Background Agranulocytosis, defined as absolute neutrophil count (ANC) < 500/µL due to antithyroid drugs (ATDs), methimazole, carbimazole, and propylthiouracil (PTU), is a rare but serious complication with an occurrence of 0.1- 0.5%. ATDs induced agranulocytosis occurs abruptly, usually in the first three months of treatment but can occur any time after initiation of treatment, and although it is more common with a higher dose of methimazole > 30 mg/day, it can also occur with lower dosages. We present a rare case of agranulocytosis in a patient with Amiodarone-induced Thyrotoxicosis (AIT) with an ANC of 0/µL. Clinical Case A 68-year-old male with atrial fibrillation and heart failure was diagnosed with AIT when he presented to ED with thyrotoxicosis. Amiodarone was discontinued. Methimazole 40 mg daily with prednisone 20 mg daily was initiated. The ANC was 4360/µL. After nine weeks on methimazole, he presented with fever and tremors. The laboratory tests during this admission revealed WBC 1310/µL (4000-11000/µL) with granulocytes 0% (35%-80%), ANC 0/µL (1820-7420/µL), TSH < 0.01 (0.45-5.3mIU/mL), free T4 5.5 (0.58-1.64 ng/dL), free T3 4.4 (2.8-4.4 pg/dL). Methimazole was discontinued and antibiotics started. He received six doses of Granulocyte colony-stimulating factor (G-CSF) Filgrastim 480 mcg with an improvement of WBC to 6370/µL with a granulocyte count of 60% on the 6th day of admission. He eventually underwent thyroidectomy for treatment of the thyrotoxicosis. Discussion The proposed mechanism of agranulocytosis with ATDs is twofold. First, there is a direct toxic effect on mature circulating neutrophils and stem cells. The second is complement-mediated antibodies against granulocytes. Furthermore, AIT is associated with a higher risk for ATD induced agranulocytosis, although the mechanism remains elusive. The most common manifestations are fever and sore throat. The management includes discontinuation of ATD and starting antibiotics. G-CSF is used to treat agranulocytosis as it decreases the recovery time, the rate of infectious complications, and the mortality rate. Patients with ANC < 100/µL have a greater risk of infectious and fatal complications than patients with ANC > 100/µL. After resolution of agranulocytosis, methimazole or any other thionamides are generally not recommended due to concern for recurrence due to cross-reactivity among thionamides. Thyroidectomy or radioiodine therapy should be considered for definitive treatment of the hyperthyroidism in this circumstance. Conclusion Due to the low incidence and abruptness of agranulocytosis from ATDs, controversy exists over the value of routine monitoring of WBC count. This case emphasizes routine WBC monitoring could be considered in patients on higher doses of methimazole, especially in the first three months, due to the increased risk of agranulocytosis. Patient education on the life-threatening complication of ATD induced agranulocytosis is vital. It helps patients recognize early symptoms and seek medical care promptly, which aids in early intervention and reduces morbidity and mortality. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
INTRODUCTION: Pulmonary Renal Syndrome (PRS) is characterized by diffuse alveolar hemorrhage (DAH) and glomerulonephritis (GN). PRS is usually associated with antibody-mediated vasculitis. PRS from ANCA negative vasculitis is a rare entity and has a fulminant course without treatment. Due to the paucity of cases, there is no established treatment strategy so far.
Background: Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor. It arises from the chromaffin cells of adrenal medulla. It is diagnosed in 5–6.5% of adrenal incidentalomas which is not common. The usual clinical presentation includes the classic triad of sweating, headache and tachycardia. However, asymptomatic cases are seen in 8% of the patients with pheochromocytoma. We present a clinically asymptomatic patient diagnosed during work up of adrenal incidentaloma. The possible etiology for silent presentation includes one of the following:(i) Presence of a smaller functional tissue (ii)Accelerated turnover of the tumor causing release of the unmetabolized catecholamines in small amounts (iii) Pulsatile tumor secretion (iv)Tumors triggered by stress (v) Laboratory errors due to inappropriate handling of specimen at high-temperature (vi) False negative test results secondary to caffeine ingestion in the prior 24 hours. Clinical Case: 59 years old Caucasian female with past medical history of type 2 diabetes mellitus, obesity, essential hypertension, nonischemic cardiomyopathy, and asthma presented to the emergency room with complaints of worsening shortness of breath and pedal edema for 1 month. Physical exam: Blood pressure 146/78 mm of Hg and heart rate 82 beats/min, mild pedal edema, no pulmonary crackles. On imaging, CT angio chest showed irregularly enhancing right adrenal mass measuring 3.4 cm. This adrenal incidentaloma was not visualized on imaging done 5 years ago. Further, MRI abdomen revealed 4.1 cm right adrenal mass. Laboratory testing showed high total plasma metanephrines: 890 pg/ml (< or = 205), 24-hour urine metanephrines: 2337 (140–785), A1C: 10%. This confirmed the diagnosis of adrenal pheochromocytoma. Preoperatively, she was started on phenoxybenzamine 10 mg BID and encouraged on liberal salt intake. During the course, her blood pressure and heart rate were monitored daily. She underwent right adrenalectomy. Surgical pathology revealed 4.1 cm pheochromocytoma, negative margins with extension to the adipose tissues and vascular invasion, PASS score = 4. Post operatively, patient declined to get labs done. Due to high risk behavior of the tumor, patient needs to be monitored annually for lifelong. Conclusion: Pheochromocytoma is an uncommon tumor with varied clinical presentation. It can manifest itself widely from being silent to aggressive disease. This warrants high suspicion, early detection and management, thereby reducing the morbidity and mortality. Lately, there has been increased incidence of adrenal incidentalomas owing to widespread use of radiological investigations. We report a case of incidental pheochromocytoma which is biochemically active but clinically asymptomatic. This emphasizes the importance of being more vigilant during the evaluation of adrenal incidentalomas.
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