Abstract

Although lymphoma may occasionally involve the adrenal glands as part of a generalized disease process, primary adrenal lymphoma (PAL) is a rare disease. We present a case of a 62-year-old woman with a history of mild/moderate hereditary spherocytosis with a well-compensated baseline haemoglobin, who presented with rapidly progressive symptomatic anaemia. During the diagnostic workup, imaging revealed bilateral large adrenal masses and she was later diagnosed with diffuse large B-cell non-Hodgkin's lymphoma (DLBCL), with the adrenal glands being the dominant site of the disease. The patient was started on systemic chemotherapy, but her disease progressed with neurological involvement which responded to second-line therapy. Her adrenal disease however was refractory to further therapy.

Highlights

  • IntroductionMalignant lymphoma arising primarily in the endocrine organs is rare, accounting for less than 8% of extranodal presentations, and among the endocrine organs, the thyroid gland is most commonly involved [1]

  • Malignant lymphoma arising primarily in the endocrine organs is rare, accounting for less than 8% of extranodal presentations, and among the endocrine organs, the thyroid gland is most commonly involved [1].primary adrenal lymphoma (PAL) is a rare condition and may present as bilateral adrenal masses which may be rapidly growing

  • A 62-year-old lady known to suffer from hereditary spherocytosis (HS) was referred to hospital with progressively worsening symptomatic anaemia (Hb 7.7 g/dL), over a threeweek period

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Summary

Introduction

Malignant lymphoma arising primarily in the endocrine organs is rare, accounting for less than 8% of extranodal presentations, and among the endocrine organs, the thyroid gland is most commonly involved [1]. PAL is a rare condition and may present as bilateral adrenal masses which may be rapidly growing. Patients usually do not have disease elsewhere and this makes the diagnosis more difficult. Symptoms of the disease are variable and patients are most commonly older males. Presenting clinical features include pyrexia, lumbar pain, and/or symptoms of adrenal insufficiency. It may be diagnosed incidentally on abdominal imaging

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