Plasmablastic lymphoma with unfavorable chromosomal abnormalities related to plasma cell myeloma: A borderline case between plasmablastic lymphoma and plasmablastic plasma cell myeloma
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ABSTRACT:
SUBMITTER: Aoyama Y
PROVIDER: S-EPMC6144273 | biostudies-literature | 2017
REPOSITORIES: biostudies-literature
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Plasmablastic lymphoma with unfavorable chromosomal abnormalities related to plasma cell myeloma: A borderline case between plasmablastic lymphoma and plasmablastic plasma cell myeloma
Aoyama Yumi Y Tsunemine Hiroko H Kodaka Taiichi T Oda Nao N Matsuoka Hirofumi H Itoh Tomoo T Takahashi Takayuki T
Journal of clinical and experimental hematopathology : JCEH 20170101 1
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Project description:Plasmablastic lymphoma (PbL) is a rare and aggressive B-cell malignancy with large neoplastic cells, most of them resembling plasmablasts that have a CD20-negative phenotype. Although initially described as being associated with HIV, over the years it has also been identified in patients with solid organ transplant and immunocompetent patients. Little is known about molecular basis that drives PbL, and still its diagnosis remains challenging given its rarity. However, proper recognition of its clinical characteristics, localization, and morphological features can establish a correct diagnosis of PbL within the spectrum of CD20-negative large B-cell lymphomas (LBCLs). PbL is characterized by CD20 and PAX5 negativity together with the expression of CD38, CD138, MUM1/IRF4, Blimp1, and XBP1 plasmacytic differentiation markers. It is usually associated with Epstein-Barr virus infections, and MYC gene rearrangements. PbL should be carefully differentiated from other CD20-negative B-cell neoplasms, ie, primary effusion lymphoma, anaplastic lymphoma kinase-positive (ALK) large B-cell lymphoma, and LBCL in human herpesvirus 8-associated multicentric Castleman disease. Despite our improved understanding of this disease, its prognosis remains dismal with short overall survival. There is no standard of care for this entity. Several chemotherapy combinations have been used with hardly any differences on its outcome. Thus, new approaches with the addition of novel molecules are needed to overcome its poor prognosis. Our current understanding and knowledge of PbL relies primarily on case reports and small case series. In this review, we revise through an extensive literature search, the clinical and biological characteristics of this entity, and the potential therapeutic options.
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Project description:Plasmablastic lymphoma (PBL) is a rare aggressive lymphoma. Although it was first described in HIV- (human immunodeficiency virus-) infected patients, PBL has been diagnosed in patients with other immunodeficiencies as well as in immunocompetent patients. PBL immunohistochemically expresses plasmacytic markers and lacks pan B-cell markers. The cells of origin of PBL are considered to be plasmablasts. MYC gene rearrangement and MYC overexpression are frequently found in PBL, but the pathogenesis of PBL is yet to be elucidated. Here, we report a case of composite lymphoma of PBL and diffuse large B-cell lymphoma (DLBCL); that is, PBL in the urinary bladder and DLBCL in the nasal cavity occurred simultaneously. We extracted DNA from the two lymphomas for polymerase chain reaction and sequenced the amplified immunoglobulin heavy variable genes and the complementarity-determining region- (CDR-) 3. The sequence of the CDR3 region of both tumors matched. MYC rearrangement was found in the bladder tumor but not in the nasal tumor. The patient was treated with R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisone), and durable remission had been obtained. The results of the DNA analysis indicated that both PBL and DLBCL emerged from common postgerminal B cells. This case may help to elucidate the pathogenesis of PBL.
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Project description:A 5-y-old female Golden Retriever was presented with a 2-wk history of hyporexia, vomiting, diarrhea, lethargy, weight loss, polyuria, and polydipsia. Clinical examination and ultrasonography revealed multiple organ enlargement with gallbladder and kidney nodules suggestive of disseminated neoplasia. Hematologic and biochemical analyses revealed pancytopenia, hypercalcemia, and monoclonal IgA gammopathy suspicious for a plasma cell neoplasm. Bone marrow and blood smear examination revealed neoplastic atypical cells highly suggestive of lymphoid origin. Autopsy confirmed the presence of homogeneous white masses and multifocal pale infiltrates in the spleen, kidney, small intestine, gallbladder, and urinary tract. Histologic features were consistent with a multicentric atypical plasma cell tumor. Tumor cells were negative for CD204, IBA-1, E-cadherin, CD3, CD5, CD79a, CD20, and PAX5, and positive for MUM1, consistent with plasma cell origin. The presence of > 20% of circulating blastic plasma cells was consistent with primary plasma cell leukemia with plasmablastic morphology, a disease rarely described in veterinary medicine.
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Project description:Diffuse Large B-Cell Lymphoma (DLBCL) is a heterogeneous disease characterized by a limited number of molecularly defined subtypes. Recently, genomic-based algorithms have been proposed for the classification of this disease. The whole exome sequencing was conducted on 108 diagnostic samples of diffuse large B-cell lymphoma (DLBCL). Somatic variants, predicted copy number alterations (CNAs), and available fusion data were utilized to classify the cases. Additionally, the enrichment of mutations in the TP53, MYC, and MAPK/ERK pathways was analyzed. Genetic subtypes were identified in approximately 55% of the cases. Cases with a specific genetic subtype exhibited a significantly higher Tumor Mutation Burden compared to molecularly unclassified cases (Mann-Whitney U test, p = 0.024). The prevalence of subtypes varied according to the cell of origin phenotypes. GC-B type DLBCL NOS were classified as EZB (5 cases, 16%), ST2 (5 cases, 16%), and BN2 (1 case, 3%). Four cases (13%) were genetically composite. Three cases of HGBCL/DLBCL double-hit (MYC & BCL2) were classified as EZB-MYC. Forty-three non-GC-B type DLBCL cases were classified as ST2 (5 cases, 11%), BN2 (6 cases, 14%), and MCD (3 cases, 7%). Nine cases were genetically composite (20%). MYC pathway mutations were enriched in cases with EZB and ST2 genetic features, while they were absent in the MCD subtype. TP53 mutations were identified in 11% of the cases. Plasmablastic lymphomas exhibit genetic diversity, with 27% of tumors classified as ST2. Recurrent somatic mutations indicate dysregulation of the JAK/STAT, MAPK/ERK, and tyrosine kinase signaling pathways.
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Project description:INTRODUCTION: Orbital cellulitis is an uncommon, potentially devastating condition that, when not promptly and adequately treated, can lead to serious sequelae. The presenting clinical signs are proptosis, swelling, ophthalmoplegia, pain and redness of the peri-orbital tissues. A number of cases have been reported in which these symptoms have been mistakenly interpreted as being secondary to an orbital infection whilst, in fact, other pathology was present. DISCUSSION: We add another case in which, on clinical grounds and after radiological assessment and laboratory tests, a working diagnosis of orbital cellulitis of the left eye was made. It was only after histopathological analysis of a soft tissue specimen from the maxillary sinus that a diagnosis of an AIDS-related plasmablastic lymphoma was made. The patient was referred to the department of haematology where chemotherapeutic treatment for the lymphoma and the HIV infection was started. This case report adds another differential diagnosis of orbital cellulitis to the existing literature.
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Project description:IntroductionPlasmablastic lymphoma is an aggressive non-Hodgkin lymphoma classically occurring in individuals infected with HIV. Plasmablastic lymphoma has a predilection for the oral cavity and jaw. However, recent case reports have shown lymphoma in the stomach, lung, nasal cavity, cervical lymph nodes and jejunum in HIV-negative individuals. We report what is, to the best of our knowledge, the first case of plasmablastic lymphoma occurring in the ano-rectal junction of an HIV-negative man.Case presentationA previously healthy 59-year-old Caucasian man presented with painless rectal bleeding. Colonoscopy revealed a lesion in the ano-rectal junction, with pathological examination demonstrating atypical lymphoid cells consisting primarily of plasmablasts with rounded nuclei, coarse chromatin, small nucleoli and multiple mitotic figures. Immunohistochemical analysis showed the atypical cells were negative for CD45, CD20, CD79a and immunoglobulin light chains, but were strongly positive for CD138 and EBV-encoded RNA. The results were consistent with a diagnosis of plasmablastic lymphoma. Aggressive systemic chemotherapy and involved field radiation therapy resulted in complete clinical and pathological remission.ConclusionIncreasing awareness of plasmablastic lymphoma in HIV-negative individuals and in this location is warranted.
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Project description:IntroductionPlasmablastic lymphoma (PBL) is a rare and aggressive variant of diffuse large B cell lymphoma characterized by weak or absent expression of conventional B cell markers and strong expression of plasma cell markers. Very few cases of PBL of the colon have been reported in HIV negative patients.Case presentationA 57 years female with HIV negative serology, a known case of hypertension under medication presented with right lower abdominal pain associated with vomiting and significant weight loss. On abdominal examination, soft, tender, and globular lump was palpable. Contrast enhanced computed tomography of abdomen and pelvis revealed asymmetrical enhancing mass like wall thickening involving ileocaecal region, caecum, and ascending colon, which on colonoscopy was found to be ulceroproliferative in nature. Open right hemicolectomy was performed and postoperative histopathology and immunohistochemistry results confirmed plasmablastic lymphoma as the final diagnosis. She was treated with nine cycles of chemotherapy.Clinical discussionAlthough PBL is commonly found in the oral cavity, and HIV positive patients, it can rarely occur in extra-oral sites, and HIV negative patients. Due to its rarity, no optimal therapeutic approach has yet been defined for the treatment of PBL. It has a poor prognosis, and the overall survival rate has been correlated with international prognostic index score and achievement of complete remission.ConclusionAs plasmablastic lymphoma is rare and highly aggressive, its delayed diagnosis will lead to poor outcome. Thus, awareness about its clinical presentation, histopathological features, and immunophenotype is essential.
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