The number of cancer survivors is estimated by 2022 to increase to almost 18 million, in part because of improvements in earlier detection and cancer therapies, leading to longer-term survival of cancer patients. This growing number of survivors has presented challenges to the healthcare community, one of which is the need to provide to the survivor a seamless transition from the oncologist to the primary care provider (PCP). A major national initiative is under way for oncologists to provide survivorship care plans to their patients and PCPs, with the aim of communicating a complete record of cancer treatment and guiding the PCP in the future care of these cancer survivors. In caring for cancer survivors, PCPs must be familiar with the long-term and late effects, both medical and psychosocial, that are associated with common cancer treatments as well as the oncologic diagnosis itself. This article provides a unique approach to the traditional history and physical of easing the transition to primary care after completing cancer therapy for the cancer survivor. The cancer survivor-focused history and physical provide the PCP with a familiar and efficient method to clinically evaluate cancer survivors that closes the gap in this important transition of care.
Background
VEXAS is a recently described inflammatory disease caused by mutations in the UBA1 gene. Symptoms are diverse and include fevers, cartilaginous inflammation, lung inflammation, vasculitis, neutrophilic dermatoses, and macrocytic anemia. Cytoplasmic inclusions in myeloid and erythroid progenitors in the bone marrow are a hallmark feature. Here we report the first case of VEXAS with non-caseating granulomas in the bone marrow.
Case presentation
A 62-year-old Asian male presented with fevers, erythema nodosum, inflammatory arthritis, and periorbital inflammation. Labs were significant for persistently elevated inflammatory markers and macrocytic anemia. Over the years his symptoms and inflammatory markers only improved with glucocorticoids and recurred when prednisone dose was lowered below 15–20 mg daily. He underwent bone marrow biopsy showing non-caseating granulomas and PET scan showing hilar/mediastinal lymphadenopathy. He was initially diagnosed with IgG4-related disease (treated with rituximab) and later sarcoidosis (treated with infliximab). After failing these agents, the possibility of VEXAS was considered and later confirmed by molecular testing.
Conclusions
To the best of our knowledge, this is the first observation of non-caseating granulomas in VEXAS, a cautionary reminder of its non-specificity since misinterpretation can lead to diagnostic delay. VEXAS should be in the differential in patients with symptoms of chronic inflammation responding positively to steroids (but not to B-cell depletion or TNF inhibition), which is in line with previous literature.
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