregulated in centroblasts in the normal GC, but jointly downregulated in a subpopulation of centrocytes. Our findings support the contention that PU.1 may also have an important role in GC B cell development.
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PMID:PU.1 protein expression has a positive linear association with protein expression of germinal centre B cell genes including BCL-6, CD10, CD20 and CD22: identification of PU.1 putative binding sites in the BCL-6 promotor. 1684 70

We report an autopsy case of malignant lymphoma of the cranial vault. The patient was an 85-year-old woman who exhibited a painless subcutaneous scalp lump associated with no neurological abnormalities. CT scan and magnetic resonance imaging of the head showed an extra-intra cranial isodensity lesion of the cranial vault. Autopsy revealed that the tumor was composed of medium-sized cells which were immunoreactive with CD45, CD20, CD79a, and CD10, and a diagnosis of peripheral B cell lymphoma, Burkitt-like, was made. Non-Hodgkin lymphoma originating from the cranial vault is extremely rare; a search of the English literature revealed only 16 previously reported cases. Herein, we review this singular calvarial lymphoma.
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PMID:Primary peripheral B cell lymphoma, Burkitt-like, of the cranial vault. 1598 33


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