Intravascular large bcell lymphoma ivlbcl is a rare disease entity of nonhodgkin lymphoma according to the current who classification. Intravascular large bcell lymphoma with a fulminant clinical. Intravascular large bcell lymphoma associated with systemic. The condition is usually systemic and can affect the brain. The asian variant of intravascular lymphoma aivl is a rare nonhodgkins lymphoma, characterized by hemophagocytic syndrome and the absence of neurological abnormality or skin lesions, which are. Apr 11, 2011 intravascular large bcell lymphoma bivl is listed as a distinct disease entity in the world health organizationwho classification for lymphoid neoplasms. Intravascular lymphoma ivl, also known as intravascular lymphomatosis, corresponds to a rare variant of extranodal diffuse large b cell lymphoma that affects small and medium sized vessels, and has no specific clinical or laboratory findings.
The precise classification of this disorder or variant is not clear as apparently not all cases of asian diffuse large bcell lymphoma with haemophagocytic syndrome exhibit intravascular features intravascular histiocytosis variant. Intravascular large bcell lymphoma ilbcl, also referred to as angiotropic largecell lymphoma. Intravascular large bcell lymphoma and other intravascular lymphomatoses ivls, including ivl with bcell phenotype and extravascular growth bivl and ivl with tcell phenotype tivl, were compared in a series of cases diagnosed at a single institution and in cases reported in the literature. Ivlbcl usually affects elderly patients with poor performance status, elevated lactic dehydrogenase serum levels, anemia, and b symptoms. Intravascular lymphomatosis ivl is a rare intravascular subtype of extranodal large cell lymphoma causing multifocal vascular occlusion and resulting in diffuse thrombosis, a high incidence of neurological and cutaneous involvement. A case of intravascular large bcell lymphoma of the. A rare subtype of dlbcl characterised by the presence of lymphoma cells only within the lumina of small vessels synonyms. We present here that the syndrome of inappropriate antidiuretic hormone secretion frequently occurs in patients with the asian variant of intravascular. Jan 02, 2012 autopsy identified widespread cd20 positive intravascular large bcell lymphoma with significant hepatosplenic involvement, characteristic of the asian variant ivlbcl. Frequency of intravascular large bcell lymphoma in japan. Pdf asianvariant intravascular lymphoma in the african race. To evaluate cns involvement, particularly recurrence. Asianvariant intravascular large bcell lymphoma ncbi. Intravascular lymphoma radiology reference article.
We report a case of hemophagocytosisrelated asian variant intravascular large bcell lymphoma ivlbcl in a patient of western origin initially diagnosed by splenectomy with diffuse large bcell lymphoma dlbcl with a micronodular pattern. Strange characteristics of ivlbcl, including the absence of marked lymphoadenopathy and the usually aggressive clinical. She met a sufficient number of diagnostic criteria outlined in the hlh2004 trial including. Intravascular large bcell lymphoma ivlbcl is a rare form of diffuse lbcl.
First described as angioendotheliomatosis proliferans systemisata by pfleger and tappeiner 1 in 1959, intravascular angiotropic lymphoma ivl is a rare variant of large cell lymphoma characterized by proliferation of neoplastic lymphoid cells within small blood vessels. It is yet unclear whether the two phenotypic variants of intravascular large cell lymphoma, namely, the more frequent bcell type and the exceedingly rare natural killer nktcell type, represent different phenotypes of one category of lymphoma or completely different entities. Mesa, asian variant intravascular lymphoma in the african race, rare tumors, vol. Intravascular large bcell lymphoma stanford university. The classical and hemophagocytic variants are present mainly in western or asian countries, respectively, although exceptions have been increasingly reported in.
It develops slightly more often in men than in women. It displays some differences in clinical presentation. The classical and hemophagocytic variants are present mainly in western or asian countries, respectively, although exceptions have been. The purpose of this study was to describe the characteristics and out. Asian variant of intravascular large bcell lymphoma. Within the entity of intravascular large bcell lymphoma are the classical, visceral form of the disease also referred to as the western form of intravascular large bcell lymphoma, a cutaneous variant, and an asian variant, each with distinct distribution of disease and different clinical manifestations table 141. Presentation and management of intravascular large bcell. Central nervous system involvement in intravascular large b. The exclusively intravascular localization of this lymphoma is attributed to a deficient property of extravasation, most probably due to a loss of the two adhesion molecules icam1 cd54 and beta1 integrin cd29. Characteristics of intravascular large bcell lymphoma on. Download citation an asian variant of intravascular large b. Aug 28, 2019 murase t, nakamura s, kawauchi k, et al. The disease is rare, and information for its exact frequency among nonhodgkin lymphoma nhl has been extremely limited.
Intravascular lymphoma definition of intravascular lymphoma. Hemophagocytosisrelated asian variant intravascular. Intravascular large cell lymphomas plastic surgery key. The cutaneous variant of intravascular lymphoma is limited to the skin 1.
A type of diffuse large b cell lymphoma with intravascular growth in skin, cns, other sites. This case uniquely highlights development of the asian variant ivlbvl in a previously undescribed race. Objective the asian variant of intravascular lymphoma aivl is a rare nonhodgkins lymphoma, characterized by hemophagocytic syndrome and the absence of neurological abnormality or skin lesions, which are typical features of classical ivl. An asian variant of intravascular large bcell lymphoma.
The clonal relationship between these two dlbcl subtypes is also investigated. As a diagnosis of intravascular lymphoma can be difficult, it is often only diagnosed on autopsy. We report a case of hemophagocytosisrelated asian variant intravascular large bcell lymphoma ivlbcl in a patient of. Retrospective analysis of intravascular large bcell lymphoma. In western countries, a cutaneous variant of ivlbcl, which only involves single or multiple lesions of the skin without systemic involvement, was recently proposed. We report a case of intravascular bcell lymphoma in a caucasian patient associated with secondary hemophagocytic syndrome. Intravascular large bcell lymphoma immunohistochemistry. Asianvariant intravascular lymphoma in the african race. Retrospective study of intravascular large bcell lymphoma c.
Intravascular nkcell lymphoma ivnkl is an extremely rare variant of nonhodgkin lymphoma. This rare form of bcell lymphoma is characterised by selective growth of tumour cells in the lumina of small vessels of various organs. Hs has been reported mainly in asia and is regarded as a distinct. Its previous name, intravascular large bcell lymphoma, asian variant, was recently changed to its current name by the world health. Presentation and management of intravascular large bcell lymphoma kazuyuki shimada, tomohiro kinoshita, tomoki naoe, shigeo nakamura intravascular large bcell lymphoma ivlbcl is a rare disease entity of nonhodgkin lymphoma according to the current who classi. Silent intravascular lymphoma initially manifesting as a. Intravascular large bcell lymphoma canadian cancer society. Ivl is a rare subtype of extranodal diffuse large bcell lymphoma. Rajan jain, shehanaz ellika, in handbook of neurooncology neuroimaging, 2008. Intravascular large bcell lymphoma ivlbcl is a rare subtype of extranodal diffuse large bcell lymphoma dlbcl, as classified by who. Intravascular large bcell lymphoma how is intravascular. Intravascular lymphoma is characterized by nearly exclusive intravascular neoplastic proliferation of b lymphocytes, usually without involvement of lymphoid tissues and peripheral blood. The final diagnosis was intravascular large bcell lymphoma, presented as mononeuritis multiplex. There are limited data about the influence of the central nervous system cns involvement on the prognosis for patients with the asian variant of intravascular large bcell.
The hemophagocytic syndromeassociated variant of ivbcl is a very rare variant of ivbcl. Episodic fevers and worsening anemia are thought to be related to episodes of hemophagocytic lymphohistiocytosis hlh triggered by her underlying lymphoma. Intravascular large bcell lymphoma ilcl may also be called intravascular angiotropic lymphoma. The skin changes and neurological symptoms are more frequent in the conventional type of intravascular large bcell lymphoma but are not rare in the asian variant.
A variant may be primary large cell lymphoma of the splenic sinuses. Definition, diagnosis, and management of intravascular large. Intravascular lymphoma usually shows rapid progression and short survival, with at best. Intravascular large bcell lymphoma, hemophagocytic syndromeassociated variant presentation.
The asian variant of intravascular large b cell lymphoma is a special type of intravascular lymphoma with hemophagocytic syndrome and hypercytokinemia including interleukin6, which stimulates antidiuretic hormone synthesis in the hypothalamus. Intravascular large bcell lymphoma with hemophagocytic. Intravascular large bcell lymphoma ivl is an extremely rare malignancy, mainly studied through european and asian series. Oct 16, 2018 intravascular large bcell lymphoma ilbcl is a rare, bcell nonhodgkins lymphoma.
Pdf asian variant of intravascular large bcell lymphoma. Intravascular large bcell lymphoma american society of hematology. Autopsy identified widespread cd20 positive intravascular large bcell lymphoma with significant hepatosplenic involvement, characteristic of the asian variant ivlbcl. Asian variant of intravascular large b cell lymphoma causes. Dec 12, 2009 intravascular large bcell lymphoma ivlbcl is a rare subtype of nonhodgkin lymphoma characterized by the presence of lymphoma cells only in the lumina vessels.
Intravascular lymphoma is also known as intravascular large bcell lymphoma, intravascular lymphomatosis, angiotropic large cell lymphoma, and malignant angioendotheliomatosis. Intravascular large bcell lymphoma cancer therapy advisor. Intravascular large bcell lymphoma is an important cause of fever of unknown origin and multiple organ failure. Intravascular lymphoma an overview sciencedirect topics. Intravascular large bcell lymphoma as mononeuritis multiplex.
Hemophagocytosisrelated asian variant intravascular large b. Definition, diagnosis, and management of intravascular large b. Pathology of intravascular large bcell lymphoma dr. The patient was diagnosed by core liver biopsy and successfully. Intravascular large bcell lymphoma ivlbcl is a rare form of an extranodal malignant nonhodgkin lymphoma nhl that. Since 2001, the intravascular large bcell lymphoma ivlbcl has been recognized as an entity in the who classification. Malignancyassociated hlh represents up to onefourth of.
Bone marrowliverspleen type of large bcell lymphoma associated with hemophagocytic syndrome. We report a case of hemophagocytosisrelated asian variant intravascular large bcell lymphoma ivlbcl in a patient of western origin initially diagnosed by splenectomy with diffuse. Intravascular lymphomas ivl are rare cancers in which malignant lymphocytes proliferate and. Ilcl usually occurs in middleaged or elderly people. Its previous name, intravascular large bcell lymphoma, asian variant, was recently changed to its current name by the world health organization, 2016. Due to the low incidence of this condition, our understanding of the clinical presentation as well as the management of ivl relies on a limited number of patients. Sep 21, 2016 intravascular large bcell lymphoma ivlbcl is a rare form of diffuse lbcl characterized by preferential intravascular growth of malignant lymphocytes, aggressive behavior, and an often fatal course. Intravascular lymphoma has a strong association with hemophagocytic syndrome in asian patients, the socalled asian variant, but not in western patients. Pathology outlines intravascular large b cell lymphoma. Intravascular large bcell lymphoma ivlbcl with hemophagocytic lymphohistiocytosis hlh is a rare entity that is typically seen in the asian variant of the condition. It is a very rare, fastgrowing aggressive subtype of diffuse large bcell lymphoma dlbcl.
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