Secondary immune thrombocytopenia (ITP) as an initial presentation of Whipple's disease.

Immune thrombocytopenia (ITP) is a heterogeneous autoimmune disease characterized by low platelet count that has been associated with a number of chronic infections but rarely described as a manifestation of Whipple's disease (WD). We present a case of Whipple's disease in a patient initially diagnosed with ITP. A 46-year old male in the fifth decade of life presented with presumed idiopathic ITP and was treated with several therapies including corticosteroids, rituximab, and thrombopoietin receptor agonists. Several years later, he developed weight loss and worsening arthralgias. He was found to have evidence of WD in a jejunal lymph node, the duodenum, and the cerebral spinal fluid (CSF). His diagnosis of WD, as a cause of secondary ITP, came a full 8 years after he was discovered to have thrombocytopenia and over 4 years after he was diagnosed with ITP. WD is an uncommon, multiorgan system disease caused by the actinomycete Tropheryma whipplei. Whipple's disease presents a diagnostic challenge due to the wide array of possible presenting clinical manifestations, as well as a prolonged time course with separation of symptoms over many years. While T. whipplei is ubiquitous in the environment, few individuals develop clinical disease, raising the prospect that select immunodeficiencies, both singular or in combination, may play a role in infection. While rare, in the appropriate clinical setting, one should consider infection with T. whipplei in addition to other chronic infections as a cause of secondary ITP regardless of how long ago the diagnosis of ITP was made.

Is parathyroid carcinoma indeed a lethal disease?

BACKGROUND: Parathyroid carcinoma is a rare malignancy with a wide range of aggressiveness. There is no current staging system. Our primary aim was to review the presentation, diagnosis, surgical treatment, and outcomes of patients, with the goal of assessing the incidence of death related to parathyroid carcinoma. METHODS: The authors present a retrospective chart review on patients with parathyroid carcinoma from 1975 to 2004, identified by the tumor registry of a single tertiary-care center. Diagnoses were confirmed histologically, clinical and radiographical data were recorded, and statistical analyses were performed. RESULTS: Twenty-three cases were identified. The mean patient age was 54 years. The female:male ratio was 1.5:1. Follow-up ranged from 1 month to 23 years (median, 134 months). Mean preoperative calcium was 12.9 mg/dL. Median parathyroid hormone was 290 pg/mL. Two patients (9%) had an asymptomatic presentation, and five (22%) presented with a palpable neck mass. Only nine (39%) underwent initial comprehensive en-bloc resection. Median survival was 22 years. Five- and 10-year survival was 85.9% and 69.4%, respectively. Five- and 10-year survival with en-bloc resection was 90% and 67.5%, respectively. Local resection resulted in survival rates of 82.5% and 70.7%. Three of ten deaths were attributed to parathyroid carcinoma. In recurrent disease, computed tomography and scintigraphy had localization rates of 53% and 67%, respectively, with a concordance of 22%. CONCLUSIONS: Long-term survival is possible with parathyroid carcinoma. Death associated with parathyroid carcinoma was uncommon. A staging may be warranted despite the rarity of this disease.

Nasal chondromesenchymal hamartoma: report of a case and review of the literature.

The sinonasal region is the site of several hamartomatous lesions, the majority of which are mesenchymal, with vascular hamartomas predominating. The occurrence of hamartomas in the nasal cavity of infants and children is especially rare. Nasal chondromesenchymal hamartoma (NCMH) is a rare lesion of the intranasal sinuses generally diagnosed in the newborn period, with the eldest reported patient presenting at 16 years of age. This neoplasm is composed of mesenchymal-stromal and chondroid tissue in varying proportions. It is felt to be analogous to the mesenchymal hamartoma of the chest wall, a lesion of similar histology generally involving the ribs and chest wall of neonates. To the best of our knowledge, only 14 cases of NCMH have been reported to date. We report a case of NCMH in an 11-year-old boy.