scholarly journals Low-Grade B Cell Lymphoproliferative Disorder Masquerading as Chronic Rhinosinusitis

Sinusitis ◽  
2021 ◽  
Vol 5 (1) ◽  
pp. 1-4
Author(s):  
Rory Chan ◽  
Chris RuiWen Kuo ◽  
Brian Lipworth

Chronic rhinosinusitis (CRS) is one of the most common persistent disorders of the developed world, requiring input from various specialists including primary care physicians, otolaryngologists, respiratory physicians, and allergologists. B-cell lymphoproliferative disorders (BLPDs) are a heterogenous group of malignant conditions defined by an accumulation of mature B lymphocytes in the bone marrow, blood, and lymphoid tissues. We present a case report of an elderly man with rhinosinusitis-like symptoms and atypical features prompting further investigations that culminated in a diagnosis of BLPD.

2013 ◽  
Vol 44 (10) ◽  
pp. 2139-2148 ◽  
Author(s):  
Zenggang Pan ◽  
Qingmei Xie ◽  
Susan Repertinger ◽  
Bill G. Richendollar ◽  
Wing C. Chan ◽  
...  

2006 ◽  
Vol 130 (4) ◽  
pp. 558-560 ◽  
Author(s):  
Barbara Semakula; ◽  
Jon V. Rittenbach ◽  
Jun Wang

Abstract Posttransplantation lymphoproliferative disorders (PTLD) are a heterogeneous group of monoclonal or polyclonal lymphoproliferative lesions that occur in immunosuppressed recipients following solid organ or bone marrow transplantation, including 4 categories: (1) early lesions (reactive plasmacytic hyperplasia, and infectious-mononucleosis–like PTLD), (2) polymorphic PTLD, (3) monomorphic PTLD (including B-cell neoplasms and T-cell neoplasms), and (4) Hodgkin lymphoma (HL) and HL-like PTLD in the current World Health Organization classification. Although HL-like PTLD has been grouped with classic HL PTLD, controversy remains as to whether it is truly a form of HL or whether it should be more appropriately considered as a form of B-cell PTLD. The current available literature data indicate the presence of important immunophenotypic, molecular genetic, and clinical differences between HL PTLD and HL-like PTLD, suggesting that HL-like PTLD is in fact most often a form of B-cell PTLD. Distinction from true HL may be important for clinical management and prognosis.


Blood ◽  
1997 ◽  
Vol 90 (2) ◽  
pp. 766-775 ◽  
Author(s):  
Bruno Royer ◽  
Dominique Cazals-Hatem ◽  
Jean Sibilia ◽  
Felix Agbalika ◽  
Jean-Michel Cayuela ◽  
...  

Abstract The occurrence of non-Hodgkin's lymphoma (NHL) is the most serious complication of Sjögren's syndrome (SS). We performed a study of 16 NHLs occurring in patients with an underlying SS. These lymphomas arose not only in salivary glands (7 cases) but also in other mucosal extranodal sites (the stomach [4 cases], the lung [3 cases], the skin [3 cases], the buccal mucosa [1 case], the thymus [1 case]) and in nodal sites (8 cases). Low-grade marginal zone lymphomas (MZL) were diagnosed in 12 of the 16 patients, 9 of mucosa-associated lymphoid tissues (MALT) type in mucosal sites and 3 exclusively nodal. The 4 other patients presented with a high-grade B-cell lymphoma that was probably a histological transformation of an underlying low-grade MZL at least in 3 of the cases involving skin, stomach, and parotid, respectively. A t(14; 18) translocation was detected in 1 of 8 lymphomas tested. We detected serum anti-p53 antibodies in 2 of the 14 studied patients. p53 protein was detected in 1 of 11 lymphomas tested. LMP protein and Eber RNAs of Epstein-Barr virus (EBV) were not detected in the 16 NHL biopsies. Using polymerase chain reaction, EBV was never detected except in 1 of 4 parotid lymphomas. No human T-lymphotropic virus 1 or human herpes virus 8 DNAs were detected in NHL biopsies. None of the patients had hepatitis C virus infection found using serological methods. Chemotherapy was usually efficient. In conclusion, lymphomas occurring in patients with an underlying SS are in most cases MZL. These lymphomas are not associated with viruses known to be present in other types of lymphomas. Some of the translocations or mutations of oncogenes or antioncogenes described in other lymphomas are detected in SS-associated lymphomas.


Blood ◽  
1996 ◽  
Vol 88 (1) ◽  
pp. 222-229 ◽  
Author(s):  
KF Norrback ◽  
K Dahlenborg ◽  
R Carlsson ◽  
G Roos

Abstract Activation of telomerase seems to be a prerequisite for immortalization and is found in permanent cell lines and most malignant tumors. Normal somatic cells are generally telomerase negative, except for bone marrow stem cells. Weak activity is also present in peripheral blood cells. In the present study strong telomerase activity was demonstrated in vivo in normal mature cells of the immune system, as well as in malignant lymphomas. Benign lymph nodes had lower telomerase activity than benign tonsils, which exhibited intermediate to high activity comparable with findings in malignant lymphomas. In benign tonsils the activity seemed to be restricted to germinal center B cells. In benign lymphoid tissues telomerase activity correlated with B-cell numbers and cell proliferation, but this was not observed in the lymphoma group. High- grade lymphomas exhibited higher levels of telomerase compared with low- grade cases. The data showed that in vivo activation of telomerase is a characteristic feature of germinal center B cells. Different signals for activation of telomerase are likely to exist, one of them being immune stimulation. The data suggest that telomerase activity in malignant lymphomas can be explained by an “induction and retention” model, ie, transformation occurs in a normal, mature B cell with reactivated telomerase, which is retained in the neoplastic clone.


2002 ◽  
Vol 39 (1) ◽  
pp. 183-188 ◽  
Author(s):  
Sanjeev Sethi ◽  
Barry P. Cuiffo ◽  
Geraldine S. Pinkus ◽  
Helmut G. Rennke

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1673-1673 ◽  
Author(s):  
Neha Bhatnagar ◽  
Faith Wright ◽  
Dimpna McAleese ◽  
Christopher J McNamara

Abstract Abstract 1673 Introduction: Mature B-cell lymphoproliferative disorders that present with a leukemic phase may have overlapping morphological and immunophenotypic features. Accurate diagnosis is essential to determine prognosis and therapy. B-cell chronic lymphocytic leukaemia (B-CLL) and mantle cell lymphoma (MCL) both express CD5 but usually have divergent clinical courses. Conventional markers are able to discriminate in many cases but a significant proportion of cases require more detailed analysis. CD200 is a membrane glycoprotein that has been reported to differentiate between B-CLL and MCL. It is expressed on resting and activated T and B cells, but not on NK cells, monocytes, granulocytes or platelets. We prospectively assessed the utility of this antibody in a single centre, servicing haematology units in North London. Methods: Over a 12 month period we prospectively assessed the expression of CD200 (Clone MRC OX-104, BD Pharmingen) on neoplastic cells of patients with presenting with mature B-cell lymphoproliferative disorders. EDTA anti-coagulated peripheral blood and bone marrow underwent a lyse/wash technique before being acquired on a BD FACS Canto II flow cytometer equipped with DIVA 6.1.2 software. A lymphoid gating strategy was used to analyse the data and expression of more than 20% of nucleated cells referenced to a negative internal control was considered positive. A diagnosis was made using all available data according to the WHO classification of haematopoietic tumours, including the 5 point CLL score. The predictive value of CD200 expression was subsequently assessed. Results: There were 100 patients (58M: 42F) with a median age of 70 years (range 29–92). CD200 was present on the neoplastic cells of all 78 patients with B-CLL; in all 7 MCL patients CD200 was negative; 15 patients had CD5 negative B-cell lymphoproliferative disorder with variable expression of CD200. The specificity of CD200 negativity in the setting of CD5 positive B-cell clone was 100% for MCL. The positive predictive value of CD200 for CLL was 97.5% with a sensitivity of 100% and a Pearson's correlation coefficient of 1. Conclusion: Our results confirm CD200 expression in the neoplastic cells of B-CLL patients but not in MCL. This antibody may contribute to the accurate differentiation between B-CLL and MCL in patients presenting with the diagnostic problem of a CD5- positive lymphoproliferative disorder. We propose to add CD200 to our diagnostic B-cell lymphoma panel. Further studies are needed to determine whether CD200 could replace other immunophenotypic strategies currently used in this situation, such as CD23, FMC7 and intensity of CD22/CD79b expression. Disclosures: No relevant conflicts of interest to declare.


2001 ◽  
Vol 23 (2) ◽  
pp. 139-142 ◽  
Author(s):  
C. Christopoulos ◽  
A. Tassidou ◽  
S. Golfinopoulou ◽  
G. Anastasiadis ◽  
S. Manetas ◽  
...  

2021 ◽  
Vol 28 (6) ◽  
pp. 5124-5147
Author(s):  
John J. Schmieg ◽  
Jeannie M. Muir ◽  
Nadine S. Aguilera ◽  
Aaron Auerbach

CD5-negative, CD10-negative low-grade B-cell lymphoproliferative disorders (CD5-CD10-LPD) of the spleen comprise a fascinating group of indolent, neoplastic, mature B-cell proliferations that are essential to accurately identify but can be difficult to diagnose. They comprise the majority of B-cell LPDs primary to the spleen, commonly presenting with splenomegaly and co-involvement of peripheral blood and bone marrow, but with little to no involvement of lymph nodes. Splenic marginal zone lymphoma is one of the prototypical, best studied, and most frequently encountered CD5-CD10-LPD of the spleen and typically involves white pulp. In contrast, hairy cell leukemia, another well-studied CD5-CD10-LPD of the spleen, involves red pulp, as do the two less common entities comprising so-called splenic B-cell lymphoma/leukemia unclassifiable: splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia variant. Although not always encountered in the spleen, lymphoplasmacytic lymphoma, a B-cell lymphoproliferative disorder consisting of a dual population of both clonal B-cells and plasma cells and the frequent presence of the MYD88 L265P mutation, is another CD5-CD10-LPD that can be seen in the spleen. Distinction of these different entities is possible through careful evaluation of morphologic, immunophenotypic, cytogenetic, and molecular features, as well as peripheral blood and bone marrow specimens. A firm understanding of this group of low-grade B-cell lymphoproliferative disorders is necessary for accurate diagnosis leading to optimal patient management.


Blood ◽  
1994 ◽  
Vol 84 (3) ◽  
pp. 733-738 ◽  
Author(s):  
S O'Brien ◽  
R Kurzrock ◽  
M Duvic ◽  
H Kantarjian ◽  
S Stass ◽  
...  

Abstract Mature T-cell lymphoproliferative disorders comprise a heterogenous group of diseases for which there is no standard therapy. These disorders are uncommon, and are usually treated similarly to their B- cell counterparts, but with less success. Nucleoside analogues have proven effective in indolent B-cell disorders but have been less well explored in T-cell malignancies. We treated 22 patients with mature T- cell lymphoproliferative diseases with 2-chlorodeoxyadenosine (2-CDA) administered as a continuous infusion at a daily dose of 4 mg/m2 over 7 days. Nineteen of the patients had received prior therapy with a median number of prior regimens of three. Eleven patients had leukemia or large granular lymphocytosis, eight patients had mycosis fungoides, and three had T-cell lymphoma. Nine patients (41%) responded to 2-CDA. Four of the patients had responses that were complete remissions, and three of these four patients remain in remission at 23, 24, and 23 months. The only important toxic effects were fever or infection, seen during 38% of courses. In conclusion, 2-CDA appears to be an effective therapy in T-cell lymphoproliferative disorders and deserves wider evaluation in this subset of patients.


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