Introduction. Senile systemic amyloidosis is a multisystem disease where wild-type insoluble transthyretin (ATTRwt) protein gets deposited in the tissues leading to organ dysfunction. Methodology. We present the case of a patient who presented with hematuria and bladder involvement by ATTRwt amyloidosis who ultimately died of multiorgan failure. Results. The patient was an 82-year-old white male with a history of ischemic cardiomyopathy (ejection fraction (EF): 20–25%), chronic atrial fibrillation, chronic kidney disease (CKD), and carpal tunnel syndrome who presented with acute hematuria, urinary retention, and progressive fatigue. He underwent cystoscopy and bladder biopsy which was positive on congo-red stain diagnostic for amyloidosis. Echocardiogram demonstrated worsening of EF to 10–15% and concentric left ventricle hypertrophy. MRI was not performed due to underlying CKD. His condition deteriorated during the hospital stay, and he developed cardiogenic shock and progressive liver dysfunction. Infectious workup was negative. Meanwhile, the biochemical investigations (serum protein electrophoresis, immunofixation, and urine kappa/lambda chains) ruled out plasma cell dyscrasias. Mass spectrometry analysis of the bladder biopsy specimen confirmed wild-type transthyretin (ATTRwt) amyloidosis consistent with senile systemic amyloidosis. Due to patients’ extremely poor prognosis, his family wished to focus on patient’s comfort-oriented measures only, and patient passed away shortly thereafter. Conclusion. Senile systemic amyloidosis can rarely present in an atypical fashion such as hematuria. The treatment options are limited in this disease process. Novel therapies are in the early phases of development. Concern also exists that in patients with multiple comorbidities, this entity is under recognized until the more advanced stages.
Pemphigoid diseases are a group of blistering autoimmune pathologies including pemphigus vulgaris (PV) and pemphigus foliaceous, which affect mucocutaneous tissues. Non-steroidal anti-inflammatory drugs, penicillamine, and angiotensin-converting enzyme inhibitors such as captopril and enalapril are associated with drug-induced pemphigoid. We present a case of lisinopril-associated PV which has not been previously reported.
Mantle cell lymphoma (MCL) is a rare and incurable subtype of non-Hodgkin's lymphoma (NHL). Primary gastrointestinal (GI) MCLs are even rarer, accounting for only 1%-4% of GI lymphomas. We describe a case of a 77-year-old female who presented with complaints of indigestion and abdominal bloating. An upper endoscopy was performed which revealed a duodenal bulb polyp, biopsies of which were consistent with MCL. She was initially observed without any chemotherapy; however, a repeat endoscopy two years later revealed that she now also had MCL of the ileocecal valve. The patient was initiated on treatment with rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP). She underwent regular surveillance with her oncologist after completion of her chemotherapy and repeat surveillance scans remained negative for any recurrence. A repeat upper endoscopy with endoscopic ultrasound and colonoscopy were performed which showed complete endoscopic and histopathological remission of her lymphoma. Patients with MCL typically have a poor prognosis; however, our patient remains symptom free and in complete remission six years from her initial diagnosis.
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