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      AA amyloidosis associated with Castleman disease : A case report and review of the literature


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          AA amyloidosis (AA) is caused by a wide variety of inflammatory states, but is infrequently associated with Castleman disease (CD). CD describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. CD can present with a solitary enlarged lymph node (unicentric CD, UCD) or with multicentric lymphadenopathy (MCD), constitutional symptoms, cytopenias, and multiple organ dysfunction due to an interleukin-6 driven cytokine storm.

          Patient Concerns:

          We are reporting a case of a 26-year-old woman with no significant past medical history who presented with a 3-month history of fatigue and an unintentional 20-pound weight loss.


          A CT-scan of the abdomen and pelvis revealed hepatosplenomegaly and a mesenteric mass. Congo Red staining from a liver biopsy showed apple-green birefringence and serum markers were suggestive of an inflammatory process. Post-excision examination of the resected mass revealed a reactive lymph node with follicular hyperplasia with kappa and lambda stains showing polyclonal plasmacytosis consistent with CD.


          The patient underwent surgery to remove the affected lymph node.


          IL-6, anemia, leukocytosis, and thrombocytosis resolved or normalized 2 weeks after resection; creatinine normalized 9 months postsurgery. Twenty two months post-surgery her IFN-γ normalized, her fatigue resolved, her proteinuria was reduced by >90% and she had returned to her baseline weight.


          Our case and literature review suggest that patients presenting with UCD or MCD along with organ failure should prompt consideration of concurrent AA amyloidosis.

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          Most cited references64

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          Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations.

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            HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy.

            Multicentric Castleman's disease (MCD) describes a heterogeneous group of disorders involving proliferation of morphologically benign lymphocytes due to excessive proinflammatory hypercytokinemia, most notably of interleukin-6. Patients demonstrate intense episodes of systemic inflammatory symptoms, polyclonal lymphocyte and plasma cell proliferation, autoimmune manifestations, and organ system impairment. Human herpes virus-8 (HHV-8) drives the hypercytokinemia in all HIV-positive patients and some HIV-negative patients. There is also a group of HIV-negative and HHV-8-negative patients with unknown etiology and pathophysiology, which we propose referring to as idiopathic MCD (iMCD). Here, we synthesize what is known about iMCD pathogenesis, present a new subclassification system, and propose a model of iMCD pathogenesis. MCD should be subdivided into HHV-8-associated MCD and HHV-8-negative MCD or iMCD. The lymphocyte proliferation, histopathology, and systemic features in iMCD are secondary to hypercytokinemia, which can occur with several other diseases. We propose that 1 or more of the following 3 candidate processes may drive iMCD hypercytokinemia: systemic inflammatory disease mechanisms via autoantibodies or inflammatory gene mutations, paraneoplastic syndrome mechanisms via ectopic cytokine secretion, and/or a non-HHV-8 virus. Urgent priorities include elucidating the process driving iMCD hypercytokinemia, identifying the hypercytokine-secreting cell, developing consensus criteria for diagnosis, and building a patient registry to track cases.
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              Surgery in Castleman's disease: a systematic review of 404 published cases.

              We undertook a systematic review of 404 published cases of Castleman's disease to identify the role of the surgeon beyond assistance in tissue-based diagnosis. Castleman's disease is a rare primary disease of the lymph node caused by infection with herpesviridae. Little is known about the role of surgery in this condition. Medline, Embase, Cochrane Database of Systematic Reviews, ISI Thompson Web of Knowledge, and hand search of articles' bibliography. Of the 1791 citations identified through the initial electronic search and screened for possible inclusion, 488 articles were retained after title and abstract reviews. Of these, 239 were accepted for this review. A complete dataset containing age, gender, centricity (unicentric vs multicentric), histopathologic type (hyaline vascular [HV] vs plasma cell [PC]), anatomical location of the only focus in unicentric Castleman's disease (UCD) or the dominant focus in multicentric Castleman's disease (MCD), nature of the surgical approach (resective vs diagnostic), and outcome (disease-free survival [DFS] vs death due to disease) was extracted. A resective or debulking surgical approach was described in 77.0% of all patients, but was far more common in unicentric (262/278; 94.2%) than multicentric (49/126; 38.9%) disease (χ² 146.8; P < 0.0001). Unicentric disease had a significantly higher overall survival (95.3% vs 61.1%; χ² 55.7; P < 0.0001), 3 year DFS (89.7% vs 55.6%; χ² 27.8; P < 0.0001), and 5 year DFS (81.2% vs 34.4%; χ² 28.6; P < 0.0001) than multicentric disease. Failure to treat unicentric disease by resective surgery resulted in a significant mortality (17.6% vs 3.8% χ²; P < 0.05). In multicentric disease, outcomes are comparable between debulking surgery alone, immunochemotherapy alone, or a combination of both (28.0% vs 28.9% vs 50.0%; P = nonsignificant). Surgery is the gold standard for treatment of unicentric Castelman's disease. The role of debulking surgery in human immunodeficiency virus (-) MCD needs to be evaluated in prospective studies.

                Author and article information

                Medicine (Baltimore)
                Medicine (Baltimore)
                Wolters Kluwer Health
                February 2020
                07 February 2020
                : 99
                : 6
                : e18978
                [a ]Penn Amyloidosis Program, Abramson Cancer Center
                [b ]Department of Pathology and Laboratory Medicine
                [c ]Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.
                Author notes
                []Correspondence: Luca Bernabei, Abramson Cancer Center, University of Pennsylvania, 3400 Civic Center Blvd, Philadelphia 19107, PA (e-mail: Luca.Bernabei@ 123456uphs.upenn.edu ).
                MD-D-19-03795 18978
                Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0

                : 16 May 2019
                : 27 November 2019
                : 30 December 2019
                Research Article
                Clinical Case Report
                Custom metadata

                aa amyloidosis,castleman disease,lymph node hyperplasia,unicentric castleman


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