Fibrin-associated Large B-cell Lymphoma: Part of the Spectrum of Cardiac Lymphomas.

Oct 2012

Gruver AM, Huba MA, Dogan A, Hsi ED.

Source

*Department of Clinical Pathology, Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH †Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

Abstract

Cardiac lymphomas are rare, and the spectrum of pathologic features is not well defined. We encountered an unusual case of cardiac lymphoma residing within a presumed thrombus. To place such cases in context, we reviewed all cardiac lymphomas presenting to a large US cardiovascular medicine referral center during a 30-year period. A total of 14 cardiac lymphomas were identified, and these included 6 primary cardiac lymphomas (PCLs) and 8 lymphomas secondarily involving cardiac structures. Upon review, 3 of the PCLs were confirmed to be diffuse large B-cell lymphoma, not otherwise specified, involving the myocardium. The other 3 cases of PCL lacked myocardial invasion and showed lymphoma cells embedded in fibrin thrombus. Acute inflammation was not evident. These lymphomas presented in immunocompetent male individuals and involved either a prolapsed myxomatous mitral valve, a pseudomyxoma from the left atrium, or a thrombus arising in a synthetic aortic root graft. All 3 consisted of large atypical lymphocytes expressing a nongerminal center B-cellimmunophenotype. Two cases were positive for Epstein-Barr virus (latency type III), but none demonstrated human herpes virus-8 latent nuclear antigen. No systemic disease was found at presentation or during follow-up. In our experience, fibrin-associated large B-cell lymphoma arising in the heart represents a substantial proportion of PCL. These lymphomas appear to represent an underrecognized variant of diffuse large B-cell lymphoma with favorable outcome. Further study is needed to understand their natural history.

American Journal of Surgical Pathology

Double-hit Lymphomas Constitute a Highly Aggressive Subgroup in Diffuse Large B-cell Lymphomas in the Era of Rituximab.

Sept 2012

Kobayashi T, Tsutsumi Y, Sakamoto N, Nagoshi H, Yamamoto-Sugitani M, Shimura Y, Mizutani S, Matsumoto Y, Nishida K,Horiike S, Asano N, Nakamura S, Kuroda J, Taniwaki M.

Source

1Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto.

Abstract

OBJECTIVE:

The incorporation of rituximab in immunochemotherapy has improved treatment outcomes for diffuse large B-cell lymphoma, but the prognosis for some diffuse large B-cell lymphomas remains dismal. Identification of adverse prognostic subgroups is essential for the choice of appropriate therapeutic strategy.

METHODS:

We retrospectively investigated the impact of so-called 'double-hit' cytogenetic abnormalities, i.e. cytogenetic abnormalities involving c-MYC co-existing with other poor prognostic cytogenetic abnormalities involving BCL2, BCL6 or BACH2, on treatment outcomes for 93 consecutive diffuse large B-cell lymphoma patients.

RESULTS:

According to the revised international prognostic index, no patients were cytogenetically diagnosed with double-hit lymphomas in the 'very good' risk group or in the 'good' risk group, while 5 of 33 patients had double-hit lymphomas in the 'poor' risk group. All the double-hit lymphoma patients possessed both nodal and extranodal involvement. The overall complete response rate was 89.3%, overall survival 87.1% and progression-free survival 75.8% over 2 years (median observation period: 644 days). The complete response rates were 93.2% for the non-double-hit lymphoma patients and 40.0% for the double-hit lymphoma patients. Significantly longer progression-free survival and overall survival were observed for the 'very good' and the 'good' risk patients than for the 'poor' risk patients. Moreover, the progression-free survival of double-hitlymphoma was significantly shorter than that of the non-double-hit lymphoma 'poor' risk patients (P = 0.016). In addition, the overall survival of the double-hit lymphoma patients also tended to be shorter than that of the non-double-hit lymphoma 'poor' risk group.

CONCLUSIONS:

The diagnosis of double-hit lymphoma can help discriminate a subgroup of highly aggressive diffuse large B-cell lymphomas and indicate the need for the development of novel therapeutic strategies for double-hit lymphoma.

Oxford - Japanese Journal of Clinical Oncology

Epstein Barr virus presence in pediatric diffuse large B-cell lymphoma reveals a particular association and latency patterns. Analysis of viral role in tumor microenvironment.

Sept 2012

Cohen M, De Matteo E, Narbaitz M, Carreño FA, Preciado MV, Chabay PA.

Source

Molecular Biology laboratory. Pathology Division. Ricardo Gutiérrez Children's Hospital. Buenos Aires, Argentina. melucohen@ffyb.uba.ar.

Abstract

Non-Hodgkin's lymphoma represents 6-10% of pediatric malignancies, and diffuse large B-cell lymphoma (DLBCL) is one of the 3 major subtypes. The 2008 WHO classification included a new entity, Epstein-Barr virus (EBV)-positive DLBCL of the elderly, affecting patients >50 years. It has been demonstrated that EBV may play a role in tumor microenvironment composition, disturbing anti-tumor immune response and disease progression.

Since most studies were performed in adults, our aim was to assess EBV presence and latency pattern, as well as T-cell microenvironment in a pediatric DLBCL series of Argentina. The study was conducted on formalin-fixed paraffin-embedded biopsies from 25 DLBCL patients. EBERs expression was performed by in situ hybridization, while EBV gene expression was analyzed using real-time PCR. LMP1, LMP2A, CD3, CD4, CD8 and Foxp3 expression were assessed by immunohistochemistry (IHC). Forty percent of cases showed EBV expression, with a significantly higher incidence among patients <10 years (p=0.018), and with immunosuppressed (p=0.023). T-cell subsets were not altered by EBV presence. 

Full EBV latency antigen expression (latency type III) was the most frequently pattern observed, together with BZLF1 lytic gene expression. One patient showed II like pattern (LMP1 without LMP2A expression). Based exclusively on IHC, some patients showed latency II/III (EBERs and LMP1 expression) or I (EBERs only). These findings suggest that EBV association in our series was higher than the previously demonstrated for elderly DLBCL and that EBV latency pattern could be more complex from those previously observed. Therefore, EBV could be an important cofactor in pediatric DLBCL lymphomagenesis.

PubMed

Radiotherapy in the Management of Localized Primary Cutaneous B-Cell Lymphoma.

Aug 2012

Pashtan I, Mauch PM, Chen YH, Dorfman DM, Silver B, Ng AK.

Abstract

Abstract The optimal therapy and radiation dose for patients with localized primary cutaneous B-cell lymphoma (PCBCL) are unknown. We retrospectively identified 23 patients with localized (T1-T2) PCBCL treated with definitive radiation to doses ranging from 30-44 Gy (median, 36 Gy). With median follow-up of 4.8 years, the 5-year overall survival rate was 100%, the relapse-free survival rate was 71% (95% CI, 46-86%) and there were no local recurrences, suggesting that radiotherapy to a dose of 30 Gy may be sufficient for cure.

Informa

Primary diffuse large B-cell lymphoma of the corpora cavernosa presented as a perineal mass.

Apr 2012

Carlos GS, Ivanna VV, Gustavo TM, Joan AC, Javier SM, Luis IS.

Source

Department of Urology, Germans Trias i Pujol Hospital, Badalona, Barcelona, Spain.

Abstract

Primary male genital lymphomas may appear rarely in testis, and exceptionally in the penis and prostate, but there is not previous evidence of a lymphoma arising from the corpora cavernosa. We report the first case in the literature of a primary diffuse cell B lymphoma of the corpora cavernosa presented with low urinary tract symptoms, perineal pain and palpable mass. Diagnosis was based on trucut biopsy, histopathological studies and computed tomographic images

PubMed

Prognostic assessment in patients with indolent B-cell lymphomas.

2012

Arcaini L, Rattotti S, Gotti M, Luminari S.

Source

Division of Hematology, Department of Hematology and Oncology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, 27100 Pavia, Italy.

Abstract

Follicular lymphoma (FL) is an indolent lymphoma with long median survival. Many studies have been performed to build up prognostic scores potentially useful to identify patients with poorer outcome. In 2004, an international consortium coordinated by the International Follicular Lymphoma Prognostic Factor project was established and a new prognostic study was launched (FLIPI2) using progression-free survival (PFS) as main endpoint and integrating all the modern parameters prospectively collected. Low-grade non-Hodgkin lymphomas were once considered as a heterogenous group of lymphomas characterized by an indolent clinical course. Each entity is characterized by unique clinicobiologic features. Some studies have been focused on prognostic factors in single lymphoma subtypes, with the development of specific-entity scores based on retrospective series, for instance splenic marginal zone lymphoma (SMZL). A widely accepted prognostic tool for clinical usage for indolent non-follicular B-cell lymphomas is largely awaited. In this paper we summarized the current evidence regarding prognostic assessment of indolent follicular and non-follicular lymphomas.

Scientific World Journal

Isolated mononeuropathy multiplex-a rare manifestation of intravascular large B-cell lymphoma.

Sept 2012

Lynch KM, Katz JD, Weinberg DH, Lin DI, Folkerth RD.

Source

*Department of Neurology, St Elizabeth's Medical Center, Boston, MA †Department of Neuropathology, Brigham and Women's Hospital, Boston, MA.

Abstract

ABSTRACT: Intravascular large B-cell lymphoma, also known as angiotrophic large cell lymphoma, is a rare disorder where neoplastic lymphoid cells proliferate within the walls of small- to medium-sized blood vessels. Peripheral neuropathy and other neurological manifestations, including stroke and dementia, are common, but cases of isolated multiple mononeuropathies in the absence of systemic symptoms are distinctly rare. We present an unusual case of biopsy-proved angiotrophic large cell lymphoma presenting exclusively with multiple mononeuropathies.

PubMed