Case Of Plasmablastic Lymphoma Research Articles

Histopathological Characteristics of Lymphomas in the Upper Aerodigestive Tract. A Single-Institute Study in Japan.

We analyzed the histopathological characteristics of lymphomas biopsied from the upper aerodigestive tract between 2000 and 2014 at the National Cancer Center Hospital in Japan. Of a total of 309 consecutive cases, the following incidences were observed: mature B-cell neoplasms, 77% (n = 239); mature T- and NK-cell neoplasms, 20% (n = 63); classical Hodgkin lymphomas, 0.7% (n = 2); and lymphoblastic lymphomas, 2% (n = 5). Lymphomas were most frequently (57%) detected in the oropharynx. The majority of cases (89%) were mature B-cell neoplasms (diffuse large B-cell lymphoma, 60%; follicular lymphoma, 10%), and 10% of cases were mature T-cell neoplasms. Six cases of plasma cell neoplasm (4 primary and 2 secondary involvement) and 2 cases of plasmablastic lymphoma in the upper aerodigestive tract were observed. Two out of 3 cases of extraosseous plasmacytoma with available biopsy material were positive for EBER1. All 3 patients received irradiation and achieved complete response; 1 had not relapsed after 17 months and the remaining 2 relapsed as plasma cell myeloma and solitary plasmacytoma of the bone. Of 47 extranodal NK/T-cell lymphoma, nasal-type cases in the upper aerodigestive tract, 38 (81%) were present in the sinonasal region and the remaining 9 (19%) were in the oropharynx (n = 4), nasopharynx (n = 3), and oral cavity (n = 2). In conclusion, since both primary lymphoma and secondary involvement of lymphoma are often diagnosed using biopsied materials from the upper aerodigestive tract, pathologists and hematologists should recognize the characteristics of lymphoma in this tissue.

Gastric plasmablastic lymphoma – case report

Plasmablastic lymphoma (PBL) is a rare, clinically aggressive B-cell lymphoma which was initially described involving the oral cavity of HIV positive patients. Subsequent reports have expanded its clinical spectrum to involve a broad spectrum of sites, other forms of immunosuppression and elderly patients without specific immune compromise. Gastric involvement is documented but uncommon, with seven reported cases of gastric plasmablastic lymphoma in the literature. We describe a case of gastric PBL in a non-immunocompromised 71 year old male. Endoscopic gastric biopsy showed an atypical sheet-like proliferation of large atypical cells with a range of plasma cell differentiation, including mature plasma cells, atypical binucleate cells, plasmablasts and immunoblasts. The tumour cells were positive for CD 45 (weak), CD 138, MUM-1, and EBV EBER in situ hybridisation, and showed Kappa light chain restriction. It is important to consider plasmablastic lymphoma in the differential diagnosis of large cell malignancies at any site and patient population, as weak CD 45 expression and lack of B and T cell markers may otherwise delay the diagnosis of lymphoma.

Plasmablastic lymphoma developing in thyroid: a rare entity in an immunocompetent individual

A case of plasmablastic lymphoma that has not been described previously in the thyroid is presented with its clinicopathological features and the diagnostic difficulties encountered. A detailed histopathology in conjunction with immunohistochemistry is recommended for appropriate diagnosis and management in the setting of HIV-negative status. Dealing with a case presenting primarily as a thyroid mass can be challenging in the scenario of a CD20-negative immunoprofile.

Plasmablastic Lymphoma in the Testis and Duodenum: A Case Report and Literature Review

Plasmablastic lymphoma (PBL) is a rare and highly aggressive subtype of non-Hodgkin’s lymphoma (NHL) associated with human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV). There are over 300 reported cases of PBL, of which over 120 are reported in HIV-positive patients. Of HIV-negative patients, patients were either immunocompetent or immunosuppressed transplant recipients. Initial reports highlighted cases arising in the oral cavity of HIV patients and this remains the most prevalent site of disease. However, other sites such as the gastrointestinal tract and skin are the next most common sites. Only three cases have involved the testes, a sanctuary site. Of gastrointestinal sites, there are only a handful of cases with gastric involvement. In the pre-human antiretroviral therapy (HAART) era, median overall survival was dismal. However, prognosis has improved since their advent. There is no significant difference in the survival of HIV-positive and HIV-negative PBL patients. It should be emphasized that due to the scarcity of cases, there are no established standards of care or prospective therapeutic trials for the management and treatment of PBL. Chemotherapy remains the standard approach, though selecting a regimen is still controversial and while response has been good, overall survival remains poor. Since 1995, autologous hematopoietic cell transplantation (AHCT) has been the standard of care for relapsed chemosensitive HIV-negative NHL. In the era of HAART, this has become feasible for HIV patients. A few studies have suggested that use of AHCT in the first line setting and relapsed or refractory disease may confer better outcomes. Herein we describe a unique case of involvement in the testis and duodenum, and there are no previous reports of a patient presenting with both gastrointestinal and urogenital involvement. J Hematol. 2015;4(3):196-201 doi: http://dx.doi.org/10.14740/jh217w

Sclerotic changes on plain X-ray led to early diagnosis and successful treatment of primary lymphoma of iliac bone.

Sir, A previously healthy 54-year-old woman presented to our hospital with a 3-month history of pelvic pain. There was no history of constitutional symptoms such as unintentional weight loss, fever, and loss of appetite. Clinical examination revealed pain on deep palpation of the right iliac region. There was no evidence of soft tissue swelling or redness in the site of patient's pain. The rest of physical exam was unremarkable. Laboratory investigations were within normal limits (erythrocytes sedimentation rate of 20 mm in the 1st h, negative C-reactive protein and white blood count of 4800 per μl). A plain radiograph of the pelvis showed slightly lytic-sclerotic changes of right iliac bone with indistinct margins, which at a shallow observation confused with bowel gases [Figure 1]. A computed tomography (CT) was performed and showed a slightly expansile lytic-sclerotic lesion with small soft tissue component in the right iliac bone [Figure 2]. The bone scan also revealed isolated uptake of technetium-99m in the right ilium [Figure 3]. According to the patient age, the differential diagnosis included metastasis, lymphoma, plasmacytoma, and chronic osteomyelitis. Figure 1 Radiograph of pelvis demonstrates lytic-sclerotic changes of right iliac bone Figure 2 Pelvic computed tomography image shows a slightly expansile lytic-sclerotic lesion in the right iliac bone with no extension to the sacroiliac joint Figure 3 Bone scan revealed isolated uptake of technetium-99 m in the right ilium The core needle biopsy result was consistent with the diagnosis of diffuse large B-cell nonHodgkin's lymphoma. There was not any other site of involvement on CT scan of the chest, abdomen, and pelvis. Positron emission tomography (PET) also showed no secondary absorption of fluorodeoxyglucose [Figure 4]. The diagnosis of primary nonHodgkin lymphoma of bone was made, and the patient was successfully treated with a combination of chemotherapy and radiotherapy. Figure 4 Positron emission tomography scan showed no secondary absorption of fluorodeoxyglucose NonHodgkin's lymphoma of bone is a rare entity which is limited to the long bones and axial skeleton, with the femur being the most common site of involvement. Primary involvement of iliac bone is extremely rare. There are no clinical or radiological characteristics which be diagnostic for primary bone lymphoma. The image findings in radiograph or CT scan are variable and nonspecific, range from near normal to diffuse permeative pattern.[1,2,3,4,5,6] A review article performed by Mulligan et al., revealed that the most common features on imaging studies are lytic (70%), including permeative or moth-eaten pattern, and mixed lytic and sclerotic density (28%). Periosteal reaction and soft tissue mass were reported in 58%, and 80-100% of cases, respectively, in this study.[2] Sequestrum formation considered as a radiological feature which may help to differentiate between plasmablastic lymphoma (PBL) and other conditions such as metastasis and myeloma. Sequestrum formation has been reported in 11-16% of PBL cases.[7] Cross-sectional imaging studies are useful adjuncts to conventional radiography. Extensive involvement of bone marrow cavity with a surrounding soft tissue component and without extensive cortical destruction is a pattern, which is reported only in PBL, Ewing sarcoma, and myeloma.[8] The key educational message of our case is that any change on plain X-ray is valuable and should not be overlooked. This case also reminds us the importance of considering primary nonHodgkin lymphoma as a valuable differential diagnosis in lytic and sclerotic lesions of iliac bone. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Human immunodeficiency virus-negative plasmablastic lymphoma in the neck: a rare case report and literature review

Plasmablastic lymphoma (PBL) is an aggressive neoplasm exclusively occurring in AIDS patients. Recently, increasing cases of human immunodeficiency virus (HIV)-negative PBL have been reported. No standard therapy protocol is currently available since there is a great difference between PBL with and without HIV infection. Here, we present a rather rare case of HIV-negative PBL in the neck that dramatically responded to radiotherapy alone. Our case highlights the possibility of PBL in the neck and helps to expand our understanding of this separate lymphoma. The related literature review summarized the clinicopathological features and treatment status of HIV-negative PBL.

Unusual presentation of duodenal plasmablastic lymphoma in an immunocompetent patient: A case report and literature review.

Plasmablastic lymphoma (PBL) is a rare and recently described entity of large B-cell lymphoma. It predominantly occurs in the oral cavity of human immunodeficiency virus (HIV)-positive patients and exhibits a highly aggressive clinical behavior without effective treatment. Recently, sporadic cases describing PBL in extraoral locations of HIV-negative patients have been reported; frequently in patients with underlying immunosuppressive states. To develop the understanding of PBL, the current study reports the unusual presentation of duodenal PBL and reviews the pathogenesis, immunohistochemical features, clinical and differential diagnoses, as well as the treatment of PBL as described in previous studies. The case of a 75-year-old female with duodenal PBL without definite immunosuppression is presented in the current report. The tumor was composed of large B-cell-like cells, and was positive for cluster of differentiation 138 and melanoma ubiquitous mutated-1, with ~80% of the tumor cells positive for Ki-67. The features of the tumor were as follows: Extraoral location, HIV-negative, immunoglobulin M λ-type M protein expression, light chain restriction (monoclonal) and Epstein-Barr virus-encoded small RNA-negative, which are considered to be unusual for PBL. These unusual features complicate the differentiation of PBL from other plasma cell diseases. To the best of our knowledge, this is the first study to report a case of duodenal PBL in an immunocompetent patient. To date, the standard treatment of PBL remains elusive, however, the most commonly administered chemotherapy treatments are CHOP [intravenous cyclophosphamide (750 mg/m2, day 1), intravenous doxorubicin (50 mg/m2, day 1), intravenous vincristine (1.4 mg/m2, day 1) and prednisone (100 mg, days 1–50)]-like regimens. The patient was administered two cycles of CHOP chemotherapy for 56 days, however, ultimately succumbed as a result of disease progression. Therefore, PBL represents a diagnostic and therapeutic challenge. PBL must be considered in the differential diagnosis of gastrointestinal tumors in daily practice, even in immunocompetent patients. Furthermore, CHOP does not appear to be an optimal treatment regimen and more intensive regimens are required.

Plasmablastic lymphoma in childhood: A report of two cases

Plasmablastic lymphoma is an aggressive non-Hodgkin lymphoma predominantly seen in adult patients. Only eight cases of plasmablastic lymphoma in children have been published to date. In this report, we present an additional two cases. The first patient was a 9-year-old girl presenting with a nasal mass, while the second was a 15-year-old girl with swelling of the right side of the face and proptosis. Both patients were HIV-positive.

A case of plasmablastic lymphoma diagnosed on liquid‐based pap test

Primary gynecological lymphomas are rare and difficult to diagnose with Pap test. Herein, we report an uncommon case of plasmablastic lymphoma in a 61-year-old female with human immunodeficiency virus that presents with vaginal bleeding. The following case report shows the cytological features on liquid-based Pap test, cell block studies with diagnostic immunohistochemistry, discussion of the diagnostic difficulties, and a literature review.

Epstein Barr virus DNA analysis in blood predicts disease progression in a rare case of plasmablastic lymphoma with effusion

BackgroundIn HIV-1-infected patients a long lasting CD4+ cell decline influences the host-EBV balance and thereby increases the risk for EBV related malignancies. In spite of a world-wide access to combination antiretroviral therapy (cART) there are still a considerable number of HIV-1-infected patients who will develop severe immunodeficiency. These undiagnosed HIV-1 infected patients, so called late testers, demonstrate an increased lymphoma risk, compared to patients diagnosed early. Consecutive individual screening for EBV DNA-load in late testers might be a useful predictor of emerging EBV-malignancy.MethodsPatient biopsies and ascites were analyzed morphologically, by immuncyto-histochemistry and in-situ hybridization. Viral DNA and RNA load were quantified by PCR. Cell lines from primary tumor and from ascites, were established in vitro and further analyzed.ResultWe here report on a case of EBV-positive lymphoma in an AIDS patient, first presenting with pleural effusion and ascites and was thus initially considered a primary effusion lymphoma (PEL) but was later diagnosed as a plasmablastic lymphoma (PBL). The patient had responded to cART with undetectable HIV-RNA and increased CD4 cell count one year prior to lymphoma presentation. At the time of lymphoma diagnosis the HIV-RNA values were <50 RNA-copies per mL blood (undetectable) and the CD4-positive cell count 170 ×106/L. The lymphoma was CD45-negative and weakly CD22- and CD30-positive. The patient had a history of Kaposi sarcoma and HHV-8 seropositivity. The lymphoma biopsies, and three cell lines derived on different occasions from the tumor cell effusion, were all EBV-positive but HHV-8 negative.A noticeable EBV-DNA load decline was observed during the remission of the lymphoma following CHOP-therapy. The EBV-DNA load increased dramatically at the time of recurrence.ConclusionEBV DNA load might be useful in monitoring the effect of lymphoma treatment as well as in estimating the risk of EBV-associated lymphoma in HIV-1 infected patients with pronounced immunosuppression.

Plasmablastic lymphoma of the oral cavity in a human immunodeficiency virus-negative patient: A case report with literature review

Plasmablastic lymphoma (PBL) is an aggressive diffuse large B-cell lymphoma variant that is most frequently observed in the oral cavity of human immunodeficiency virus (HIV)-infected individuals. However, in recent years, some cases have emerged in patients without HIV infection and involve other sites like stomach, lung, nasal cavity, and jejunum. We report a rare case of PBL in the maxillary anterior area of a 62-year-old man without HIV infection. The tumor cells were characterized by non-cohesive round or oval shape cells with eccentrically-placed nuclei with a prominent perinuclear halo. Immunohistochemical studies showed that the tumor cells were strongly positive for MUM1, VS38c, VMT, and κ light chain, focally positive for LCA and CD79a, and negative for CD3, CD20, CD56, λ light chain, CK-pan, EMA, and HMB45. The patient was treated with chemotherapy using cyclophosphamide, doxorubicin, vincristine, and prednisone. The lesion showed partial remission.

Plasmablastic lymphoma of the maxillary sinus in an HIV-negative patient: a case report and literature review

Plasmablastic lymphoma (PBL) is a rare and aggressive variant of diffuse large B cell lymphoma. The prognosis of PBL patients is poor. The majority of patients succumb to a fulminant disease course, with most dying in the first year after diagnosis. The small number of HIV-negative PBL cases reported in the literature to date is composed of single case reports and small case series. Consequently, the natural history of the disease in HIV-negative individuals and the optimum treatment are not well characterized. Intensive induction chemotherapy has been associated with marked improved overall survival. However the optimal regimen has not been defined. We describe the third case of PBL of the maxillary sinus which occurred in a 24-year old HIV-negative man. We outline the clinicopathological features and report success using a hyper-CVAD regimen with 6 cycles and consolidation radiation therapy yielding a complete remission of four years.

A case of plasmablastic lymphoma of the liver without human immunodeficiency virus infection

Plasmablastic lymphoma (PBL) is a very rare B-cell lymphoproliferative disorder was with an aggressive clinical behavior that recently characterized by the World Health Organization. Although PBL is most commonly observed in the oral cavity of human immunodeficiency virus (HIV)-positive patients, it can also be observed at extra-oral sites in HIV-negative patients. Epstein-Barr virus (EBV) may be closely related the pathogenesis of PBL. PBL shows different clinicopathological characteristics between HIV-positive and -negative patients. Here, we report a case of PBL of the liver in a 79-year-old HIV-negative male. The patient died approximately 1.5 mo after examination and autopsy showed that the main lesion was a very large liver mass. Histopathological examination of the excised lesion showed large-cell lymphoma with plasmacytic differentiation diffusely infiltrating the liver and involving the surrounding organs. The neoplastic cells were diffusely positive for CD30, EBV, Bob-1, and CD38. The autopsy findings suggested a diagnosis of PBL. To our knowledge, the present case appears to be the first report of PBL with initial presentation of the liver in a patient without HIV infection.

Plasmablastic lymphoma of the stomach in an HIV‐negative patient

Plasmablastic lymphoma (PBL) is a rare B-cell neoplasm with an aggressive clinical behavior that predominantly occurs in the oral cavity of human immunodeficiency virus (HIV)-positive patients. However, it has recently been recognized that PBLs can also affect individuals without HIV infection, and suggested that these neoplasms show different clinicopathological characteristics between HIV-positive and -negative patients. Herein we describe a case of gastric PBL in a female HIV-negative patient. The tumor was composed of a diffuse and cohesive proliferation of large neoplastic cells, which resembled immunoblasts or plasmablasts with a starry sky appearance. Immunophenotypically, the neoplastic cells were diffusely positive for CD138, MUM1, IgM, and BOB-1, and negative for CK, LCA, CD3, CD20, CD79a, Pax5, kappa, lambda, CD30, ALK, S-100, HMB-45, MPO, and HHV-8. The MIB-1 index was nearly 100%. Epstein-Barr virus-encoded RNA in situ hybridization was negative. A monoclonal immunoglobulin heavy chain gene rearrangement was detected in polymerase chain reaction (PCR) and heteroduplex analyses. A combination of PCR-based analysis of immunoglobulin gene rearrangement and immunohistochemistry can be useful to substantiate the diagnosis by utilizing routine paraffin-embedded tissue sections, because PBL in the setting of extra-oral localization and immunocompetence is a diagnostic challenge, given its rarity, morphology, and absence of CD20 expression.

Plasmablastic lymphoma of the elderly: a clinicopathological comparison with age‐related Epstein–Barr virus‐associated B cell lymphoproliferative disorder

Plasmablastic lymphoma (PBL) is an aggressive lymphoma with a terminally differentiated B cell phenotype; half of patients with this disease have Epstein-Barr virus (EBV) infection. The majority of PBL cases are associated with human immunodeficiency virus (HIV) infection, while the remaining HIV-negative cases were accompanied by other immunodeficiency conditions or immunosenescence in the elderly. To characterize HIV-negative PBL of the elderly (PBL-E), we compared the clinicopathological characteristics of 10 cases of PBL-E and 124 cases with age-related EBV-associated B cell lymphoproliferative disorder (AR-EBVLPD). The 10 PBL-E (eight men, two women; median age: 68 years) were associated with a more indolent clinical behaviour and a better overall survival than AR-EBVLPD. Extranodal involvement was higher in PBL-E (50%) than AR-EBVLPD; notably, the nasal cavity was affected most frequently in PBL-E (60%). Immunoglobulin heavy chain/(IGH)/MYC translocation was detected in half of the PBL-E cases. PBL-E shares some clinical features with AR-EBVLPD, such as HIV negativity, old age, and EBV infection, no known immunosuppressive condition but there are some differences such as a higher ratio of extranodal involvement and better prognosis. PBL-E is a newly recognized condition and should be distinguished from HIV-positive PBL, sharing features with AR-EBVLPD in particular, immunosenescence of the elderly.

Lack and/or aberrant localization of major histocompatibility class II (MHCII) protein in plasmablastic lymphoma

Lack and/or aberrant localization of major histocompatibility class II (MHCII) protein in plasmablastic lymphoma

Plasmablastic lymphoma of the retroperitoneum in an HIV- and HCV-positive patient: hard to diagnose and harder to treat

Plasmablastic lymphoma (PBL) is an aggressive diffuse large B-cell lymphoma (DLBCL) with plasmablastic features that was initially described in the oral cavity of HIV-infected individuals. PBL remains a diagnostic challenge given its close morphologic resemblance and overlapping immunophenotypic patterns to other B-cell lymphoid malignancies and plasmablastic plasma cell myeloma (PCM) with extramedullary involvement. The presence of serum monoclonal protein and radiographic evidence of lytic bone lesions favors the diagnosis of plasma cell myeloma over PBL. Distinguishing PBL from PCM is important as PBL is treated with a completely different chemotherapy regimen compared to PCM. PBL carries a guarded prognostic profile among DLBCLs with high relapse rate and poor median survival. We present a case of a 44-year-old HIV-positive man who presented with a large retroperitoneal mass associated with obstructive uropathy, sacral radiculopathy, and inferior vena caval compression. The mass was initially mistaken to be a PCM on histopathology; however, subsequent investigations revealed an extra-oral PBL with plasmacytic differentiation. To our knowledge, this will be the first case of PBL of the retroperitoneum in an HIV- and HCV-positive patient and the second one at this location in the English-language literature. In this report, key differentiating points between PBL versus PCM and newer therapeutic agents such as proteasome inhibitors have been discussed along with related review of literature.

Plasmablastic lymphoma in an elderly immunocompetent patient

Plasmablastic lymphoma (PBL) is a distinct type of diffuse B cell lymphoma that typically occurs in the oral cavity of patients with HIV infection or immunodeficiency status. PBL is characterized by its plasmablastic morphology and an immunophenotype indicative of a plasma cell differentiation. We present a case of a 75-year-old HIV-negative and Epstein-Barr virus (EBV)-negative patient presenting with an isolated oral cavity mass. The tumor consisted of a monotonous proliferation of undifferentiated large cells with relatively abundant cytoplasm, eccentrically located round nuclei with prominent nucleoli and numerous mitoses. Immunohistochemically, these cells were negative for CD45 and B cell antigens, while they showed diffuse positivity of CD138 and focal staining for epithelial membrane antigen (EMA), indicating plasma cell differentiation. Based on these histopathological and immunohistochemical characteristics, we diagnosed it as PBL. The patient received chemotherapy and is alive with locally persistent disease 3 years after diagnosis. To date, only several cases of oral PBL have been reported in HIV-negative, EBV-negative and immunocompetent patients. PBL should be included in the differential diagnosis of oral mass lesions and careful evaluation of the morphology and awareness of the existence of this uncommon type of lymphoma can lead to a correct diagnosis.

A case report of cecal plasmablastic lymphoma in a HIV-negative patient

Plasmablastic lymphoma (PBL) is a unique type of diffuse proliferation of large neoplastic lymphoid cells most of which resemble B immunoblasts, but all tumor cells show the immunophenotype of plasma cells. It has a strong predilection for jaw and oral cavity in HIV-positive patients. Incidences of extraoral location of this tumor is increasingly being recognized especially in HIV-negative patients for example, stomach, jejunum, omentum, anorectum, lungs, testes, soft tissues, lymph nodes, bone marrow, skin, and central nervous system. We present a case of PBL found in cecum in an HIV-negative patient. It was accompanied by lung and lymph node involvement and presented as abdominal mass. This is only the second reported case of PBL originating in cecum.

Plasmablastic lymphoma of the oral cavity, a B cell-derived lymphoma associated with HIV infection: a case series

Plasmablastic lymphoma (PBL) of the oral cavity is a rare form of non-Hodgkin lymphoma that is most frequently met in human immunodeficiency (HIV) positive patients. Only a few cases have been reported worldwide since 1997. This clinical entity may escape detection due to its unusual immunophenotype and rare occurrence. Our aim is to present two cases with this rare condition that were diagnosed and treated in our department. We describe two cases of PBLs in HIV-infected patients, who presented with an expanding painless oral lesion and summarize the literature in order to elucidate the nature of this malignancy. The first patient received chemotherapy with additional radiotherapy that led to complete remission of the disease, while the second experienced a relapse 6 months after treatment with chemotherapy, that caused his death after refusal of further treatment. Because of the consistent epidemiological association of PBL with immunosuppression, any patient diagnosed with PBL should be tested for HIV. The clinical picture of PBL, including its affinity with HIV-infection, male sex, and its predilection for the oral cavity, may contribute to the differential diagnosis. Any oral mass occurring in an immunosuppressed patient should be referred for biopsy, since the early diagnosis of these tumors leads to better prognosis of the patients.