scholarly journals Bone Marrow-Liver-Spleen Type of Large B-Cell Lymphoma Associated with Hemophagocytic Syndrome: A Rare Aggressive Extranodal Lymphoma

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Kirill A. Lyapichev ◽  
Jennifer R. Chapman ◽  
Oleksii Iakymenko ◽  
Offiong F. Ikpatt ◽  
Uygar Teomete ◽  
...  

Recently, an unusual subtype of large B-cell lymphoma (LBCL) with distinctive clinicopathologic features has been recognized; it is characterized by involvement of bone marrow with or without liver and/or spleen, but no lymph node or other extranodal sites, usually associated with fever, anemia, and hemophagocytic lymphohistiocytosis (HLH). Because of this distinctive clinical presentation, it has been designated “bone marrow-liver-spleen” (BLS) type of LBCL. To date there is only one series of 11 cases of BLS type of LBCL with detailed clinical, pathologic, and cytogenetic data. Herein, we describe a case of BLS type LBCL presenting with associated HLH in a 73-year-old female. The bone marrow core biopsy showed cytologically atypical large lymphoma cells present in a scattered interstitial distribution and hemophagocytosis and infrequent large lymphoma cells were seen in the bone marrow aspirate smears. Circulating lymphoma cells were not seen in the peripheral blood smears. The patient underwent treatment with chemotherapy (R-CHOP) but unfortunately passed away 2 months after initial presentation. BLS type of LBCL is a very rare and clinically aggressive lymphoma whose identification may be delayed by clinicians and hematopathologists due to its unusual clinical presentation and pathologic features.

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 7588-7588
Author(s):  
K. Lee ◽  
J. Yi ◽  
I. Choi ◽  
J. Kim ◽  
D. Kim ◽  
...  

7588 Background: Although several studies have been reported about non-Hodgkin’s lymphomas (NHL) with bone marrow (BM) involvement, most of them have not performed immunophenotypic studies and contained heterogeneous NHL histologies. Until now, only a few studies with small sample sizes have been reported about clinical characteristics and prognostic factors in diffuse large B-cell lymphoma (DLBCL) with BM involvement. Methods: Between January 1993 and March 2004, 486 patients were diagnosed with DLBCL. Among 84 DLBCL patients who had BM involvement at initial diagnosis, 9 were not ineligible because of the lack of clinical data or unavailability of BM specimen. So, clinical factors and patterns of BM involvement of 75 patients were analyzed in this study. Results: At initial diagnosis, the median age was 57 years (range: 25∼79). In addition to BM, lymph nodes (76%), spleen (23%), Waldeyer’s ring (19%), gastrointestinal tract (16%), lung/pleura (15%), bone (15%), central nervous system (9%), nasal cavity (8%) and liver (7%) were also involved. Among 75 patients, 67 patients received anthracycline-containing chemotherapy; 4 patients received non-anthracycline-containing chemotherapy and 4 could not receive systemic chemotherapy because of combined medical conditions. The median survival was 32.3 months (5-year overall survival [OS]: 38%). In univariate analysis for prognostic factors, high-intermediate or high international prognostic index (IPI), B-symptoms, leucopenia, anemia, thrombocytopenia, pattern of BM involvement (interstitial or diffuse pattern), > 10% replacement of BM area by lymphoma cells, > 10% of large cell infiltration in BM-involved area by lymphoma at initial diagnosis were associated with poor OS (p < 0.05). Multivariate analysis indicated that > 10% replacement of BM area by lymphoma cells (p < 0.001), peripheral thrombocytopenia (p = 0.001) and high-intermediate or high IPI (p = 0.042) were independent predictors of poor OS. Conclusions: To our knowledge, this is the largest study about DLBCL patients with BM involvement. The BM areas involved by lymphoma cells, peripheral thrombocytopenia and IPI at initial diagnosis are independent prognostic factors in these patients. No significant financial relationships to disclose.


1999 ◽  
Vol 17 (8) ◽  
pp. 2479-2479 ◽  
Author(s):  
Philippe C. Bishop ◽  
Wyndham H. Wilson ◽  
Debra Pearson ◽  
John Janik ◽  
Elaine S. Jaffe ◽  
...  

PURPOSE: The risk of CNS involvement by non-Hodgkin's Lymphoma (NHL) has been associated with bone marrow and/or testicular involvement; however, it was recently reported that the number of extranodal sites is a more reliable predictor of CNS disease. Because primary mediastinal thymic large B-cell lymphoma (PMLCL) has a high propensity for involving extranodal sites, we investigated the frequency and pattern of CNS involvement in PMLCL. PATIENTS AND METHODS: The medical records of 219 patients with aggressive NHL, consecutively entered onto protocols at the National Cancer Institute between 1987 and 1998, were retrospectively reviewed. RESULTS: Twenty-three patients (11%) had clinical and pathologic features of PMLCL. These patients were young (median age, 29 years), female (61%), and presented with massive mediastinal adenopathy (70%). Extranodal disease occurred at presentation in 70% and at relapse in 93% of patients and involved contiguous intrathoracic structures and/or distant sites, including the lungs, kidneys, liver, adrenals, ovaries, pancreas, and bone. Six patients (26%) developed CNS involvement, two (9%) at presentation and four (27%) at relapse. All had extranodal disease, but only one had bone marrow involvement. Parenchymal and leptomeningeal CNS disease occurred in four and three patients, respectively. CONCLUSION: CNS involvement in PMLCL is associated with extranodal involvement other than bone marrow and may reflect the unique biology of this disease. The propensity to involve the CNS parenchyma raises the concern that intrathecal prophylaxis may not be effective and suggests that CNS imaging should be considered in patients with extranodal disease.


2010 ◽  
Vol 152 (2) ◽  
pp. 237-238 ◽  
Author(s):  
Yasuo Miura ◽  
Yusuke Matsui ◽  
Noriko Sugino ◽  
Yuko Nakato ◽  
Hideyo Takeda ◽  
...  

2014 ◽  
Vol 60 (06/2014) ◽  
Author(s):  
Yi-Qun Che ◽  
Peng Liu ◽  
Yang Luo ◽  
Di Shen ◽  
Jia-Jie Hao ◽  
...  

BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wei Wang ◽  
Yan Li ◽  
Xavier Rivera Rivera ◽  
Linjun Zhao ◽  
Di Mei ◽  
...  

Abstract Background Flow cytometry plays a key role in detecting bone marrow (BM) involvement in patients with diffuse large B-cell lymphoma (DLBCL). To improve its detection sensitivity, we need to explore novel markers. In this study, we detected the expression CD54 on lymphoma cells in BM specimens from DLBCL patients and clarified its diagnostic significance in BM involvement by DLBCL. Methods We collected BM specimens from 76 patients with DLBCL (germinal center B-cell (GCB) = 25, non-GCB = 51) and 10 control patients without lymphoma. We detected and compared the expression of CD54 on lymphoma cells and normal mature B cells by using 10-color panels. Results Normal plasma cells expressed a higher level of CD54 as compared with hematogones (p < 0.05) and normal mature B cells (p < 0.05). Among 76 patients, 23 of them (GCB = 12, non-GCB = 11) had BM involvement. Lymphoma B cells from 12 cases (GBC = 4, non-GCB = 8) expressed a higher level of CD54 compared to normal mature B cells (p < 0.05). Additionally, lymphoma cells of the non-GCB subtype frequently expressed a higher level of CD54 in comparison to the GCB subtype (p < 0.05). And the high expression of CD54 was not related to plasmacytoid differentiation. Conclusion Aberrant expression of CD54 on lymphoma cells is frequently seen in patients’ BM specimens involved by DLBCL, especially in the non-GCB subtype. CD54 could be used as a new marker to gate on lymphoma cells and improve the detection sensitivity of BM involvement in patients with DLBCL.


2021 ◽  
Vol 39 (S2) ◽  
Author(s):  
J. Devin ◽  
T. Cañeque ◽  
Y.‐L. Lin ◽  
L. Mondoulet ◽  
J.‐L. Veyrune ◽  
...  

2020 ◽  
Vol 17 (17) ◽  
pp. 2735-2743
Author(s):  
Linjun Hu ◽  
Junjun Zhao ◽  
Yang Liu ◽  
Xin Liu ◽  
Qiliang Lu ◽  
...  

Author(s):  
Xinying Zhao ◽  
Xudong Guo ◽  
Wenqin Yue ◽  
Jianmin Wang ◽  
Jianmin Yang ◽  
...  

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