Plasmablastic lymphoma presenting as a large intracardiac mass and bilateral pleural effusions.

Plasmablastic lymphoma (PBL) is a rare aggressive lymphoma arising most frequently in the oral cavity of HIV-infected patients. Rare cases of PBL have been reported in extra-oral sites, as well as in HIV-negative patients. Cardiac involvement by lymphoma is very rare. The most common primary cardiac lymphoma is diffuse large B-cell lymphoma. We report an unusual case of PBL in a 49-year-old, HIV-positive man presenting with a large intracardiac mass and bilateral pleural effusions. Histological examination of the cardiac mass biopsy and cytological evaluation of the pleural fluid demonstrated large lymphoma cells with plasmablastic differentiation. By immunohistochemistry, the large lymphoma cells expressed CD30, CD45, CD138, MUM1, and kappa light chain, were weakly positive for EMA, and were negative for T-cell and B-cell markers, lambda light chain, and human herpes virus 8 (HHV8). In situ hybridization for Epstein Barr Virus-encoded RNA (EBER) was negative in large lymphoma cells. To our knowledge, in the English literature, this is the second reported case of PBL with cardiac origin and the first reported case of PBL that presents as a combination of intracardiac mass and pleural effusions.

Elevated monoclonal and polyclonal serum immunoglobulin free light chain as prognostic factors in B- and T-cell non-Hodgkin lymphoma.

The serum immunoglobulin free light chain (FLC) assay quantitates free kappa (κ) and lambda (λ) light chains. FLC elevations in patients with diffuse large B-cell lymphoma (DLBCL), Hodgkin lymphoma (HL), and chronic lymphocytic leukemia (CLL) are associated with an inferior survival. These increases in FLC can be monoclonal (as in myeloma) or polyclonal. The goal was to estimate the frequency of these elevations within distinct types of B-cell and T-cell non-Hodgkin lymphoma (NHL) and whether the FLC measurements are associated with event-free survival (EFS). We studied serum for FLC abnormalities using normal laboratory reference ranges to define an elevated κ or λ FLC. Elevations were further classified as polyclonal or monoclonal. Four hundred ninety-two patients were studied: 453 B-cell and 34 T-cell NHL patients. Twenty-nine % (142/453) of patients had an elevated FLC of which 10% were monoclonal elevations. Within B-cell NHL, FLC abnormalities were most common in lymphoplasmacytic (79%), mantle cell (68%), and lymphomas of mucosa associated lymphoid tissue (31%); they were least common in follicular (15%). The hazard ratio (HR) for EFS in all patients was 1.41 (95% CI; 1.11-1.81); in all B-cell NHL the HR was 1.44 (95% CI 1.11-1.96); in all T-cell NHL the HR was 1.17 (95% CI 0.55-2.49). FLC abnormalities predicted an inferior OS (HR = 2.75, 95% CI: 1.93-3.90, P < 0.0001). The serum FLC assay is useful for prognosis in both B-cell and T-cell types of NHL. In B-cell NHL further discrimination between a monoclonal and polyclonal elevation may be helpful and should be analyzed in prospective clinical trials.

Extraoral plasmablastic lymphoma detected using ascitic fluid cytology and flow cytometry: a case report with a review of the literature.

BACKGROUND: Plasmablastic lymphoma (PL) is a relatively new category of lymphoma, which has been considered to be found predominantly in the oral cavity and has a strong association with HIV. CASE: We report a case of extraoral/mesenteric PL detected using cytological examination of ascitic fluid assisted by flow cytometric (FC) analysis. The cells were positive for CD38, CD138, CD10, CD45 and CD56 and negative for CD3, CD19, CD20 and CD79a, with cytoplasmic lambda light-chain restriction. We also reviewed 67 cases of extraoral PL from the available literature and found them to be less often associated with HIV (than oral PL), occurring mostly in males aged 30-60 years, with the most common extraoral site being the anorectal region. CONCLUSION: A high index of suspicion at the level of the cytopathologist is imperative for identifying lymphoma cells in a body fluid. A rare entity like PL can also be diagnosed on cytology assisted by ancillary techniques (like FC), without the need for a biopsy. We also suggest that the minimum panel to diagnose PLs should include CD138, MUM-1, Ki-67, ALK-1, CD3, immunoglobulin light-chains, CD20 and PAX5.

Acute kidney injury and bilateral symmetrical enlargement of the kidneys as first presentation of B-cell lymphoblastic lymphoma.

Lymphoblastic lymphoma is an uncommon subtype of lymphoid neoplasm in adults. Acute kidney injury at initial presentation due to lymphoblastic lymphoma infiltration of the kidneys has rarely been described. We report a 19-year-old woman who presented with acute kidney injury due to massive lymphomatous infiltration of the kidneys. The diagnosis of B-cell lymphoblastic lymphoma was established by immunohistochemical study of the biopsied kidney. The patient had an excellent response to the VDCLP protocol (vincristine, daunomycin, cyclophosphamide, asparaginase, and dexamethasone) with sustained remission. We recommend that lymphomatous infiltration be considered in patients presenting with unexplained acute kidney injury and enlarged kidneys.

Plasmablastic lymphoma of the retroperitoneum in an HIV-negative patient.

Herein is reported a case of plasmablastic lymphoma (PBL) of the retroperitoneum in an HIV-negative patient. This is the first reported case of PBL at this location and of PBL from Japan in the English-language literature. A 76-year-old Japanese man was admitted to hospital with a chief complaint of right inguinal lymph node swelling. Lymph node biopsy indicated large tumor cells with both diffuse and cohesive growth patterns, and conspicuous tumor cell proliferation in lymph node sinuses. The initial pathological diagnosis was metastatic carcinoma. The patient died approximately 1 month after admission, and autopsy showed that the main lesion was a very large retroperitoneal mass. On histology diffusely proliferated plasmablast-like or immunoblast-like tumor cells were identified, which were positive on immunohistochemistry for CD138 and negative for B-cell and epithelial markers. Approximately 90% of the tumor cells were positive for Ki-67. Tumor cells were diffusely positive for EBV-encoded small RNA on in situ hybridization. The autopsy findings suggested a diagnosis of PBL. Accordingly, PBL should be considered as a differential diagnosis when lymph node biopsy findings resemble those of the present patient.

Primary immunoblastic B-cell lymphoma of the cranial vault.

We present the first reported patient with primary immunoblastic non-Hodgkin's lymphoma of the cranial vault, with a fatal outcome in spite of complete surgical removal.

Plasmablastic lymphoma in the orbit: case report.

Plasmablastic lymphoma (PBL) is a rare entity most commonly identified in the oral cavity of immunodeficient patients. The immunophenotype of this condition shows a poor expression for B-cell markers but, in contrast, a strong reactivity for well-differentiated plasma cells markers, such as CD138, CD38, and epithelial membrane antigens. PBL survival is limited due to its highly aggressive local and metastatic behavior and poor response to treatment. Although it can involve different organs, there have been only a few cases involving the ocular adnexa. We describe a case of atypical rapidly progressive pre-septal brawny induration affecting the right orbit in a patient with HIV-related lymphoma.

[Febrile cellulitis surrounding a scar revealing a large immunoblastic B-cell lymphoma]. / Dermohypodermite fébrile péricicatricielle révélatrice d'un lymphome B immunoblastique à grandes cellules.

BACKGROUND: Secondary skin sites of lymphoma appear in the advanced stages of the disease. We report the first case of a pericicatricial skin infiltration, mimicking febrile dermohypodermitis, revealing diffuse immunoblastic large B-cell non-Hodgkin's lymphoma. PATIENTS AND METHODS: Four months after decompressive cervical laminectomy, a 56-year-old man presented an inflammatory pericicatricial patch evoking cellulitis in a setting of hyperthermia and lymphadenopathy. Blood cultures and bacteriological analysis of skin biopsy samples were negative. The images showed infiltration of the soft subcutaneous areas and polyadenopathy. Two weeks later, several subcutaneous nodules appeared on the trunk. Histological analysis and immunolabelling pointed to immunoblastic large B-cell non-Hodgkin's lymphoma. A clone of B lymphocytes CD45+, CD20+, CD79a+, Bcl2+, CD5+, MUM1+, CD3-, CD10-, CD23- and Bcl6- was seen. The remainder of the extension examination was negative. CHOP-rituximab polychemotherapy resulted in complete regression of all lesions, notably the inflammatory cervical plaque. DISCUSSION: Secondary skin manifestations of lymphoma are generally non-specific (pruritus, ichthyosis, purpura, etc.) rather than specific in terms of lymphoid infiltration. As in our patient, certain cutaneous sites of lymphoma may have a misleading clinical presentation, histological analysis alone was able to provide a conclusive diagnosis. In our patient, the highly specific infiltration seen around the entire scar could either suggest a Koebner phenomenon or point to a role of the cutaneous aggression within the development of an inflammatory process contributing to pericicatricial infiltration by lymphoid cells. Locoregional invasion from the osseous part of the cervical spine and not macroscopically diagnosed during neurosurgery could also be responsible.

Large cleaved and immunoblastic lymphoma may represent two distinct clinicopathologic entities within the group of diffuse large B-cell lymphomas.

PURPOSE: The reliability of immunohistochemistry for subdividing diffuse large B-cell lymphomas (DLBCL) into germinal center B-cell-like (GCB) and non-GCB prognostic subgroups is debated. In this study we evaluated the prognostic significance of such subgrouping on a series of 153 DLBCL patients. Furthermore, we investigated whether both subgroups could comprise clinicopathologic entities recognized by their morphology and characterized by a distinct phenotype, specific genetic abnormalities, and clinical characteristics. PATIENTS AND METHODS: All samples from patients were reviewed and morphologically subdivided into large cleaved, immunoblastic, and not otherwise specified DLBCL. GCB and non-GCB immunohistochemical profiles were established. The presence of chromosomal translocations involving BCL2, BCL6, and MYC and/or rearrangements of these genes was investigated. RESULTS: Subdividing DLBCL with either a GCB or non-GCB immunophenotypic profile was not of prognostic significance. Nevertheless, CD10 expression was a predictor of favorable outcome, whereas high bcl-2 expression and BCL6 rearrangement were adverse predictors of disease-free survival. Interestingly, large cleaved DLBCL was clearly associated with a GCB immunophenotypic profile, CD10 expression, BCL2 rearrangement, age younger than 60 years, and low to low/intermediate International Prognostic Index risk, but was not of prognostic significance. In contrast, immunoblastic morphology was associated with a non-GCB profile and was a significant predictor of unfavorable DFS. CONCLUSION: Subdividing DLBCL into subgroups based on their immunohistochemical profile was not of prognostic significance. Nevertheless, it allowed the additional characterization of two lymphoma subgroups previously recognized in the Working Formulation. Both correspond to two distinct clinicopathologic entities within the DLBCL.

[Angioimmunoblastic T-cell lymphoma suspected to recur in the cranium after complete remission: A case report].

A 46-year-old woman presenting to the Department of Hematology with swelling of the mandibular lymph nodes was diagnosed with angioimmunoblastic T-cell lymphoma (AITL) in June 2013. The patient went into complete remission in December 2013 with chemotherapy; however, she was re-evaluated because of mental confusion during May 2014. In addition to the memory disturbances, elevated cerebrospinal fluid cell count and protein were noted. Fluid attenuated inversion recovery cranial magnetic resonance imaging revealed multiple hyperintense areas in both the mammillary bodies and thalamus accompanied by contrast-enhancing in some areas. The diagnosis of recurrent AITL was made based on the brain biopsy. AITL recurrence in the cranium should be considered in patients exhibiting central nervous system symptoms although such recurrences have not been reported previously.

Lymphoma-induced polyradiculopathy in AIDS: two cases.

Progressive polyradiculopathy is a rare, well-documented complication of the acquired immunodeficiency syndrome in man. It has been commonly attributed to a cytomegalovirus (CMV) infection. We report two HIV-infected patients with clinical and electrophysiological features of a unique, subacute, progressive polyradiculopathy. Post-mortem examination in case 1 disclosed an infiltration of the leptomeninges, the lumbar spinal cord, and the anterior and posterior roots by a B-cell immunoblastic lymphoma. Immunochemistry for HIV1 and CMV was negative in the peripheral and the central nervous system. Case 2 showed bone-marrow involvement by a Burkitt type lymphoma. Specific chemotherapy was followed by both clinical improvement of the polyradiculopathy and complete remission on a second bone-marrow biopsy. These findings may indicate that a lymphoma must also be considered a possible cause of polyradiculopathy in AIDS.

Immunoblastic large B-cell lymphoma of the peripancreatic head region: MR findings.

We report the magnetic resonance (MR) appearance of a large B-cell lymphoma in the peripancreatic head region, in a 38-year-old male who presented with a 1-month history of pruritus and jaundice. Routine laboratory examination at presentation revealed an elevated bilirubin. The tumor was a large, solitary well-defined mass with no evidence of necrosis, which showed mild diffuse heterogeneous enhancement. The tumor was closely applied to the lateral margin of the head of the pancreas. The constellation of MR findings was interpreted as consistent with the correct eventual diagnosis of lymphoma.

Childhood polymyositis as a paraneoplastic phenomenon.

Childhood polymyositis and dermatomyositis are rare conditions that are not generally associated with malignancy even though an association between adult-onset and malignancy has been reported. A child is presented with typical polymyositis in whom an immunoblastic sarcoma subsequently became manifest; the literature also is reviewed concerning the association between malignancy and these childhood conditions.

Combined chemotherapy and radiotherapy in diffuse large cell immunoblastic lymphoma: a phase II study of CHOP/bleomycin/methotrexate alternating with ifosfamide/methotrexate/etoposide.

The clinical outcome of 23 patients with high grade diffuse large cell immunoblastic lymphoma (Working Formulation, category H) treated by an intensive shortened schedule regimen of chemotherapy is described. Alternating cycles of cyclophosphamide, doxorubicin, vincristine, bleomycin and prednisolone, and ifosfamide, etoposide and methotrexate were given over an 18-week (range 16.0-20.8) period. External beam radiotherapy was administered as consolidation therapy to sites of original bulky disease in 17 patients. Treatment was well tolerated, though there were two toxic deaths. A 90% response rate was obtained. Sixteen of 18 patients followed for a minimum of 36 months are alive and in complete remission, representing a disease free survival of 69.5%; two further patients are alive following autologous bone marrow transplant. The 3-year disease free survival was 73% (+/- 9%) and the overall 3-year survival 78% (+/- 9%).

[Perirenal immunoblastic lymphoma. Description of a case]. / Linfoma immunoblastico perirenale. Descrizione di un caso.

The primary perirenal localization of non-Hodgkin lymphomas is rare and normal methods of image diagnosis do not enable a reliable preoperative diagnosis. In the majority of cases renal function is not affected and this pathology is often presented as an occasional finding. The pathologies included in the differential diagnosis are renal neoplasias, abscess and inflammatory processes in a perirenal site. Echotomography shows the lesion as an hypoanechoic zone surrounding the kidney. Computed tomography show it as isodense with the renal parenchyma. Histological tests together with immunohistochemical tests identified a malignant large B cell immunoblastic-type lymphoma in the case described here, with plasmoblastic-plasmocytic differentiation and high malignancy according to the Working Formulation. The pathogenesis of this rare localisation is controversial. We maintain that lymphomatous proliferation may be triggered off by lymphatic follicles present in the perirenal space. The concomitant presence of other clinical signs, such as splenomegalia and adenopathies, may contribute to the diagnosis. On the contrary, monolateral involvement in the absence of other signs, as in this case, raises considerable problems of differential diagnosis. Perirenal lymphoma must therefore always be borne in mind in the diagnosis of renal or perirenal masses.

Primary malignant lymphoma of the ovary: an unusual presentation of a rare disease.

Because the outcome of patients with primary ovarian non-Hodgkin's lymphoma (NHL) is controversial, we present the incidental finding of a primary malignant lymphoma of the ovary in a 50-year-old patient. Three and a half years following ablative surgery and adjuvant chemotherapy, the patient is alive and disease free. Ovarian lymphoma is a disease of reportedly poor prognosis. However, many previously reported cases of ovarian lymphoma actually represented ovarian involvement by a more diffuse lymphomatous process. If stringent criteria are used for case selection, true primary ovarian lymphoma is a very rare disease and usually carries a favorable prognosis.

[Is Dacron carcinogenic? Apropos of a case and review of the literature]. / Le Dacron est-il cancérigène ? A propos d'un cas et revue de littérature.

Primary malignant cardiac tumours are extremely rare. The authors report a case of primary cardiac lymphoma nine years after implantation of a double leaflet mitral valve prosthesis. Malignant lymphoma is a haematological form of sarcoma. Exceptionally rare, it is a tumour of the immune system occurring principally in immuno-depressed patients. It typically presents as a nodular or diffuse myocardial infiltrate explaining its clinical expression as cardiac failure and atrioventricular block. In view of the usual degree of infiltration, surgery is rarely possible. Survival after "pure" medical therapy (chemotherapy alone or associated with radiotherapy) is 6 to 8 months after diagnosis. Dacron has been implicated in the pathogenesis of primary cardiac sarcoma. Oppenheimer demonstrated experimental induction of sarcoma in the rat by subcutaneous implantation of polymers. In conclusion, although primary cardiac lymphoma is a rare condition, it should be considered, as with thrombosis, a possible differential diagnosis of acute dysfunction of cardiac valvular prostheses.