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      Ground-glass opacities and a solitary nodule on chest in intravascular large B-cell lymphoma

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          Abstract

          A 74-year-old woman presented with dyspnea on exertion and nocturnal cough. Chest computed tomography (CT) revealed scattered bilateral ground-glass opacities without a zonal dominance. Bronchoalveolar lavage elicited increased lymphocytes, but transbronchial lung biopsies were not performed because of hypoxemia during the examination. She received steroid therapy because of her subsequent worsening respiratory condition, but her condition continued to deteriorate. The ground-glass opacities partially consolidated with the appearance of new ground-glass opacities and a nodular shadow. Hepatosplenomegaly was observed on CT while soluble interleukin-2 receptor was elevated. A biopsy of a Campbell de Morgan spot of the trunk yielded a diagnosis of intravascular large B-cell lymphoma. There was marked clearing of the pulmonary infiltrates and significant symptomatic improvement in response to systemic chemotherapy.

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          Most cited references5

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          An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome.

          Diffuse large B-cell lymphoma with haemophagocytic syndrome (BCL-HS) has been reported mainly in Asia and is regarded as a distinct variant of intravascular lymphoma (IVL). However, it is unclear whether all cases of BCL-HS fall within the framework of IVL and available clinical information is limited. We analysed 25 cases with BCL-HS, including 11 autopsied cases (median, 66 years; male-female ratio, 1.1:1). The patients presented with fever, anaemia, thrombocytopenia, hepatosplenomegaly, haemophagocytosis, bone marrow invasion, respiratory disturbance and disseminated intravascular coagulopathy, but usually lacked lymphadenopathy, mass formation, neurological abnormalities and skin lesions. The clinical course was aggressive with a median survival of 7 months. The morphological findings were uniform: large lymphoid cells infiltrated vessels and/or sinusoids of the liver, marrow, lung, kidney and other organs. They were positive for CD19, CD20, CD79a and HLA-DR, but negative for CD10, CD23 and CD30. CD5 was positive in five out of 17 cases. Our critical review indicates that BCL-HS is the equivalent of the Asian variant of IVL.
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            Use of random skin biopsy for diagnosis of intravascular large B-cell lymphoma.

            Intravascular lymphoma (IVL) is a rare type of extranodal lymphoma with an aggressive clinical course characterized by proliferation of large lymphoma cells within the lumina of the small vessels. Because of its varied clinical symptoms and the absence of lymphadenopathy, diagnosis of IVL is extremely difficult and requires histological confirmation. We report here 6 consecutive patients with IVL, admitted to Kameda General Hospital, Kamogawa-shi, Japan, from June 7, 2006, to February 28, 2007, whose IVL was diagnosed by random skin biopsy of healthy-appearing skin. Three patients presented with progressive neurological deterioration and 2 others with hypoxemia with interstitial infiltration on chest radiography. One patient presented with confusion and severe hypoxia without apparent interstitial infiltration. Two patients showed localized skin involvement. Irrespective of the presence of skin lesions, almost all skin biopsy specimens showed obliteration of small vessels of subcutaneous fat tissues by lymphoma cells, allowing a prompt diagnosis of IVL. Early institution of rituximab-based chemotherapy induced favorable responses in all patients treated. Because diagnosis based on tissue other than skin is usually difficult in patients with suspected IVL, random skin biopsy should be considered even in patients with no evident skin lesions.
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              Benefits of skin biopsy of senile hemangioma in intravascular large B-cell lymphoma: A case report and review of the literature

              Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of B-cell lymphoma characterized by selective growth of clonal B-cells in the lumen of the small vessels of various organs including the liver, spleen, lungs, skin, brain, and kidney. An 86-year-old male presented with weight loss, fever and night sweats (known as B symptoms). Blood examination revealed pancytopenia, high lactate dehydrogenase and high soluble interleukin-2 receptor, suggesting hematopoietic malignancy. However, there were no abnormal hematopoietic cells in the peripheral blood. No lymph node swelling was identified on examination by whole-body computed tomography scan. Therefore, IVLBCL was suspected, and random skin biopsies and a skin biopsy from a senile hemangioma were carried out. A small number of large atypical lymphoid cells resided in the small blood vessels in the deep dermis and subcutaneous tissue of the random skin biopsies, and numerous atypical lymphoid cells were identified in the small vessels of the senile hemangioma. These results suggest the usefulness of skin biopsy from senile hemangiomas in the diagnosis of IVLBCL.
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                Author and article information

                Journal
                Respirol Case Rep
                Respirol Case Rep
                rcr2
                Respirology Case Reports
                John Wiley & Sons, Ltd (Chichester, UK )
                2051-3380
                2051-3380
                September 2015
                06 August 2015
                : 3
                : 3
                : 108-111
                Affiliations
                [1 ]Second Department of Internal Medicine, Nara Medical University Kashihara, Japan
                [2 ]Department of Dermatology, Nara Medical University Kashihara, Japan
                [3 ]Department of Diagnostic Pathology, Nara Medical University Kashihara, Japan
                Author notes
                Correspondence, Koichi Tomoda, Second Department of Internal Medicine, Nara Medical University, 840 Shijo-cho, Kashihara City, Nara 634-8522, Japan. E-mail: ktomoda@ 123456naramed-u.ac.jp
                Article
                10.1002/rcr2.116
                4571741
                26392859
                42b5d14c-ca11-4c94-a3a8-9fbc453171f8
                © 2015 The Authors. Respirology Case Reports published by John Wiley & Sons Ltd on behalf of The Asian Pacific Society of Respirology.

                This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

                History
                : 01 May 2015
                : 19 June 2015
                : 03 July 2015
                Categories
                Case Reports

                campbell de morgan spot,chest ct imaging,heterogeneous ground-glass opacities,intravascular large b-cell lymphoma (ivlbcl),solitary nodule

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