Lymphoma in acquired generalized lipodystrophy

Brown, Rebecca; Chan, Jean L.; Jaffe, Elaine S.; Cochran, Elaine; DePaoli, Alex M.; Gautier, Jean‐François; Goujard, Cécile; Vigouroux, Corinne; Görden, Phillip

doi:10.3109/10428194.2015.1040015

Cited by 36 publications

(28 citation statements)

References 28 publications

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“…Lymphomas, particularly peripheral T-cell lymphoma, occur in AGL, with a prevalence of approximately 7% (4, 35). Appropriate screening has not been established but would reasonably include annual skin and lymph node examination.…”

Section: Screening For Comorbiditiesmentioning

confidence: 99%

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The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline

Brown

Araújo‐Vilar

Cheung

et al. 2016

The Journal of Clinical Endocrinology & Metabolism

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394

497

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Objective:Lipodystrophy syndromes are extremely rare disorders of deficient body fat associated with potentially serious metabolic complications, including diabetes, hypertriglyceridemia, and steatohepatitis. Due to their rarity, most clinicians are not familiar with their diagnosis and management. This practice guideline summarizes the diagnosis and management of lipodystrophy syndromes not associated with HIV or injectable drugs.Participants:Seventeen participants were nominated by worldwide endocrine societies or selected by the committee as content experts. Funding was via an unrestricted educational grant from Astra Zeneca to the Pediatric Endocrine Society. Meetings were not open to the general public.Evidence:A literature review was conducted by the committee. Recommendations of the committee were graded using the system of the American Heart Association. Expert opinion was used when published data were unavailable or scarce.Consensus Process:The guideline was drafted by committee members and reviewed, revised, and approved by the entire committee during group meetings. Contributing societies reviewed the document and provided approval.Conclusions:Lipodystrophy syndromes are heterogeneous and are diagnosed by clinical phenotype, supplemented by genetic testing in certain forms. Patients with most lipodystrophy syndromes should be screened for diabetes, dyslipidemia, and liver, kidney, and heart disease annually. Diet is essential for the management of metabolic complications of lipodystrophy. Metreleptin therapy is effective for metabolic complications in hypoleptinemic patients with generalized lipodystrophy and selected patients with partial lipodystrophy. Other treatments not specific for lipodystrophy may be helpful as well (eg, metformin for diabetes, and statins or fibrates for hyperlipidemia). Oral estrogens are contraindicated.

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Section: Screening For Comorbiditiesmentioning

confidence: 99%

“…Additional serious adverse events occurring during metreleptin treatment are likely related to the underlying lipodystrophy syndrome, rather than metreleptin. These include T-cell lymphoma in patients with AGL (35), pancreatitis (47), and worsening of liver (47) and kidney (34) disease.…”

Section: Treatment Of Lipodystrophy Syndromesmentioning

confidence: 99%

The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline

Brown

Araújo‐Vilar

Cheung

et al. 2016

The Journal of Clinical Endocrinology & Metabolism

Self Cite

394

497

View full text Add to dashboard Cite

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“…Because of the loss of adipose tissue, levels of the adipocyte-secreted hormone leptin may be low and leptin replacement could be a therapeutic option in our patient, in an attempt to overcome morphological and metabolic abnormalities. However lymphoma after leptin therapy for patients with acquired lipodystrophy has been described [28] . In addition, no single drug or associations of drugs has been effective in the treatment of calcinosis in JDM [29] .…”

Section: Discussionmentioning

confidence: 99%

Juvenile dermatomyositis forty years on: Case report

Figueiredo

Castro

Bernardino

et al. 2018

Neuromuscular Disorders

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We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.

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“…However, antibodies with in vivo neutralizing activity have only been detected in a small number of patients (96). T-cell lymphoma has been reported in a few patients with AGL treated with metreleptin (97,98). Immune dysfunction is a feature of the natural history in patients with AGL (42,44).…”

Section: Metreleptin Therapymentioning

confidence: 99%

Current Diagnosis, Treatment and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Young People

Özen

Akıncı

Oral

2020

Jcrpe

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Lipodystrophy is a heterogeneous group of disorders characterized by lack of body fat in characteristic patterns, which can be genetic or acquired. Lipodystrophy is associated with insulin resistance that can develop in childhood and adolescence, and usually leads to severe metabolic complications. Diabetes mellitus, hypertriglyceridemia, and hepatic steatosis ordinarily develop in these patients, and most girls suffer from menstrual abnormalities. Severe complications develop at a relatively young age, which include episodes of acute pancreatitis, renal failure, cirrhosis, and complex cardiovascular diseases, and all of these are associated with serious morbidity. Treatment of lipodystrophy consists of medical nutritional therapy, exercise, and the use of anti-hyperglycemic and lipid-lowering agents. New treatment modalities, such as metreleptin replacement, promise much in the treatment of metabolic abnormalities secondary to lipodystrophy. Current challenges in the management of lipodystrophy in children and adolescents include, but are not limited to: (1) establishing specialized centers with experience in providing care for lipodystrophy presenting in childhood and adolescence; (2) optimizing algorithms that can provide some guidance for the use of standard and novel therapies to ensure adequate metabolic control and to prevent complications; (3) educating patients and their parents about lipodystrophy management; (4) improving patient adherence to chronic therapies; (5) reducing barriers to access to novel treatments; and (5) improving the quality of life of these patients and their families.

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Lymphoma in acquired generalized lipodystrophy

Cited by 36 publications

References 28 publications

The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline

The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline

Juvenile dermatomyositis forty years on: Case report

Current Diagnosis, Treatment and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Young People

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