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

Abstract

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

Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy

“…Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy [9][10][11] In patients with acute myeloid leukemia (AML), HLH has been occasionally described in case-reports. AML patients may be prone to develop HLH due to their disease-and/or treatment-related impaired immune response and their high susceptibility to severe infections, which act as triggering factors.…”

Intravascular Large B-Cell Lymphoma

“…4 Two forms have been described: classical, also known as Western form, with a cutaneous variant; and an Asian form occurring more frequently in the Far East. 5 Intravascular lymphoma is most frequently a disease of B lymphocytes, although rare cases of thymus-cell (T-cell) and natural killer-cell disease have been reported; 6 the World Health Organization considers these cases to be a separate entity.…”

Intravascular large B-cell lymphoma with prominent cutaneous manifestation — case report

“…Skin manifestations are described by a variety of different lesions including diffuse erythematous streaks, purpuric macules and plaques, livedo-like erythema and telangiectasias like in our case [9]. Patients with isolated "cutaneous variant" are mostly females of younger age with good performance status, normal leukocyte and platelet counts and have a better survival rate than patients with other clinical presentations (3 -yr survival 56%) [4,10]. The most effective treatment seems to be the combination Intravascular large B-cell lymphoma with prominent cutaneous manifestation of chemotherapy (CHOP) with the anti-CD20 antibody rituximab, as all tumour cells express the CD20 antigen [11,12].…”

“…This lymphoma subtype is usually widely disseminated in extranodal sites at presentation (CNS, skin, lungs, kidneys, adrenals). In general this is an aggressive lymphoma with the poor clinical behavior (3 -yr survival less then 22%) [4]. Up to now, predictive factors that are useful for risk stratifi cation of patients have not been established.…”

“…It results in the delay of timely and accurate diagnosis and fatal complications. This lymphoma subtypes involves all types of organs, such as skin, lung, adrenal gland, kidney, liver, spleen, thyroid, gastrointestinal tract, bone marrow and central nervous system (CNS) [2][3][4][5][6][7][8]. The clinical manifestations differ between Asian and European countries.…”