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      Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases

      research-article
      1 , 1 , 2 , 3 , 4 , 5 , 4 , 5 , 1 , 6
      Endocrinology, Diabetes & Metabolism Case Reports
      Bioscientifica Ltd
      Geriatric, Male, White, Norway, Adrenal, Adrenal, ACTH, Cortisol, Adrenal insufficiency, Adrenal lymphoma*, Dizziness, Anorexia, Nausea, Arthralgia, Weight loss, Renal failure, Fatigue, Hyponatraemia, Vomiting, Pyrexia, Tachycardia, Hypotension, Abdominal discomfort, Bowel movements - bleeding, Adrenal antibodies, CT scan, Biopsy, ACTH, Creatinine (serum), Potassium, Sodium, Cortisol, ACTH stimulation, Urine osmolality, MRI, Radiotherapy, Fluid repletion, Glucocorticoids, Sodium chloride, Fludrocortisone, Mineralocorticoids, Cortisone acetate, Doxorubicin, Prednisolone, Vincristine*, Cyclophosphamide*, Hydrocortisone, Rituximab, Oncology, Radiology/Rheumatology, Urology, Unique/unexpected symptoms or presentations of a disease, March, 2020

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          Abstract

          Summary

          Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids.

          Learning points:
          • Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency.

          • Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically.

          • Steroid treatment before biopsy may affect diagnosis.

          • Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.

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          Most cited references4

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          Clinical, immunological, and genetic features of autoimmune primary adrenal insufficiency: observations from a Norwegian registry.

          Primary adrenal insufficiency [Addison's disease (AD)] is rare, and systematic studies are few, mostly conducted on small patient samples. We aimed to determine the clinical, immunological, and genetic features of a national registry-based cohort. Patients with AD identified through a nationwide search of diagnosis registries were invited to participate in a survey of clinical features, health-related quality of life (HRQoL), autoantibody assays, and human leukocyte antigen (HLA) class II typing. Of 664 registered patients, 64% participated in the study. The prevalence of autoimmune or idiopathic AD in Norway was 144 per million, and the incidence was 0.44 per 100,000 per year (1993-2007). Familial disease was reported by 10% and autoimmune comorbidity by 66%. Thyroid disease was most common (47%), followed by type 1 diabetes (12%), vitiligo (11%), vitamin B12 deficiency (10%), and premature ovarian insufficiency (6.6% of women). The mean daily treatment for AD was 40.5 mg cortisone acetate and 0.1 mg fludrocortisone. The mean Short Form 36 vitality scores were significantly diminished from the norm (51 vs. 60), especially among those with diabetes. Concomitant thyroid autoimmunity did not lower scores. Anti-21-hydroxylase antibodies were found in 86%. Particularly strong susceptibility for AD was found for the DR3-DQ2/ DRB1*0404-DQ8 genotype (odds ratio, 32; P = 4 x 10(-17)), which predicted early onset. AD is almost exclusively autoimmune, with high autoimmune comorbidity. Both anti-21-hydroxylase antibodies and HLA class II can be clinically relevant predictors of AD. HRQoL is reduced, especially among diabetes patients, whereas thyroid disease did not have an impact on HRQoL. Treatment modalities that improve HRQoL are needed.
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            Adrenal insufficiency due to bilateral adrenal metastases – A systematic review and meta-analysis

            Objective Bilateral adrenal metastases may cause adrenal insufficiency (AI) but it is unclear if screening for AI in patients with bilateral adrenal metastases is justified, despite the potential for adrenal crises. Method A search using PubMed/Medline, ScienceDirect and Cochrane Reviews was performed to collect all original research articles and all case reports from the past 50 years that describe AI in bilateral adrenal metastases. Results Twenty studies were included with 6 original research articles, 13 case reports and one case series. The quality was generally poor. The prevalence of AI was 3–8%. Of all cases of AI (n = 25) the mean pooled baseline cortisol was 318 ± 237 nmol/L and stimulated 423 ± 238 nmol/L. Hypotension was present in 69%, hyponatremia in 9% and hyperkalemia in 100%. Lung cancer was the cause in 35%, colorectal 20%, breast cancer 15% and lymphoma 10%. The size of the adrenal metastases was 5.5 ± 2.8 cm (left) and 5.5 ± 3.1 cm (right), respectively. There was no correlation between basal cortisol, stimulated cortisol concentration or ACTH with the size of adrenal metastases. The median time to death was 5.0 months (IQR 0.6–6.5). However, two cases were alive after 12–24 months. Conclusion The prevalence of AI in patients with bilateral adrenal metastases was low. Prognosis was very poor. Due to the low prevalence of AI, screening is likely only indicated in patients with symptoms and signs suggestive of hypocortisolism.
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              Clues for early detection of autoimmune Addison's disease - myths and realities

              Early detection of autoimmune Addison's disease (AAD) is important as delay in diagnosis may result in a life-threatening adrenal crisis and death. The classical clinical picture of untreated AAD is well-described, but methodical investigations are scarce.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                10 March 2020
                2020
                : 2020
                : 19-0131
                Affiliations
                [1 ]Department of Endocrinology , Akershus University Hospital, Lorenskog, Norway
                [2 ]Department of Haematology , Akershus University Hospital, Lorenskog, Norway
                [3 ]Department of Radiology , Akershus University Hospital, Lorenskog, Norway
                [4 ]Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders , University of Bergen, Bergen, Norway
                [5 ]Department of Medicine , Haukeland University Hospital, Bergen, Norway
                [6 ]Institute of Clinical Medicine , Faculty of Medicine, University of Oslo, Oslo, Norway
                Author notes
                Correspondence should be addressed to K Grønning; Email: kaja.gronning@ 123456gmail.com
                Article
                EDM190131
                10.1530/EDM-19-0131
                7077515
                32163909
                8b5bc31a-7df9-4344-96ad-aca9f3dc17b6
                © 2020 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License..

                History
                : 17 December 2019
                : 14 February 2020
                Categories
                Geriatric
                Male
                White
                Norway
                Adrenal
                Adrenal
                ACTH
                Cortisol
                Adrenal Insufficiency
                Adrenal Lymphoma*
                Dizziness
                Anorexia
                Nausea
                Arthralgia
                Weight loss
                Renal failure
                Fatigue
                Hyponatraemia
                Vomiting
                Pyrexia
                Tachycardia
                Hypotension
                Abdominal discomfort
                Bowel movements - bleeding
                Adrenal antibodies
                CT scan
                Biopsy
                ACTH
                Creatinine (serum)
                Potassium
                Sodium
                Cortisol
                ACTH stimulation
                Urine osmolality
                MRI
                Radiotherapy
                Fluid repletion
                Glucocorticoids
                Sodium chloride
                Fludrocortisone
                Mineralocorticoids
                Cortisone acetate
                Doxorubicin
                Prednisolone
                Vincristine*
                Cyclophosphamide*
                Hydrocortisone
                Rituximab
                Oncology
                Radiology/Rheumatology
                Urology
                Unique/Unexpected Symptoms or Presentations of a Disease
                Unique/Unexpected Symptoms or Presentations of a Disease

                geriatric,male,white,norway,adrenal,acth,cortisol,adrenal insufficiency,adrenal lymphoma*,dizziness,anorexia,nausea,arthralgia,weight loss,renal failure,fatigue,hyponatraemia,vomiting,pyrexia,tachycardia,hypotension,abdominal discomfort,bowel movements - bleeding,adrenal antibodies,ct scan,biopsy,creatinine (serum),potassium,sodium,acth stimulation,urine osmolality,mri,radiotherapy,fluid repletion,glucocorticoids,sodium chloride,fludrocortisone,mineralocorticoids,cortisone acetate,doxorubicin,prednisolone,vincristine*,cyclophosphamide*,hydrocortisone,rituximab,oncology,radiology/rheumatology,urology,unique/unexpected symptoms or presentations of a disease,march,2020

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