1/6. Expression of cell cycle regulating proteins in an unusual transformation of mantle cell lymphoma.We describe here two patients with mantle cell lymphoma (MCL) who after a few years, developed to the diffuse large cell lymphoma (DLCL) (anaplastic centrocytic lymphoma) growing in a diffuse sheets without the classical MCL component. In both the initial and second biopsy specimens, in each case, tumor cells were positive for cyclin d1, sIgM, sIgD, and CD5, but were negative for CD10 and CD23. In a study of immunoglobulin heavy chain (IgH) gene rearrangement, using the polymerase chain reaction (PCR) method, the products obtained from each paired biopsy tissue sample were the same size, and in one case had an identical sequence to the non-mutated VH gene. immunohistochemistry was used to examine the expression of p53, p27Kip1 and cyclin e. Interestingly, there was clear overexpression of p53 protein in case 1 but not in case 2, compared with other typical MCL cases. The expression of p27Kip1 in the second biopsies of each case was decreased compared with those in the initial biopsies. In case 2, however, p27Kip1 was clearly expressed in the first and second biopsies, in contrast to other typical MCL cases. Thus these 2 cases demonstrate not only that the variant form of MCL may arise de novo, but also that MCL may transform to DLCL at the time of relapse. Although the mechanism of tumor progression/transformation is still poorly understood, the overexpression of p53 or p27Kip1 may be linked to a cellular mechanism involved in the development of the variant form of MCL.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
2/6. Unusual case of leukemic mantle cell lymphoma with amplified CCND1/IGH fusion gene.We describe a case of leukemic mantle cell lymphoma (MCL) with complex karyotype and amplification of the CCND1/IGH fusion gene. Testing for the presence of t(11;14), the hallmark of MCL, revealed multiple copies of the fusion signals. We therefore conducted extensive molecular cytogenetic studies to delineate the nature and consequences of such an abnormality. We localized the amplification to the der(14)t(11;14) and to a der(2) chromosome in a form of interspersed chromosome 11 and 14 material. This resulted in high expression of cyclin d1 mRNA and the protein expressed independently of the cell cycle phase. CGH analysis revealed that the overrepresentation on chromosome 11 included chromosomal band 11q23 in addition to the CCND1 locus at 11q13. The band 11q23 harbors the ataxia telangiectasia mutated (ATM) gene recently proposed to be involved in the pathogenesis of MCL with high incidence of deletions in this locus. Using YAC 801e11, containing the ATM gene, we demonstrated several hybridization signals, suggesting that this region also formed part of the amplicon. This case also showed TP53 gene abnormalities: protein expression, monoallelic deletion, and a mutation in exon 5. The clinical course was aggressive, and the patient died within 6 months of presentation. This is to our knowledge the first description of amplification of the CCND1/IGH fusion gene in a human neoplasm, which may have played a role in the fulminating course of the disease in this patient.- - - - - - - - - - ranking = 0.25keywords = cycle (Clic here for more details about this article) |
3/6. Spontaneous (pathological) splenic rupture in a blastic variant of mantle cell lymphoma: a case report and literature review.Spontaneous (pathological) splenic rupture (SPSR) in hematological malignancies is rare. This report describes a 71-year-old male diagnosed with mantle cell lymphoma-blastic variant (MCL-BV) who experienced an SPSR a few days before the initial diagnosis. The patient underwent a splenectomy and recovered without incident. Partial remission was seen following several cycles of CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone). However, relapse was rapid, with leukemic meningitis occurring several months later. It was successfully treated by intrathecal methotrexate and cranial spinal radiation. A progressive lymphocytosis developed, which responded to rituximab. Lymphadenopathy and skin involvement ensued, followed by pneumonia and death. The literature on SPSR in patients with MCL-BV and other lymphoproliferative disorders showed similar clinical and postoperative findings. Clinical presentation included Kehr's sign and acute abdominal pain. Postoperative findings included blood in the peritoneal cavity, multiple splenic hematomas, splenic infarcts, and splenic necrosis. Most strikingly, the majority of the patients reviewed appeared to have undergone some type of blastic transformation. One or any combination of these findings that has been noted above in addition to a bleeding diathesis could be the foundation to SPSR. We recommend consideration of splenic rupture in patients with a lymphoproliferative disorder coupled with rapid progression of marked or massive splenomegaly.- - - - - - - - - - ranking = 0.25keywords = cycle (Clic here for more details about this article) |
4/6. Follicular conjunctivitis caused by a mantle cell lymphoma.BACKGROUND: lymphoma of the conjunctiva as part of a systemic disease is rare. The follicular appearance of the lymphocyte hyperplasia may mimic the clinical picture of infectious or allergic conjunctivitis. We report on a case of marked chronic follicular conjunctivitis, finally diagnosed as mantle cell lymphoma. history AND SIGNS: A 52-year old male with a history of epiphora for one year, presented with follicular conjunctivitis in both eyes, a nodal mass in the upper right eyelid and nuchal lymphadenopathy. No infectious or allergic aetiology could be found. Conjunctival biopsy revealed a mantle cell lymphoma. Magnetic resonance tomography showed a bilateral spread into the orbital cavity. Other sites of involvement were the epipharynx and the cervical, axillary and paratracheal lymph nodes. THERAPY AND OUTCOME: Chemotherapy with hyper-CVAD (cyclophosphamide, vincristine, doxorubicine, dexamethasone) was initiated. After 4 of 6 cycles, all clinical tumour manifestations had disappeared completely. CONCLUSIONS: Conjunctival lymphoma may clinically resemble follicular conjunctivitis. Conjunctival biopsy may lead to early diagnosis and initiation of therapy.- - - - - - - - - - ranking = 0.25keywords = cycle (Clic here for more details about this article) |
5/6. Multiple lymphomatous polyposis of the gastrointestinal tract.CONTEXT: Gastrointestinal multiple lymphomatous polyposis is a rare type of malignant lymphoma that has aggressive biological behavior, early systemic dissemination and poor prognosis. It is considered to be a manifestation of non-Hodgkin lymphoma and represents the gastrointestinal counterpart of mantle cell nodal lymphoma. OBJECTIVE: A case of gastrointestinal multiple lymphomatous polyposis is presented and the anatomopathological, clinical, diagnostic and treatment aspects of this unusual neoplasia are discussed. CASE REPORT: The patient was a 59-year-old white male with a complaint of asthenia, night sweating, alteration in intestinal habit and weight loss over the preceding two months. The physical examination showed pallid mucosa and a palpable mass in the epigastrium and mesogastrium. endoscopy of the upper digestive tract showed the presence of gastric and duodenal polyps. An opaque enema showed multiple polypoid lesions, especially in the cecum. A rectal biopsy revealed infiltration of the mucosa and submucosa by diffuse lymphoma consisting of small cleaved cells. Immunohistochemical study showed lymphocytes that expressed the antibody CD20 (L-26) and light-chain kappa (k) immunoglobulin, but not light-chain lambda (l) immunoglobulin. The patient presented a condition of acute intestinal obstruction with the presence of a mesenteric mass formed by agglutinated lymph nodes that surrounded the proximal ileum, thereby obstructing its lumen. He was submitted to a segmental enterectomy and gastrotomy with excisional biopsies of the gastric polypoid lesions. After two cycles of chemotherapy there was a worsening of the general state, with an increase in the dimensions of the abdominal masses and sepsis, accompanied by progressive respiratory insufficiency, leading to death.- - - - - - - - - - ranking = 0.25keywords = cycle (Clic here for more details about this article) |
6/6. Complete and durable remission of refractory mantle cell lymphoma with repeated rituximab monotherapy.We encountered a 53-year-old man with general fatigue. bone marrow investigations revealed an infiltration of CD20 CD5 CD23- cells and the presence of cyclin d1 lymphoid cells, leading to a diagnosis of mantle cell lymphoma, clinical stage IV. The first 2 lines of chemotherapy, CyclOBEAP (cyclophosphamide, vincristine, bleomycin, etoposide, doxorubicin, and prednisolone) and fludarabine-cyclophosphamide, produced only a transient decrease in serum lactic dehydrogenase levels, without a clinical remission. Because of the persistence of bone marrow hypoplasia, monotherapy with 375 mg/m2 rituximab was administered. The pancytopenia gradually improved, and a complete remission was obtained after 4 cycles of rituximab. The patient remains in complete remission 21 months after the third rituximab therapy for maintenance.- - - - - - - - - - ranking = 0.25keywords = cycle (Clic here for more details about this article) |