[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.

Plasmablastic lymphoma of the larynx: report of two cases.

OBJECTIVE: To report two cases of laryngeal plasmablastic lymphoma, a rare and relatively recently described form of non-Hodgkin's lymphoma. It has not previously been described in the larynx, nor associated with upper airway obstruction. CASE REPORTS: We describe the clinicopathological features of two such cases in human immunodeficiency virus positive patients, and we discuss their unusual presentations and diagnostic features. CONCLUSION: When evaluating a laryngeal tumour, plasmablastic lymphoma and other non-Hodgkin's lymphomata should be considered as differential diagnoses, particularly in the setting of a high prevalence of human immunodeficiency virus infection. Biopsy with detailed histopathological and immunohistochemical evaluation is recommended to ensure correct diagnosis and optimal management.

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

OBJECTIVES: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

A case report with plasmablastic lymphoma of the jejunum.

Plasmablastic lymphoma (PBL) is a recently identified entity that is considered to be a type of diffuse large B-cell lymphoma with a unique immunophenotype and a predilection for the oral cavity of patients with the human immunodeficiency virus (HIV). Although its clinical features may help in the differential diagnosis, an extraoral location in a patient without HIV makes it more difficult to suspect clinically. This case report is the first to describe a patient with PBL originating from the jejunum in a 60-yr-old, HIV-seronegative man. Computed tomography of the face, chest and abdomen showed about a 9.4 x 9.0 cm mass of the proximal jejunum, multiple masses in the musculoskeletal soft tissue, and multiple lymphadenopathies. The histological examinations demonstrated a large cell lymphoma with plasmablastic differentiation. The neoplastic cells were diffusely positive for MUM1, epithelial membrane antigen and lambda light chains, and focally positive for CD79a; but negative for CD3, CD20, CD30, CD34, CD45RO, CD56, CD99, and CD117. The proliferation index by Ki-67 immunohistochemistry was approximately 70%. These findings were compatible with the diagnosis of PBL. The findings in this case suggest that PBL should be included in the differential diagnosis of a small bowel mass even in a HIV-negative patient.

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.

HIV-associated plasmablastic lymphoma: lessons learned from 112 published cases.

Plasmablastic lymphoma (PBL) is a distinct subtype of non-Hodgkin B-cell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus (HIV)-infected individuals. Data regarding patient age and gender, HIV status, initiation of and response to highly active antiretroviral therapy (HAART), tumor extent, pathology, treatment, and outcome were extracted from 112 cases of PBL identified in the literature. The median age at presentation was 38 years with a male predominance of 7:1, and the median CD4+ count was 178 cells/mm(3). PBL presented on average 5 years after diagnosis of HIV. Common primary sites of presentation included the oral cavity, gastrointestinal tract, and lymph nodes. Most cases presented with either stage I or stage IV disease. There was a variable expression of B-cell markers in tumor cells, but plasma cell markers were expressed in all cases. EBV was detected in 74%. Chemotherapy was used to treat 55% patients and was combined with radiotherapy in 21% cases. Complete response was obtained in 66% of treated cases; the majority of these responses were seen after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). The refractory/relapsed disease rate was 54%. Death occurred in 53% of patients, with a median overall survival of 15 months. Sex, CD4+ count, viral load, clinical stage, EBV status, primary site of involvement, and use of CHOP failed to show an association with survival. PBL is an aggressive B-cell lymphoma that presents in both oral and extra-oral sites of chronically HIV-infected immunosuppressed young men.

Relationship between the response to treatment and the prognosis of patients with aggressive lymphomas treated with chemotherapy followed by involved-field radiotherapy: radiographic assessment.

OBJECTIVE: We examined the relationship between the response to treatment and prognosis of patients with aggressive lymphoma. METHODS: We reviewed 33 patients with aggressive lymphoma treated with chemotherapy consisting of the CHOP regimen followed by radiotherapy. Twelve patients had Stage I, 13 had Stage II, 6 had Stage III and 2 had Stage IV disease. According to the International Prognostic Index (IPI), 13 had low, 15 had low-intermediate, 2 had high-intermediate and 3 had high IPI. After three to six cycles of chemotherapy, involved-field radiotherapy was performed. We evaluated the response to treatment by computed tomography (CT), magnetic resonance imaging (MRI) and gallium scintigraphy (Ga-67) at the time of completion of chemotherapy and at the time of completion of radiation therapy. The median follow-up period was 48 months (4-80). RESULTS: The 2-year progression-free survival rates of the patients with Ga-67 positive uptake and Ga-67 negative uptake after completion of chemotherapy were 78 and 26% (P = 0.009), respectively. However, there were no statistically significant correlations between progression-free survival and the response after completion of chemotherapy determined by CT (P = 0.75) or MRI (P = 0.19). The response to treatment at the time of completion of overall treatment was not useful for prediction of prognosis. CONCLUSIONS: Ga-67 positive uptake at the completion of chemotherapy before radiotherapy may be associated with poor prognosis.

AIDS-related plasmablastic lymphoma of the oral cavity associated with an IGH/MYC translocation--treatment with autologous stem-cell transplantation in a patient with severe haemophilia-A.

Plasmablastic lymphoma is an AIDS related lymphoma that continues to have a poor prognosis despite significant advances in the management of HIV and lymphoproliferative diseases. In part this has been due to limited insights into the biology of this disease and the molecular mechanisms of oncogenesis. To date molecular abnormalities have not been described in plasmablastic lymphoma, and its aggressive clinical behaviour has been difficult to understand. We describe the first reported cytogenetic abnormality in plasmablastic lymphoma, an IgH/MYC translocation. It is also the first description of autologous stem cell transplantation in a patient with severe haemophilia A.

[Results of intensive chemotherapy followed by hematopoietic stem-cell rescue in 22 patients with refractory or recurrent primary CNS lymphoma or intraocular lymphoma]. / Chimiothérapie intensive avec support hématopoïétique chez 22 patients ayant un lymphome primitif du système nerveux central ou un lymphome intraoculaire réfractaire ou en rechute: mise à jour des résultats.

Rescue therapies for relapsing/refractory primary central nervous system lymphoma (PCNSL) and intraocular lymphoma (IOL) remain a challenging problem for clinicians. In 2001, we published encouraging results for 22 patients treated at relapse with a CYVE regimen combining high doses of Ara-C (50 mg/m(2)/d in 12 hours infusion dl through d5; 2 g/m(2)/d d2 through d5) and VP16 (200 mg/m(2)/d d2 through d5), followed by intensive chemotherapy based on high doses of thiotepa (250 mg/m(2)/d d-9 through d-7), busulfan (10 mg/kg total dose d-6 through d-4) and cyclophosphamide (60 mg/kg/d, d-3 and d-2 with hematopoietic cell rescue at d0. Patients were enrolled onto the study for a relapse (n = 10; 2 IOL, 3 CSF, 5 brain lesion) or for a refractory disease (n = 12; 9 IOL, 3 brain lesion). CYVE rescue was not administered to patients with refractory IOL who had previously received high doses of methotrexate and Ara-C as part of their first-line treatment. Twenty patients received the intensive chemotherapy and hematopoietic cells rescue. We updated our results in March 2003. Seven patients had neurologic adverse events during the entire procedure. With a median follow up of 6.2 years, the median overall survival is 91 months, and the median survival after intensive chemotherapy has not been reached.

[Varicella zoster virus infection after bone marrow transplant. Unusual presentation and importance of prevention]. / Infection par le virus varicelle-zona après greffe de moelle osseuse. Présentations inhabituelles et intérêt de la prophylaxie.

BACKGROUND: Leukemeia and lymphoproliferative disease are associated with a high risk of varicela-zoster virus (VZV) infection. Although infrequent, visceral involvement can be fatal. We report two cases of patients presenting severe VZV infection after bone marrow transplantation. CASE REPORTS: The first patient was a 42-year old man who received an allogeneic bone marrow transplantation for chronic myelogenous leukemia. A severe graft-versus-host reaction occurred. Three months after discontinuing VZV prophylaxis, VZV transverse myelitis was diagnosed, leading to death despite prompt treatment with acyclovir. The second patient was a 42-year-old woman treated with autologous bone marrow transplantation for lymphoma. She developed acute viral pancreatitis one month after discontinuing VZV prophylaxis. Recovery was achieved with intravenous treatment. DISCUSSION: These two cases illustrate the potential gravity of VZV infection after bone marrow transplantation. These observations point to the need for revisiting the duration of VZV prophylaxis.

Results of intensive chemotherapy followed by hematopoietic stem-cell rescue in 22 patients with refractory or recurrent primary CNS lymphoma or intraocular lymphoma.

PURPOSE: To assess the feasibility and efficacy of intensive chemotherapy with hematopoietic stem-cell rescue (IC + HCR) in patients with refractory or recurrent primary CNS lymphoma (PCNSL) or intraocular lymphoma (IOL). PATIENTS AND METHODS: IC consisted of thiotepa 250 mg/m(2)/d days -9 through -7, busulfan 10 mg/kg (total dose) days -6 through -4, and cyclophosphamide 60 mg/kg/d days -3 and -2. Intravenous clonazepam 2 mg/d was given prophylactically from the day before initiation of busulfan therapy to the day after completion of busulfan therapy. Patients with refractory or recurrent PCNSL underwent IC + HCR only if they were chemosensitive to two cycles of salvage treatment with cytarabine (2 g/m(2)/d days 2 through 5 and 50 mg/m(2)/d days 1 through 5 in a 12-hour infusion) and etoposide (VP-16; 200 mg/m(2)/d days 2 through 5) (CYVE). Patients with IOL refractory to high-dose methotrexate (MTX) and cytarabine entered the IC + HCR program directly. RESULTS: Twenty-two patients (10 with relapses, 12 with refractory disease) were enrolled. Twenty patients entered the IC + HCR program: twelve entered after CYVE treatment, seven entered directly, and one had previously been retreated with high-dose MTX. Before IC, eight patients were in complete remission (CR), four were in partial remission (PR), one had stable disease, and seven had refractory disease. After IC + HCR, 16 patients entered CR, two remained in PR, one had stable disease, and one had disease progression. Fourteen patients remained alive (median follow-up time, 41.5 months). The overall probability of survival at 3 years was 63.7%. After IC, that probability was 60% and the 3-year probability of event-free survival was 53%. Seven patients had neurologic adverse events during the entire procedure. CONCLUSION: IC + HCR proved feasible and effective in patients with refractory or recurrent PCNSL or IOL. The entire procedure seemed to be most toxic in patients > or = 60 years. A prospective multicenter study is ongoing.

CD3- CD56+ non-Hodgkin's lymphomas with an aggressive behavior related to multidrug resistance.

CD56 expression has been reported previously in some non-Hodgkin's lymphoma (NHL) characterization. They principally involve the nasopharynx, are related to Epstein-Barr virus (EBV), and may be classified as either T- or non-T-natural killer (NK) cells according to CD3/T-cell receptor (TCR) status at the genomic or protein level. The present study reports three cases of non-nasal NK-NHL with the following characteristics: an agressive clinical behavior, heterogenous morphological data evoking pleomorphic T-cell malignant lymphoma, a non-T-NK phenotype using flow cytometry, and immunochemistry. The three cases were CD56+ without membrane expression of specific T markers (CD3, CD5, and TCR). Heterogenous results were observed concerning different antigens: CD2, CD4, CD8, CD16, CD94, CD122, TiA1, perforin, and granzyme B. There was no evidence of detectable clonal TCR gene rearrangement with polymerase chain reaction. No NK activity was detected in the two tested cases, and no relation was found with EBV. Multidrug resistance investigations suggest that agressive clinical findings could be related to MDR1 gene expression as confirmed by MDR1 mRNA detection, MDR1 gene product (Pgp) expression, and a functional multidrug resistance study using rhodamine efflux by flow-cytometry.

Impact of high-dose salvage therapy (BEAM) on overall survival in younger patients with advanced large-cell lymphomas entered into BNLI trials.

The survival of two cohorts of patients with stage III/IV large-cell lymphomas treated by CHOP has been compared. In the first cohort of 88 patients (1974-1982), high-dose therapy with autologous bone marrow transplantation (ABMT) was not available as salvage therapy and in the second cohort of 87 patients (1987-1992), this was the recommended salvage for patients with disease that was still chemosensitive to conventional-dose therapy. The actuarial overall survivals at five years were 40% and 44% in the first and second cohorts, respectively, indicating that the availability of ABMT had made little impact. Of the 62 patients in the second cohort who failed CHOP therapy, 8 died before second-line chemotherapy could be given, 1 refused more therapy, and 8 were considered unsuitable for further combination chemotherapy. Seven patients with localized disease remaining received local radiotherapy. Of the 38 patients given salvage therapy, 14 had chemoresistant disease. Only 9 patients received high-dose BEAM chemotherapy and ABMT, and 7 remain disease-free. ABMT was restricted to a highly select patient group, and as a result more widespread application of this strategy might result in only a modest further improvement.