Can Cutaneous Low-Grade B-cell Lymphoma Transform Into Primary Cutaneous Diffuse Large B-cell Lymphoma? An Immunohistochemical Study of 82 cases

AbstractLineage switch involves change in the phenotypic characteristics from one type to another. It is a rare phenomenon described in mature lymphoid neoplasms which transform to histiocytic/dendritic cell tumor, more commonly described in low-grade lymphoma like follicular, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), wherein the neoplasm loses the phenotypic characteristics of non-Hodgkin lymphoma and acquires the markers of histiocytic differentiation. Here, we present a case of diffuse large B cell lymphoma transforming to histiocytic sarcoma post 6 months of start of therapy. Histiocytic sarcoma being a very aggressive tumor, the patient had a very rapid deteriorating course and succumbed to disease.

Download Full-text

Clinical Features and Outcome of 147 Patients with Diffuse Large B-Cell Lymphoma and Hepatitis C Virus Infection.

Blood ◽

10.1182/blood.v106.11.1921.1921 ◽

2005 ◽

Vol 106 (11) ◽

pp. 1921-1921

Author(s):

Carlo Visco ◽

Luca Arcaini ◽

Michele Merli ◽

Annalisa Andreoli ◽

Sara Burcheri ◽

...

Keyword(s):

Hepatitis C Virus ◽

Hepatitis C ◽

B Cell ◽

Cell Lymphoma ◽

International Prognostic Index ◽

B Cell Lymphoma ◽

Hcv Infection ◽

Low Grade ◽

Large B Cell Lymphoma ◽

Large B Cell

Abstract Hepatitis C virus (HCV) has been implicated in the pathogenesis of a subset of low-grade non-Hodgkin lymphomas. Furthermore, diffuse large B-cell lymphoma (DLBCL) has been correlated to HCV infection in several series from our geographical area (north-east of Italy), but little is known about the characteristics of such high-grade tumors. We analyzed presentation features of 147 previously untreated HCV-positive patients with DLBCL who presented to the three participating centers between 1993 and 2004. All patients were provided with complete clinical information, were HIV negative, and had been tested at tumor onset for HCV antibodies by ELISA and RIBA. Median age at presentation was 64 years old (range 29–88), 47% were males, ECOG performance status was >1 in 20%, Ann Arbor stage was I in 20%, II in 27%, III in 26%, IV in 27%, and B-symptoms were present in 37% of patients. The International Prognostic Index (IPI) value at diagnosis was low in 18%, int/low in 23%, int/high in 32%, and high in 27% of patients. Surprisingly, DLBCL transformed from a low-grade histology represented only 7% of the whole population, while primary mediastinal DLBCL were extremely rare (1/147, <1%). Patients frequently presented as primary extranodal DLBCL (65/147, 44%). Most involved extranodal sites were skin, liver, stomach, and spleen, with the latter being the most represented syte (33% of patients). Remarkably, spleen was the only extranodal involved organ in 20% of patients. Treatment was delivered with cure-intent, and consisted of CHOP-like regimens +/− Rituximab for the large majority of patients, except for 16 (11%) patients with cirrhosis or severe hepatic dysfunction, who received mono-chemotherapy or radiotherapy. Only three (2%) HCV-positive patients had to discontinue chemotherapy due to liver function impairment. The addition of Rituximab to chemotherapy did not seem to affect patients’ tolerance to treatment. With a median follow-up of 48 months for survivors, 5-year overall survival (OS) was 75%, while 5-year failure-free survival (FFS) was 51%. In particular, the 65 patients with primary extranodal DLBCL shared a better 5-year OS (83% vs 71%, p=0.01) and FFS (75% vs 39%, p=0.009) than their nodal counterpart. Nodal origin of the tumor resulted the strongest independent adverse factor both in terms of OS and FFS in multivariate analysis. The peculiar clinical behavior shared by HCV-positive DLBCL may disclose relevant biological features of these tumors, and may be relevant for future studies aiming to clarify the link between HCV infection and aggressive lymphoproliferative disorders.

Download Full-text

Primary and Secondary Ocular Adnexal Lymphoma: A Single Institutional Experience

Blood ◽

10.1182/blood.v118.21.4956.4956 ◽

2011 ◽

Vol 118 (21) ◽

pp. 4956-4956

Author(s):

Darcie Deaver ◽

Kenneth S. Zuckerman ◽

Celeste M. Bello ◽

Eduardo M. Sotomayor ◽

Salvador Bruno ◽

...

Keyword(s):

B Cell ◽

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Staging System ◽

Low Grade ◽

Time To Progression ◽

Ocular Adnexal Lymphoma ◽

Large B Cell Lymphoma ◽

Large B Cell

Abstract Abstract 4956 Background: The incidence of ocular adnexal lymphoma (OAL) is rare and usually presents in the setting of central nervous system (CNS) involvement. There are no rigid guidelines for the treatment of OAL, most probably because of the variety of characteristics of the disease. Objectives: To analyze clinical pathological features, therapy and outcomes of patients with primary and secondary OAL. Research Design and Methods: Retrospective chart review of 17 consecutive patients diagnosed with OAL at Moffitt Cancer Center from 2004–2011. Characteristics of the participants were median age 68 years, 15 (88%) white, 2 (12%) Hispanic, and 11 (65%) male. Chlamydia serology testing was negative in all patients tested. Secondary OAL patients were staged per the Ann Arbor Staging System, 2 (22%) stage III and 7 (78%) stage IV. The primary OAL patients were staged utilizing the TNM staging system for OALs (Coupland et al, Arch Pathol Lab Med, 2009). Six (75%) patients were stage T1 and 2 (25%) patients were stage T2. Results: Seventeen patients with a diagnosis of OAL were evaluated in our institution. Patients with OAL are commonly stratified into 2 groups, primary and secondary. Eight (50%) of the patients were diagnosed with primary OAL; of these there were 4 (50%) marginal zone, 3 (37%) diffuse large B cell, and 1 (12%) follicular lymphoma. Nine (50%) patients were diagnosed with secondary OAL; 4 (44%) marginal zone, 1 (11%) diffuse large B cell, 1 (11%) mantle cell, 1 (11%) CLL, and 2 (22%) progressed from low grade to diffuse large B cell lymphoma. In the primary OAL, radiation in combination with systemic chemotherapy was the preferred treatment in diffuse large B cell lymphoma and radiation was preferred in patients with low-grade lymphoma. In secondary OAL, systemic chemotherapy was the preferred treatment for aggressive lymphoma. The choice of systemic Rituximab, radiation, or observation was the preferred treatment of low-grade lymphoma. Aggressive primary OAL had a relapse rate of 2 (66%) patients with a median time to progression of 8 months. Aggressive secondary OAL demonstrated a relapse rate of 50% with median time to progression 6 months. All patients who experienced relapsed disease received salvage chemotherapy. No cases of relapse were observed in the low-grade, primary or secondary, OAL patients. Median duration of response in low-grade primary lymphoma was 6 months and the low-grade secondary lymphoma was 54 months. Conclusion: In our patient population diffuse large B cell lymphoma and marginal zone lymphoma were the most common diagnoses. Ocular adnexal lymphoma has been associated with the presence of CNS disease and it is estimated that 80–90% of patients diagnosed with OAL will experience progression to the CNS. Treatment depends on the extent of disease and the subtype of lymphoma that is histologically identified. Treatment may consist of involved field radiation in localized disease and has approximately less than 10% local recurrence rate. Intravitreal methotrexate and itraorbital injections of Rituximab or a combination of localized radiation and systemic high dose methotrexate are also options for treatment. In the event that there is systemic disease, single agent IV Rituximab or standard chemotherapy regimens such as CHOP-R or CVP-R in conjunction with ocular directed therapy. When disease is localized to the ocular compartment, the burden of disease is low and there is a greater chance of eradicating the disease. The delay in diagnosis increase the risk of CNS involvement and decreases overall survival. Disclosures: No relevant conflicts of interest to declare.

Download Full-text

Bone Marow (BM) Involvement in Diffuse Large B Cell Lymphoma (DLBCL): Clinical Impact of Discordant Disease.

Blood ◽

10.1182/blood.v108.11.2031.2031 ◽

2006 ◽

Vol 108 (11) ◽

pp. 2031-2031

Author(s):

Mukesh Chhanabhai ◽

Joseph Connors ◽

Wayne Seville ◽

Dan Matso ◽

Randy Gascoyne

Keyword(s):

Bone Marrow ◽

B Cell ◽

De Novo ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Clinical Impact ◽

Low Grade ◽

Large B Cell Lymphoma ◽

Clinical Records ◽

Large B Cell

Abstract Background and Methods: Most published studies have suggested that survival of de novo DLBCL with discordant BM involvement by small B cell lymphoma is indistinguishable from patients having a negative staging BM. The aim of the study was to investigate the incidence and clinical impact of BM involvement by concordant and discordant B cell lymphoma in patients with DLBCL seen in a single institution over a 5 year period (1\1\2000 – 31\12\2004). The cases were identified from pathology records and BCCA Lymphoid Cancer Database. Results: The group of interest for this study comprised 652 patients with de novo DLBCL with staging marrow available for review. 60 of 652 (9.2%) of patients with DLBCL had concordant large B-cell lymphoma in their bone marrow. 523 (80.2%) were negative, 16 patients showed what we considered to be atypical lymphoid (ALH) infiltrates lacking definitive features of malignancy. In 50 patients (7.7%) the bone marrow showed discordant histology with predominantly small B-cells, some showing paratrabecular localization. In total there were five cases of TCRBCL in the study, three with marrow involvement. Furthermore, all 54 cases of primary mediastinal B-cell lymphoma (PMBCL) had a negative BM. Of the 652 cases with DLBCL with staging bone marrows available, merging the pathology and clinical databases resulted in 599 patients with complete clinical records. Of these, bone marrows were either not done or deemed inadequate in 101 cases. Therefore, there were 488 patients with DLBCL with an interpretable marrow of which 344 had advanced stage disease. Staging marrows in these patients were negative = 264, positive 41, discordant 28, and ALH 11. The overall survival and progression free survival were strongly affected by the IPI score for these 344 cases (P<0.00001) (see figure). Compared to those without BM involvement patients with concordant large B-cell lymphoma in their BM (n=41) had the worst outcome and those with discordant small B-cell lymphoma in the bone marrow (n=28) had an inferior but intermediate outcome (median survivals (months) = not reached;12 and 20, respectively). Conclusion: Diffuse large B-cell lymphoma is a heterogenous group of lymphomas as demonstrated by gene expression profiling. Our data suggests that discordant low grade B-cell lymphoma in patients who have coincident DLBCL has a poorer prognosis and the presence or absence of BM disease has clinical significance. Though we had very few cases, TCRBCL show a higher incidence of BM involvement in keeping with the reported literature. The absence of marrow disease in PMBCL is consistent with recent data indicating it is a biologically distinct form of DLBCL. Figure Figure

Download Full-text

Non-Hodgkin lymphoma across the pediatric and adolescent and young adult age spectrum

Hematology ◽

10.1182/asheducation-2016.1.589 ◽

2016 ◽

Vol 2016 (1) ◽

pp. 589-597 ◽

Cited By ~ 18

Author(s):

John T. Sandlund ◽

Mike G. Martin

Keyword(s):

B Cell ◽

Anaplastic Large Cell Lymphoma ◽

Cell Lymphoma ◽

Tumor Biology ◽

B Cell Lymphoma ◽

Large Cell ◽

Low Grade ◽

Large B Cell Lymphoma ◽

Large Cell Lymphoma ◽

Large B Cell

Abstract The non-Hodgkin lymphomas (NHLs) occurring in children and adolescents and young adults (AYA) are characterized by various age-related differences in tumor biology and survival. Children generally present with high-grade lymphomas, such as Burkitt lymphoma, diffuse large B-cell lymphoma, lymphoblastic lymphoma, and anaplastic large cell lymphoma, whereas low-grade histologic subtypes, such as follicular lymphoma, occur more frequently with increasing age. Treatment outcome for children with NHL is generally superior to that observed in adults. Factors contributing to this discrepancy include psychosocial factors, patient factors, and differences in tumor biology and therapy. These factors will be reviewed, with particular attention to the biological features of diffuse large B-cell lymphoma and anaplastic large cell lymphoma and corresponding therapeutic challenges. Novel targeting agents have been developed, which have been shown to be active in some patients. There is clearly a need for treatment protocols with eligibility criteria that cover the full span of the pediatric and AYA age range and that incorporate detailed molecular characterization of the tumors.

Download Full-text