The immunophenotypic spectrum of primary mediastinal large b. Mediastinal grey zone lymphoma from the lysa full text view. A rare case of grey zone lymphoma successfully treated. Pathogenesis, diagnosis, and treatment of composite lymphomas. Such composite lymphomas can be combinations of two nonhodgkin lymphomas or a combination of a nonhodgkin lymphoma and a hodgkins lymphoma.
In 2008, the who defined two provisional categories, including mediastinal grey zone lymphomas mgzl biopsies showing overlapping features between primary mediastinal large cell lymphoma pmbcl and nodular sclerosis hodgkin lymphoma nshl and cases that lie between diffuse large b cell lymphoma dlbcl and burkitt lymphoma bl. World health organization bcell lymphoma, unclassifiable, with features intermediate between dlbcl and chl. Mediastinal gray zone lymphoma has a distinct epigenetic profile intermediate between chl and pmbl but different from that of dlbcl are grey zone cases different from composite or sequential cases. Abstractor notes prior to 2010, the term for this code was composite hodgkin and nonhodgkin lymphoma. The 2008 who lymphoma classification introduced the provisional category of bcell lymphoma, unclassifiable with features intermediate between dlbcl and chl to encompass cases that do not fulfill the morphological andor phenotypic criteria for pmbl or chl in the mediastinum but exhibit transitional features between these two entities mgzl.
To identify aggressively behaving classical hodgkin lymphoma chl of mediastinum and primary mediastinal bcell lymphoma pmbcl. Main subclass only acquired code subclassification other. New promise of cancer immunotherapy to treat rare lymphoma. The term grayzone lymphoma has been used to denote a group of lymphomas with overlapping histological, biological, and clinical features. However, as diagnostic criteria are imprecise, reliable identification of gzl cases remains challenging.
Although several studies have evaluated different therapeutic. Marginal zone lymphoma, malt hepatosplenic gd lymphoma marginal zone lymphoma, nodal subcutaneous panniculitislike marginal zone lymphoma, splenic anaplastic large cell lymphoma, system. Given the rarity of this diagnosis and a paucity of prospective randomized trials, there is no clear standard of care for treatment of either the initial diagnosis or relapse of gray zone lymphoma. Mediastinal bcell lymphomas present in the mediastinum of young patients and are mostly represented by nodular sclerosis hodgkin lymphoma nshl and primary mediastinal bcell lymphoma pmbl. Clinicopathologic consensus study of gray zone lymphoma with. Original article intrahospital survival of nonhodgkins.
I did 8 rounds of epochr and was one of the original receptients of this chemo. This information page is about rare types of highgrade bcell lymphoma that are difficult to classify. Gray zone lymphoma genetic and rare diseases information. It can affect people of all ages and is the most common blood cancer. Like most people we had never heard of mgzl and i have found there are only around 300 cases a year. Gray zone lymphoma is a rare type of lymphoma, cancer of a part of the immune system called the lymph system. A prospective study of mediastinal grayzone lymphoma. Heras, kaleigh thomas, and jennie matuschak ari 20. Gray zone lymphoma has features of classical hodgkinlymphoma and pmbl. B mediastinal gray zone lymphoma with morphological features reminiscent of.
These lymphomas are most commonly associated with mediastinal disease, but similar cases have been reported in peripheral lymph node groups as the primary site. Bcell lymphoma, lymphoma, lymphoma in childhood introduction the term gray zone lymphoma was 1st used in 1998 in the proceedings of the workshop on hodgkins disease and related diseases 1 to describe cases of malignant lymphoma at the borderline that could not be reliably classified as hodgkin lymphoma hi. My wife was recently diagnosed with mgzl mediastinal gray zone lymphoma. If you like to talk about some of the side effects i would glad to lend you my ear. Gray zone lymphoma, often presenting as large tumors in the mediastinum, is a type of lymphoma that is characterized by having cellular features of both classic hodgkins lymphomas chl and large bcell lymphomas sources. The grayzone investigative journalism on politics and empire. Treatment of grey zone lymphoma using the rcodoxmrivac. The name of this lymphoma refers to the fact that cancer cells in this condition are in a gray zone an uncertain category because they appear similar to that of two other types of lymphoma, classical hodgkin lymphoma and mediastinal large bcell lymphoma. In the united states and other developed countries, the most common subtypes are burkitt lymphoma, diffuse large b cell lymphoma, lymphoblastic t cell or b cell lymphoma, and anaplastic large cell lymphoma. Grey zones in the differential diagnosis of lymphoma pathology. It is extremely difficult to identifiy in some cases, and misdiagnsosis can greatly complicate treatment and lower outcome. Gray zone lymphoma gzl with features intermediate between classical hodgkin lymphoma chl and diffuse large bcell lymphoma dlbcl. Mediastinal gray zone lymphoma is intermediate between classic hodgkins lymphoma and primary mediastinal bcell lymphoma.
She has rosacea which is an autoimmune disease and we think that made her more susceptible to lymphoma. Appraise recent clinical trial data on the use of panobinostat in combination with chemotherapy for relapsed or refractory hodgkin lymphoma. The term gray zone lymphoma was firstly used in 1998 at the workshop on hodgkins disease and related diseases to. Au 1 fact sheet grey zone lymphoma gzl overview lymphoma is the 6th most common cancer in australia in adult men and women. Dec 29, 2015 grey zone lymphoma gray zone lymphoma is a cancer of the immune system. These lymphomas have been reported in the literature using different terms, such as borderline lymphomas, bcell lymphomas unclassifiable, atypical burkitt lymphoma, burkittlike lymphomas, or gray zone lymphomas. Treatment of grey zone lymphoma using the rcodoxmrivac p. Grayzone lymphoma between chl and large bcell lymphoma. The morphology of gray zone lymphoma gzl is variable with tumor cells spanning the spectrum of diffuse large bcell lymphoma dlbcl and classical hodgkin lymphoma. Jun 14, 2018 gray zone lymphoma shares features with two other types of lymphoma, classical hodgkin lymphoma chl and diffuse large bcell lymphoma dlbcl. Mediastinal gray zone lymphoma mgzl has immunopathologic features between classical hodgkin lymphoma chl and primary mediastinal thymic bcell lymphoma pmbl, leading to uncertainty regarding its biological relationship to these entities. It usually presents as a mediastinal mass, asdoes pmbl, and demonstrates histological, immunohistochemical and genetic similarities to classical hodgkin lymphoma and pmbl. Pdf on nov 14, 2014, maher salamoon published gray zone lymphoma.
Sep 18, 2018 gray zone lymphoma gzl is a very rare and aggressive lymphoma, which is often present as a large mass in the mediastinum. Hairy cell leukemia anaplastic large cell lymphoma, cutan. Mediastinal cases are often referred to as mediastinal grey zone lymphoma mgzl and nonmediastinal cases are grey zone lymphoma gzl. Composite lymphomas pose a particular diagnostic challenge, and there are currently no agreed standards for treatment. Listing a study does not mean it has been evaluated by the u. Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Getting the facts lymphoma research foundation lrf. The clinical outcome in pmbl appears favorable in comparison to typical dlbcl, but this may reflect the tendency of these patients to present with lowerrisk features such as young age and limited stage 4.
The lymphoma guide il page 7 part 1 understanding lymphoma lymphoma is the general term for many different types of blood cancer. Classification of nonhodgkin lymphoma diffuse large bcell lymphoma 72 65 7 burkitt lymphoma 36 32 4 mature bcell lymphoma 27 18 9 precursor bcell lymphoma 15 2 gray zone lymphoma 14 1 precursor tcell lymphoma 36 31 5 mature nktcell lymphoma 65 61 4 tumor stage 0. Though rare, a lymphoma can have features of both classical hodgkin lymphoma and diffuse large bcell lymphoma dlbcl the dlbcl subtype most commonly identified is primary mediastinal bcell lymphoma pmbl. The term gray zone lymphoma has been applied to tumors that demonstrate transitional morphologic and immunophenotypic features between classical hodgkins lymphoma and diffuse large bcell. In cases with diffuse growth pattern, the detection of residual fdc meshwork is important. The lymphatic system is part of your bodys immune system. Traverseglehen a1, pittaluga s, gaulard p, sorbara l, alonso ma, raffeld m, jaffe es. Distinguishing classical hodgkin lymphoma, gray zone lymphoma, and large bcell lymphoma. The two main forms of lymphoma are hodgkin lymphoma and nonhodgkin lymphoma nhl. How i manage patients with grey zone lymphoma athena kritharis, 1monika pilichowska2 and andrew m.
Hodgkin lymphoma i page 1 table of contents 2 introduction 2 here to help 6 hodgkin lymphoma 7 incidence, causes and risk factors 8 signs and symptoms 9 diagnosis 10 subtypes of hodgkin lymphoma 12 staging 15 treatment planning 16 treatment 21 treatment side effects 22 survivorship 24 research and clinical trials 26 normal blood and marrow and the lymphatic system. Three patients whose disease had become refractory to. Gray zone lymphoma gzl with features intermediate between classic hodgkin lymphoma chl and large bcell lymphoma lbcl was introduced as a provisional entity into the world health organization classification in 2008. Gray zone lymphoma with features intermediate between classical hodgkin lymphoma and diffuse large bcell lymphoma. In many cases, the original diagnosis of gray zone lymphoma is later reclassified as a. Shades of gray between large bcell lymphomas and hodgkin. Mediastinal gray zone lymphoma mgzl b cell lymphoma, unclassifiable, features intermediate chl and dlbcl morphology variable microscopic appearance in different areas sheets of tumor cells ranging from centroblastlike to lacunalike or hodgkinlike, in a diffusely fibrotic stroma. It is called gray zone lymphoma because it has features intermediate between classical hodgkin lymphoma and diffuse large bcell lymphoma dlbcl, but cannot be assigned specifically to either type. An external file that holds a picture, illustration, etc. Burkitt lymphoma, castlemans disease, marginal zone lymphoma e. We report our experience with 2 patients diagnosed with grey zone lymphoma gzl. Diffuse large cell lymphoma adult tcell lymphomaleukemia burkitts lymphoma ptld 18.
Gene expression profiling of mediastinal gray zone. Gray zone lymphoma gzl is described as sharing features with classical hodgkin. Nonhodgkin lymphoma and hodgkin lymphoma are the two main types. Gene expression profiling of mediastinal gray zone lymphoma and its relationship to primary mediastinal bcell lymphoma and classical hodgkin lymphoma stefania pittaluga, alina nicolae, george w. Because these lymphomas are treated differently, the optimal therapy for gray zone lymphoma is unclear. The diagnosis of bcell lymphoma, unclassifiable, with features intermediate between diffuse large bcell lymphoma dlbcl and classical hodgkins lymphoma chl, also referred to as grey zone lymphoma gzl, is a challenging diagnosis. Jan 31, 2017 mediastinal grey zone lymphoma from the lysa the safety and scientific validity of this study is the responsibility of the study sponsor and investigators.
Other b cell nhls, particularly small lymphocytic lymphomachronic lymphocytic leukemia and extranodal marginal zone lymphoma, may be associated with small mspikes overview of hodgkin lymphoma in children and adolescents view in chinese. Recently, mediastinal lymphomas with features intermediate between pmbl and nshl, called mediastinal grayzone lymphomas, have been. The term gray zone lymphoma was firstly used in 1998 at the workshop on hodgkins disease and related diseases to designate lymphomas at the border of. Since grey zone lymphoma gzl was originally included in the 2008 world health organization classification as a b. The condition refers to unclassifiable bcell lymphoma with characteristics. Recall the clinical features, treatment, survival outcomes and prognosis of gray zone lymphoma. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for gray zone lymphoma. A 26yearold man presenting with bulky mediastinal mass. Diffuse large bcell lymphoma overview lymphoma is the most common blood cancer. I am a 10 year survivor of iv b gray zone lymphoma.
Follicular lymphoma is a common type of nhl, and occurs almost exclusively in adults. Lymphoma is a cancer of the immune system and effects lymphocytes which are a type of white blood cell. About the authors ilan goldenberg is a senior fellow and director of the middle east security. Jul 26, 2012 the term mediastinal gray zone lymphoma mgzl has been used for these tumors, which are included in the world health organization classification as bcell lymphoma, unclassifiable, with features intermediate between diffuse large bcell lymphoma and classic hodgkin lymphoma. Gray zone lymphoma can be a therapeutic challenge,as it does not respond as expected to standard. A large retrospective multicenter analysis of clinical characteristics, treatment, outcomes and prognosis in the current era.
An exclusive investigation by the grayzone reveals new details on the critical role sheldon adelsons las vegas sands played in an apparent cia spying operation targeting julian assange, and exposes the sands security staff who helped coordinate the malicious campaign. Conclusions mediastinal gray zone lymphoma it appears to be a distinct entity more often localized than pmbcl most patients will require radiotherapy for cure a 52 year old man is sent to you because of a scalp nodule that was found to be a cutaneous diffuse large bcell lymphoma on excisional biopsy. Many questions regarding these grey zone cases is this a unique tumor that has overlapping. Gray zone lymphoma is a disease at the interface of. A the tumor is composed of large pleomorphic cells, some resembling reedsternberg cells and variants occurring in sheets as seen in dlbcl hematoxylin. Gray zone lymphoma, often presenting as large tumors in the mediastinum, is a type of lymphoma that is characterized by having cellular features of both classic.
When the lymphoma has features of both types of lymphoma, it is sometimes referred to as grey zone lymphoma. Despite the rarity of grey zone, being a bcell lymphoma, treatment is fairly well established. Aimthe aim of this paper was to analyze morphological and phenotypic characteristics of three diagnostically difficult cases of. In rare instances, two distinct lymphomas concurrently occur in a patient. Primary mediastinal bcell lymphoma and mediastinal gray zone. A prospective study of mediastinal grayzone lymphoma blood. Nlphl with predominantly diffuse proliferation pattern is morphologically similar to thrbcls, and the differential diagnosis between nlphl and thrbcl can be challenging figure 2a.
Distinguishing classical hodgkin lymphoma, gray zone. Gray zone lymphomas ahmet dogan mayo clinic, rochester, mn, usa the term gray zone lymphoma is mostly used for cases of malignant lymphoma which can not be reliably classified as hodgkin lymphoma or nonhodgkin lymphoma 1 3. Clinicopathologic consensus study of gray zone lymphoma. How i manage patients with grey zone lymphoma kritharis. Is there a way to predict which pmbl cases will relapse as chl or vice versa. We performed gene expression profiling from patients with mgzl 20, chl 18, and pmbl 17 and show mgzl clusters between chl and pmbl. Lymphoma is a cancer that develops in the white blood cells lymphocytes of the lymphatic system. Wright, christopher melani, mark roschewski, seth steinberg, dawei huang, louis m. Bcell lymphoma, unclassifiable, with features intermediate between diffuse large bcell. As your excellent advice notes, pathology is everything in lymphoma. As this differential diagnosis has direct implications for management strate. About marrow, blood and blood cells the information in. A third grey zone also exists between cases of nodular lymphocyte predominant hodgkin lymphoma nlphl and socalled t cell histiocyterich large b cell lymphoma tchrbcl.
Grey zones in the differential diagnosis of lymphoma. The diagnosis and treatment of gray zone lymphoma remains challenging. Countering iran in the gray zone what the united states should learn from israels operations in syria ilan goldenberg, nicholas a. Pd1 blockade in mediastinal grayzone lymphoma nejm. Mediastinal grey zone lymphoma mgzl cancer survivors network. Nodular sclerosis hodgkin lymphoma nshl and primary mediastinal bcell lymphoma pmbl are the common types, whereas mediastinal grayzone. Gray zone lymphoma, often presenting as large tumors in the mediastinum, is a type of lymphoma that is characterized by having cellular features of both classic hodgkins lymphomas chl and large bcell lymphomas. It has also been referred to as mediastinal grey zone lymphoma. Jun 14, 2018 gray zone lymphoma is a rare type of lymphoma, cancer of a part of the immune system called the lymph system. Evens 1division of hematologyoncology, tufts medical center, and 2department of pathology, tufts medical center, boston, ma, usa summary since grey zone lymphoma gzl was originally included in the 2008 world health organization classi. Improving the clinical diagnosis and classification of. Clinically, the initial case descriptions of gzl were primarily with mediastinal presentation.