Intravascular large B‐cell lymphoma with bone marrow involvement at presentation and haemophagocytic syndrome: two Western cases in favour of a specific variant

Dufau, Jean-Philippe; Tourneau, A. Le; Molina, Thierry Jo; Houcq, M Le; Claessens, Y E; Rio, Bernard; Delmer, Alain; Diébold, J

doi:10.1046/j.1365-2559.2000.00980.x

Cited by 38 publications

(20 citation statements)

References 20 publications

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“…Among 321 cases of IVL diagnosed in Western countries, and published in English literature, morphologically confirmed HPC has been reported in only five cases (1.5%). [12][13][14][15] However, two of these patients showed HPC features only at relapse and not at initial diagnosis, 14,15 and two others were of Caribbean and Vietnamese origin. 12,16 Comprehensively, the rare cases of HPC-associated IVL diagnosed outside Japan exhibited clinical features similar to those displayed by patients with HPC-associated IVL diagnosed in Japan (J-HPC), i.e., significantly higher rates of advanced disease, fever, hepato-splenic involvement, bone marrow infiltration, fatigue, jaundice, dyspnea, anemia, thrombocytopenia, and altered liver function tests.…”

Section: Discussionmentioning

confidence: 94%

Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions

et al. 2007

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Background and Objectives This study explored variations in the clinical manifestations of intravascular lymphoma (IVL) on the bases of the association with hemophagocytosis and the country where the diagnosis was made. Design and Methods The clinical features of 50 Western patients with IVL were compared with those of 123 patients with IVL diagnosed in Eastern countries (87 diagnosed in Japan and 36 in other Asian countries), previously reported in English literature, and collected by an electronic bibliographic search. Results Hemophagocytosis was absent in Western patients, but reported in 38 (44%) Japanese patients (p=0.00001) and in seven (19%) patients from other Asian countries (p=0.002). No clinical differences were evident between patients with hemophagocytosis-negative IVL diagnosed in Western countries, Japan and other Asian Countries. Conversely, Japanese and non-Japanese patients with hemophagocytosisrelated IVL more frequently had stage IV disease, fever, hepato-splenic involvement, marrow infiltration, dyspnea, anemia, and thrombocytopenia, and rarely exhibited cutaneous or central nervous system involvement. Lymph node and peripheral blood involvement was uncommon in all subgroups. In Western patients, anthracycline-based chemotherapy was associated with a 52% remission rate, and a 2-year overall survival of 46%. Interpretation and Conclusions The clinical features of IVL vary according to the association with hemophagocytosis, regardless of the country in which the diagnosis is made. Western, Japanese and other Asian patients with hemophagocytosis-negative IVL display similar clinical characteristics and should be considered as having classical IVL. Patients with hemophagocytosis-related IVL show significantly different clinical features. Both forms have a poor prognosis. Extensive molecular studies are needed to explore whether these clinical differences might reflect discordant biological entities within IVL

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Section: Discussionmentioning

confidence: 94%

Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions

et al. 2007

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“…Unexplained pyrexia, as in our patient, is one of the many possible clinical presentations [4]. Haematological manifestations include autoimmune haemolytic and other anaemias, thrombocytopenia, pancytopenia, haemophagocytic syndrome and disseminated intravascular coagulation [1,5].…”

Section: Follow-up and Discussionmentioning

confidence: 96%

Case 36: A difficult diagnosis in a patient with fever and progressive multi-organ failure

Gabriel¹,

Chong²,

Rice

et al. 2007

Leukemia & Lymphoma

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“…[12][13][14] These include a purportedly distinct variant of intrasinusoidal splenic LBCL occurring primarily in Asians which is associated with histocytic proliferation, prominent hemophagocytosis, and aggressive behavior. [15][16][17] The nature of these latter cases remains unclear and it has been speculated that some may represent a variant of intravascular LBCL.…”

Section: Discussionmentioning

confidence: 99%

Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases

2005

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Two unusual cases of large B-cell lymphoma with predominant splenic and bone marrow (BM) involvement and similar clinical and histopathologic features are described. Both patients presented with nonspecific constitutional symptoms, unexplained cytopenias, and splenomegaly. Splenectomy revealed diffuse red pulp involvement by large B-cell lymphoma. The perisplenic lymph nodes were also involved diffusely with effacement of normal nodal architecture, excluding a diagnosis of intravascular large B-cell lymphoma. BM biopsies revealed striking erythroid hyperplasia without overt morphologic evidence of involvement by lymphoma. Immunoperoxidase staining of the marrow biopsies with antibodies to CD20 and erythroidassociated antigens revealed involvement by large B-cell lymphoma morphologically resembling the early pronormoblasts. In both cases there was prominent, but not exclusive, intravascular/intrasinusoidal lymphomatous marrow infiltration. These cases represent an unusual variant of large B-cell lymphoma with distinctive patterns of splenic and BM involvement. Furthermore, they underscore the difficulties in identifying intrasinusoidal marrow infiltration by lymphoma in H&E-stained biopsy slides and demonstrate that this pattern of marrow infiltration may be seen in cases of large B-cell lymphoma distinct from the intravascular variant. Keywords: diffuse large B-cell lymphoma; intravascular lymphoma; spleen; bone marrow; erythroid hyperplasia; non-Hodgkin lymphoma Diffuse large B-cell lymphoma (LBCL) can involve nodal or extranodal sites; however, initial presentation in the spleen occurs infrequently. 1 Typically when diffuse LBCL involves the spleen it forms discrete tumoral masses within the splenic parenchyma. 2 A recent report detailed other less common patterns of splenic involvement by LBCL including diffuse infiltration of the splenic red pulp. 3 The clinicopathologic features of diffuse LBCL with these unusual patterns of splenic involvement have yet to be fully elucidated. Described herein are the distinctive clinical and histopathologic features of two cases of splenic LBCL with diffuse red pulp infiltration and concurrent involvement of lymph nodes and bone marrow (BM). These cases had strikingly similar clinical presentations chiefly characterized by dyspnea, unexplained cytopenias, and splenomegaly. In both instances, splenectomy performed to alleviate the cytopenias revealed diffuse, homogeneous involvement of the splenic red pulp by diffuse LBCL. Although the pattern of splenic red pulp involvement and the clinical features raised the possibility of intravascular LBCL, in both cases there was parenchymal involvement of splenic hilar lymph nodes, excluding this diagnosis as defined by the most recent WHO criteria. 4 Furthermore, both cases had prominent BM involvement by lymphoma, which was difficult to detect by routine morphologic evaluation of hematoxylin and eosin (H&E)-stained biopsy slides. This difficulty was due both to the presence of prominent erythroid hyperplasia as well as a predominan...

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Intravascular large B‐cell lymphoma with bone marrow involvement at presentation and haemophagocytic syndrome: two Western cases in favour of a specific variant

Cited by 38 publications

References 20 publications

Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions

Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions

Case 36: A difficult diagnosis in a patient with fever and progressive multi-organ failure

Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases

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