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Le lymphome intravasculaire est une forme rare de lymphome non hodgkinien à grandes cellules B, de présentation clinique hétérogène et de diagnostic histologique difficile. Dans ce contexte, l'intérêt diagnostique de la tomographie par émission de positons a déjà été rapporté. Nous rapportons le cas d'une patiente âgée de 45 ans, hospitalisée pour bilan diagnostique d'une hyperthermie avec syndrome inflammatoire biologique et syndrome d'activation macrophagique sans étiologie retrouvée en première intention. La description en tomographie par émission de positons d'une hyperfixation corticale rénale ayant d'abord poussé au diagnostic de pyélonéphrite a finalement mené à la réalisation d'une ponction biopsie rénale permettant le diagnostic de lymphome intravasculaire. L'atteinte rénale est rare dans le lymphome intravasculaire et se présente souvent comme une insuffisance rénale protéinurique. L'hyperfixation rénale globale en tomographie par émission de positons dans le contexte de lymphome intravasculaire a déjà été décrite ; néanmoins, la fixation corticale prédominante est plus rare et n'a jamais été rapportée avec une histologie associée. Cet aspect doit donc, dans le contexte d'une hyperthermie prolongée non expliquée, faire évoquer le diagnostic de lymphome intravasculaire et conduire à la réalisation d'une ponction biopsie rénale afin d'en obtenir la preuve histologique. Intravascular large B cell lymphoma is a rare non-Hodgkin large B cell lymphoma disease, with heterogeneous clinical manifestation and difficult pathological diagnosis. Positron emission tomography may be helpfull in this context and has already been reported. A 45-year-old woman was admitted for persistent high fever, inflammatory syndrome and unexplained haemophagocytic syndrome. Bilateral cortical renal hypermetabolism at positron emission tomography initially misled to pyelonephritis diagnosis and secondarily led to kidney biopsy, which showed intravascular large B cell lymphoma. Renal involvement in intravascular large B cell lymphoma is rare and is usually characterized by acute renal failure and proteinuria. Global hypermetabolism at positron emission tomography has already been described in this context, but cortical hypermetabolism has never been associated with pathological findings. In front of persistent high fever without etiology, this positron emission tomography feature must lead to intravascular large B cell lymphoma suspicion and to kidney biopsy to obtain pathological proof. [ABSTRACT FROM AUTHOR]

T cell prolymphocytic leukemia (T-PLL) is a rare, aggressive neoplasm derived from post-thymic T cells. Patients are typically middle-aged with a slight male predominance who present with a high white blood cell count, hepatosplenomegaly, lymphadenopathy, and other symptoms typically associated with leukemia. Although cutaneous involvement has been reported in up to 30% of cases of T-PLL, to our knowledge, none have presented with a presentation resembling livedoid vasculopathy. In the correct clinical context, an underlying hematolymphoid neoplasm should be included in the differential diagnosis of a patient presenting with livedoid vasculopathy., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Résumé: Introduction: Le lymphome à grandes cellules B intravasculaire est une prolifération de cellules tumorales aboutissant à l’occlusion de la lumière des petits vaisseaux. Il s’agit d’une hémopathie maligne rare et de diagnostic difficile en raison d’une présentation clinique polymorphe. Observation: Nous rapportons ici l’observation d’un patient atteint d’un lymphome intravasculaire révélé par un syndrome d’activation macrophagique. Cette association est très souvent marquée par une plus grande sévérité clinique et impose une prise en charge thérapeutique précoce et adaptée. Conclusion: En raison du caractère polymorphe et de la présentation systémique du lymphome à grandes cellules B intravasculaire, l’interniste peut être confronté à cette pathologie, plus grave encore lorsqu’elle s’associe à un syndrome d’activation macrophagique. Il faut donc savoir l’évoquer d’autant que le pronostic dépend de la rapidité de la prise en charge avec notamment la mise en route précoce d’une polychimiothérapie associée au rituximab. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To present a comprehensive description of the clinical features of patients with renal manifestations during lymphoma. Methods: Retrospective review of medical records from all patients diagnosed with lymphoma associated with kidney involvement in our hospital between 1996 to 2004. Four cases were identified and analysed. Results: Four patients presented a non-Hodgkin''s lymphoma. One patient showed intravascular large B-cell lymphoma, revealed by proteinuria. Another patient had a nephrotic syndrome, and two had a renal mass. Renal histology allowed diagnosis of lymphoma in 3 cases. Conclusion: The diagnosis of lymphoma associated with renal involvement is rather difficult, and more specifically in case of intravascular large B-cell lymphoma, or even primary renal lymphoma. We present here a comprehensive review of the literature and we discuss pathogenesis of these conditions. [Copyright &y& Elsevier]

Intravascular large B cell lymphoma is a rare non-Hodgkin large B cell lymphoma disease, with heterogeneous clinical manifestation and difficult pathological diagnosis. Positron emission tomography may be helpfull in this context and has already been reported. A 45-year-old woman was admitted for persistent high fever, inflammatory syndrome and unexplained haemophagocytic syndrome. Bilateral cortical renal hypermetabolism at positron emission tomography initially misled to pyelonephritis diagnosis and secondarily led to kidney biopsy, which showed intravascular large B cell lymphoma. Renal involvement in intravascular large B cell lymphoma is rare and is usually characterized by acute renal failure and proteinuria. Global hypermetabolism at positron emission tomography has already been described in this context, but cortical hypermetabolism has never been associated with pathological findings. In front of persistent high fever without etiology, this positron emission tomography feature must lead to intravascular large B cell lymphoma suspicion and to kidney biopsy to obtain pathological proof., (Copyright © 2020 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.)

Background: Intravascular large B-cell lymphoma (ivLBCL) is a rare blood dyscrasia that is difficult to diagnose. Healthy skin biopsies may prove useful in diagnosis of the condition. Herein we report a case of ivLBCL diagnosed using this type of examination, and we provide a literature review to determine the sensitivity of such testing., Patients and Methods: A 67-year-old woman was hospitalised for unexplained prolonged fever (UPF) and impaired general well-being. Laboratory tests revealed inflammatory syndrome, elevated LDH>2000IU/L, hepatic cytolysis and decreased prothrombin time at 47 %. Analysis for infection and medical imaging ruled out both an infectious or inflammatory origin and solid tumour. A healthy skin biopsy enabled confirmation of the diagnosis of ivLBCL., Discussion: This clinical case illustrates the value of healthy skin biopsy in establishing a diagnosis of ivLBCL in patients hospitalised for UPF. Following a systematic literature review in PubMed/Medline, we included eight studies involving at least three patients designed to assess the value of healthy skin biopsy in the diagnosis of ivLBCL. The diagnostic sensitivity of this approach ranged from 67% to 100%, with a sensitivity of 100% being seen in four of the eight studies. Details of the biopsy sites were available in three studies and diagnostic sensitivity was similar overall between samples taken from the thigh, abdomen and arms., Conclusion: Healthy skin biopsy sampling from at least two sites constitutes a sensitive and relatively non-invasive procedure for early diagnosis of ivLBCL., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Intravascular lymphoma is a rare form of non-Hodgkin's lymphoma with varied but highly evocative clinical presentations, particularly in terms of dermatology. Histological examination of the skin may be sufficient to allow diagnosis., Patients and Methods: Herein, we report the case of a 75-year-old woman with a history of infiltrative ductal carcinoma of the left breast who was hospitalised for neurological evaluation following repeated falls. During the course of her hospital stay, the patient's neurological state deteriorated rapidly, and onset of marked laboratory-documented inflammatory syndrome was observed, together with disseminated intravascular coagulation (DIC). At the same time, she developed orange-peel type skin lesions on her left breast followed by a rapidly-spreading livedoid infiltrated plaque on her side. The skin biopsy enabled a diagnosis to be made of intravascular lymphoma based on immunohistochemical demonstration of intravascular tumoral proliferation of lymphoid cells expressing CD45 marker and B-cell marker CD20, as well as Bcl2, MUM 1 and CD5., Discussion: Intravascular B-cell lymphoma is a rare subtype of diffuse large B-cell lymphoma. It carries a poor prognosis and the clinical appearance varies extremely widely. The signs are primarily neurological and dermatological. Because of the varied nature of presentations as well as the rarity of the disease, diagnosis may be delayed, with worsening of the prognosis of the disease, which nevertheless requires rapid and aggressive management. However, the appearance of infiltrated and livedoid lesions on the trunk or limbs is evocative and skin biopsy constitutes a readily accessible diagnostic tool that offers rapid confirmation., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: Intravascular large B cell lymphoma is a neoplastic cell proliferation leading to the occlusion of the lumen of small vessels. This is a rare haematological malignancy, which is difficult to diagnose because of a heterogeneous clinical presentation., Case Report: We report a 62-year-old man who presented a macrophage activation syndrome as the presenting manifestation of an intravascular lymphoma. This association is frequently marked by a greater severity and clinical care requires an early and appropriate treatment., Conclusion: Due to the polymorphism and the systemic presentation of intravascular large B cell lymphoma, the internist may be confronted with this disease, which is considered to be more severe if associated with a macrophage activation syndrome. Awareness of the intravascular large B cell lymphoma is important because the prognosis depends on the rapidity of the initiation of chemotherapy associated with rituximab., (Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)