Rare Complication of a Rare Malignancy: Case Report of Cardiac Amyloidosis Secondary to Waldenstrom Macroglobulinemia.

Cardiac amyloidosis is a rare disorder caused by the myocardial deposition of abnormal fibrils. A 52-year-old man was referred to our center with clinical features of heart failure, after cardiac magnetic resonance imaging showed restrictive cardiomyopathy. Abdominal fat pad biopsy showed features of amyloidosis, and after hematological workup, he was diagnosed with Waldenstrom macroglobulinemia (WM). He was initiated on a rituximab-based chemotherapy regimen, and his cardiac function was assessed serially. Because of non-response, he was switched to a bortezomib-based regimen. Unfortunately, three days into this regimen, the patient died. WM is a rare plasma cell dyscrasia with a nonspecific presentation. It uncommonly presents with sequelae of amyloidosis-the IgM subtype of amyloid-light chain (AL) amyloidosis. Diagnostic delays are common, contributing to an already poor prognosis. Amyloidosis in WM requires urgent treatment - clonal chemotherapy, and supportive cardiac care in heart involvement. Bortezomib-based regimens are commonly recommended, with diuretics as the mainstay for cardiac treatment. However, in most advanced cases, the prognosis is poor; thus, a high degree of suspicion is necessary for early diagnosis. This case illustrates the possible presentation of cardiac amyloidosis as a rare malignancy.

Clinical and Radiological Profile of Cerebrovascular Disease in Polycythemia: Analysis of Neurologic Manifestations from a Tertiary Center in South India.

PURPOSE: Patients with polycythemia, either primary or secondary, are at elevated risk for thrombotic complications, including stroke. We aimed to investigate the clinical and radiological characteristics of cerebrovascular disease (CVD) in polycythemia, and describe other neurologic manifestations. METHODOLOGY: We conducted a cross-sectional study of patients diagnosed with polycythemia between 2014 and 2019 at a tertiary care center and collected relevant medical data with a special focus on cerebrovascular disease and neurologic manifestations. We performed descriptive and inferential analyses. We have also described and analyzed the available neuroimaging features. RESULTS: We analyzed data from 56 patients. 20 patients (35.7%) had ischemic stroke. The incidence of CVD was higher in those with primary polycythemia (43%) than in those with secondary polycythemia (8%). The most common subtype of stroke was large vessel disease, and the most common arterial territory was the anterior circulation. There was no statistically significant difference in the hematocrit level between those with or without CVD. Neuroimaging revealed multiple large vessel intracranial stenoses on MR Angiography, and hyperdense vessels on plain CT. Other neurologic manifestations included headache, seizures, dizziness, visual symptoms and papilledema, and these were significantly more common in primary polycythemia. CONCLUSIONS: CVD is common in patients with polycythemia. The most common type observed was large vessel occlusion, predominantly in the anterior circulation. In stroke patients, multiple vessel stenosis and hyperdense vessels may be clues to polycythemia.