Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.24.11 (CD10)
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Mucosa-associated lymphoid tissue (MALT) lymphoma is listed in the new World Health Organization classification as extranodal marginal zone B-cell lymphoma of MALT. Most cases occur predominantly in adults and are rare in children or adolescents. We report, with a review of earlier literature, a MALT lymphoma of the lower lip in a 7-year-old immunocompetent child. The patient had a sessile, indurated, and firm mass on the lower lip without regional lymphadenopathy. Microscopically, dense lymphoid cell infiltrates composed of small- to medium-sized centrocyte-like cells and plasmacytoid cells infiltrating around scattered reactive germinal centers were observed, with formation of typical lymphoepithelial lesions. Immunohistochemical staining demonstrated positivity of atypical lymphoid cells for CD20, CD43, CD79a, and Bcl-2 and negativity for CD3, CD5, CD10, CD23, Ki-1, Bcl-10, and ALK. Cytoplasmic kappa light chain restriction was demonstrated. We conclude that this case represents the youngest patient with a MALT lymphoma of the lip.
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PMID:Pediatric mucosa-associated lymphoid tissue (MALT) lymphoma of lip: a case report and literature review. 1912 58

We report a 68-year-old Korean man presenting with asymptomatic erythematous polycyclic annular firm plaques on his back that spread to the right shoulder. Histopathologic examination showed dense, diffuse infiltrates involving the entire dermis, consisting of atypical lymphocytes with many centrocytes and a few centroblasts. Spindle-shaped cells with elongated, twisted nuclei containing dispersed chromatin were also seen. Immunohistochemical analysis showed that all of the cells were strongly positive for CD20, CD21, CD79a and CD45, while they were negative for CD3, CD5, CD10, CD23, CD35, CD43, CD45RO and CD68. The spindle cells were also negative for smooth-muscle actin, desmin, S-100 and CD34. They consistently expressed nuclear bcl-6, but did not express bcl-2, multiple myeloma-1 and p16. We diagnosed him with primary cutaneous spindle cell B-cell lymphoma (PCSBCL) and treated him with six cycles of cyclophosphamide, adriamycin, vincristine, prednisone and rituximab (R-CHOP) chemotherapy; his skin lesions disappeared completely. Immunohistochemical profiles suggest that PCSBCL is a variant of primary cutaneous follicle center lymphoma.
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PMID:Primary cutaneous spindle cell B-cell lymphoma with multiple figurate erythema-like manifestation. 1912 34

Follicular lymphoid hyperplasia (FLH) of the palate is a very rare benign, proliferative process, with only 20 cases published so far in the English language literature. We describe a case, in a 55-year-old Caucasian female, who developed a swelling in the left posterior hard palate. Bony involvement was absent. Importantly, the medical history was positive for a previous non-Hodgkin lymphoma. Following incisional biopsy, histological examination revealed a vaguely nodular lymphoid proliferation composed of small well-differentiated lymphocytes. The lymphoid follicles were positive for CD20, CD79a, CD10, CD21 and BCL6, while negative for BCL2. The parafollicular areas revealed positivity for CD3, CD5, CD30, and CD15. Both areas were CD45 positive. Subsequent immunoglobulin heavy chain gene rearrangement analysis revealed a polyclonal lesion. No further treatment was instituted, and the patient is currently being followed-up every three months. This report demonstrates that FLHs are especially important due to their clinical and, occasionally, microscopic resemblance to follicular lymphomas. Morphologic and immunohistochemical analyses and molecular studies are essential to achieve accurate diagnosis and to implement appropriate management.
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PMID:Follicular lymphoid hyperplasia of the palate: case report and literature review. 1913 28

The role of BCR/ABL isoforms and their relationship to leukemia phenotype have been of major concern. Atypical BCR/ABL mRNA transcripts lacking exon a2 have been reported in 12 cases of acute lymphoblastic leukemia (ALL) to date; among them, a b3a3 type transcript has been reported only once in the childhood ALL. Reported here is the case of a patient with Philadelphia-positive (Ph(+)) ALL expressing a b3a3 type transcript, a rare type of BCR/ABL mRNA lacking ABL exon a2 sequences. Bone marrow showed a hypercellular marrow with leukemic blasts positive for CD10, CD19, CD79a, and cytoplasmic mu, which is consistent with pre-B ALL. The G-banding and fluorescence in situ hybridization analyses indicated Ph(+). After the patient was diagnosed with ALL-L2, induction chemotherapy was performed and imatinib mesylate was thereafter given as the maintenance therapy. Sequencing analysis showed deletion of ABL a2 in the polymerase chain reaction product, which corresponded to a b3a3 fusion transcript. To our knowledge, this is the second report of an aberrant BCR/ABL product lacking ABL exon a2 in childhood ALL.
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PMID:BCR/ABL rearrangement with b3a3 fusion transcript in a case of childhood acute lymphoblastic leukemia. 1921 96

Epstein-Barr virus positive diffuse large B-cell lymphoma (EBV+ DLBCL) of the elderly is a rare B-cell lymphoproliferative disorder (B-LPD) that occurs in patients > 50 years with no known history of immunodeficiency or lymphoma. Patients present with moderate to severe clinical B-symptoms. These lesions show complete effacement of normal tissue/nodal architecture by large atypical lymphoid cells/immunoblasts and Hodgkin/Reed-Sternberg-like giant cells with variable amounts of inflammatory cells in the background. The ratio of neoplastic to inflammatory cells, degree of mitoses and necrosis can be quite variable; hence EBV+ DLBCL of the elderly was historically divided into low grade polymorphic and high grade monomorphic types. Further studies have shown both types to be different points in the spectrum of disease, and are all high grade lymphomas. The neoplastic large lymphoid cells show expression of CD20/CD79a and PAX-5, with variable expression of CD30, LMP-1 and EBNA-2, but CD15, CD10 and BCL6 are generally negative. Neoplastic cells show EBER positivity and high Ki-67 expression. Differential diagnoses include EBV+ B-LPD, classical Hodgkin lymphoma and EBV-DLBCL. EBV+ DLBCL of the elderly is highly aggressive with a median survival of 2 years. These patients are less responsive to standard chemotherapy compared with other B-LPD.
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PMID:Epstein-Barr virus positive diffuse large B-cell lymphoma of the elderly. 1925 22

An 88-year-old female was admitted to our hospital due to a right breast tumor and biclonal gammopathy (IgG-lambda and IgA-lambda). CT scan showed systemic lymphadenopathy. Tumor specimens were comprised of medium-sized atypical lymphocytes. Lymphoepithelial lesions and plasmacytic differentiation were observed. The atypical lymphocytes were positive for CD20, CD79a, Bcl-2, CD5 and negative for CD10, Cyclin D1. Moreover, the cells expressed IgG-lambda or IgA-lambda. We diagnosed the patient's disease as lymphoplasmacytic lymphoma (LPL). Although biclonal gammopathy with IgM elevation has been reported in LPL patients, this is the first case of IgG+IgA type biclonal gammopathy.
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PMID:[Lymphoplasmacytic lymphoma with a breast tumor and IgG+IgA type biclonal gammopathy]. 1940 26

We present a case of primary cutaneous diffuse large B-cell lymphoma, leg type, with an unusual clinical picture. A 41-year-old man presented with a 2-year history of slowly progressive plaques, nodules, and garland-like patches on his chest, right upper arm, and back. Complete staging investigations revealed no extracutaneous involvement. Histological examination of a nodule revealed a diffuse nonepidermotropic infiltrate mainly composed of large blast cells with features of immunoblasts and centroblasts and spindle cells seen at the periphery of the infiltrate. Histological examination of a garland-like lesion showed perivascular infiltrates composed predominantly of small lymphocytes admixed with only occasional large blasts. The blasts from the nodule and garland-like lesions and spindle cells identified in the nodule exhibited an identical phenotype: they stained positively for CD20, CD79a, and bcl-2 and tested negative for bcl-6, CD5, CD10, and TdT. CD35 revealed no networks of follicular dendritic cells. Widespread garland-like lesions are not a typical feature of primary cutaneous diffuse large B-cell lymphoma.
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PMID:Unusual clinicopathological presentation of primary cutaneous diffuse large B-cell lymphoma, leg type, with multiple nodules and widespread garland-like lesions. 1946 Dec 42

We report a case of extranodal CD20-positive peripheral T-cell lymphoma (PTCL). A 59-year-old man was admitted because of a right testicular mass in April 2006. CT scan revealed bilateral adrenal masses and he underwent right orchiectomy. The enlarged testis showed diffuse infiltration of large CD20-positive lymphocytes with slight CD3-positive cells. These cells were negative for CD10 and showed a high MIB-1 index. The pathological diagnosis was diffuse large B-cell lymphoma. He received R-CHOP, but developed brain involvement. He received whole brain radiotherapy following high-dose methotrexate, but he died of disease progression in August 2007. At autopsy, lymphoma cells were definitely positive for CD3 and negative for CD20. Monoclonal TCR gamma gene rearrangement was detected in the brain specimen without IgH rearrangement by PCR. The testicular tumor also showed the same clonal bands. Immunohistochemical re-evaluation of the testis showed CD20+, CD79a-, PAX5-, MUM1-, CD3 p+, CD5 p+, CD4-, CD8-, CD7 p+, granzyme B+, and TIA1+. Based on the clinical course and immunohistology, we finally diagnosed this case as extranodal PTCL-nos (not otherwise specified) with aberrant CD20 expression, which is extremely rare. The detection of gene rearrangement, plural immunohistochemical markers and knowledge of the possibility of CD20+ PTCL-nos are necessary for such cases.
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PMID:Extranodal CD20-positive peripheral T-cell lymphoma presenting with adrenal and testicular masses. 1948 2

A patient with history of B cell lymphoma treated with rituximab-based chemotherapy relapsed with a blastic CD4+/CD56+ neoplasm that was negative for CD20, CD79a and CD3. The relapse morphology and immunophenotyping were unusual and plasmacytoid dendritic cell (PDC) tumor enters the differential diagnosis. However, expressions of Oct-2 and CD10 in the relapse tumor were both more compatible with B cell than PDC lineage. Molecular investigations showed clonal rearrangements for both immunoglobulin heavy chain (IgH) and T cell receptor (TCR) gamma chain gene by polymerase chain reaction (PCR). Furthermore, a clonal relationship with the original B cell lymphoma was demonstrated for all PCR products. Our case illustrated the potential pitfalls and ambiguity of lineage classification based on morphology and immunophenotyping alone, especially for rare and poorly defined entities.
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PMID:A unique case of B cell lymphoma relapsing as CD4/CD56 blastic neoplasm. 1950 95

Primary presentation of intradural non-Hodgkin lymphoma is rare. Recently, B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) have been recognized as an important pathologic subtype. When MALT lymphomas present in the central nervous system (CNS), they are distinguishable from primary high-grade CNS lymphomas. We present the clinicopathologic features of 5 patients with primary CNS MALT lymphoma treated at our institution from 1999 to 2006. Four out of 5 patients were women, and all patients presented with headaches, focal motor deficits, or cranial nerve palsy. Radiologic studies demonstrated ill-defined dural masses in 3 and well-defined masses in 2 patients. Pathology revealed small to medium-sized cells with a moderate amount of cytoplasm and irregular nuclear borders, expressing pan B-cell markers (CD19, CD20, and CD79a) but lacking CD10, CD23, and cyclin D1, confirming low-grade MALT lymphoma. Plasma cells were encountered in all the biopsies with variable reactive T-cell infiltration. wedge chain restriction was seen in 3 patients. Therapy consisted of either surgical resection, whole-brain radiation, or systemic or intrathecal chemotherapy. There was no evidence of recurrence or systemic relapse in 4 patients at 4 years of follow-up. One patient died in 2 months, unrelated to CNS lymphoma. This case series illustrates the rare occurrence of low-grade dural B-cell lymphoma and the need to consider this entity in the differential diagnosis of CNS lesions.
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PMID:Primary central nervous system mucosa-associated lymphoid tissue lymphoma: case report and literature review. 1952 85


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