Pachydermoperiostosis Accompanied by Heart Failure

Pachydermoperiostosis or primary hypertrophic osteoarthropathy is an uncommon disease of acromegaloid facial feature, but characterized by unique phenotype (digital clubbing and pachydermia) and distinctive radiographic appearances like periostosis. We experienced a case with complete form of pachydermoperiostosis accompanied by heart failure. He presented with typical features consisting of clubbing with enlargement of the hand, thickening of facial skin and periosteal new bone formation involving lower leg. Echocardiography revealed severely decreased left ventricular systolic function. Treatment with medications resulted in an improvement of cardiac function and symptom. There is no previous report documenting pachydermoperiostosis accompanied by heart failure. We report that case for the first time.

Peripheral Blood Eosinophilia: An Unusual Presentation of Bone Marrow Involvement in a Patient with Relapsed Thyroid Papillary Carcinoma

Peripheral blood eosinophilia is a well-known paraneoplastic manifestation, but its underlying mechanism is still unclear. Bone marrow metastasis may be a cause of malignancy-associated eosinophilia. However, there is limited evidence of the relationship between bone marrow metastasis and eosinophilia. Herein, we present a unique case of peripheral blood eosinophilia associated with bone marrow invasion in a patient having a history of papillary thyroid carcinoma. A 68-year old woman showed peripheral blood eosinophilia (91,525/mm3). Since the time she was initially diagnosed as having papillary thyroid carcinoma, eosinophilia had never been found and the other causes of eosinophilia were excluded. A bone marrow study revealed cancer cell infiltration; multiple lymphadenopathies and liver metastasis were also detected. We treated her with steroid; however, her eosinophilia did not respond to steroid and the patient died due to disease progression. Although peripheral blood eosinophilia and bone marrow metastasis are rare findings in patients with papillary thyroid carcinoma, we suggest that eosinophilia might be a sign of the bone marrow metastasis of papillary thyroid carcinoma.

A Case of Primary Intestinal Lymphangiectasia / 대한소화기내시경학회지

We described a 22-month-old girl, who suffered from a gereralized edema and intermittent diarrhea for 1 month. Intestinal lymphangiectasia was confirmed by endoscopic biopsy. Endoscopic examination revealed showed the characteristic appearance of tiny white flakes scattered in the duodenum, which a histological examination confirmed to be dilated lymph vessels. The other findings from laboratory examinations included lymphopenia, hypoproteinemia, hypogammaglobulinemia and increased fecal a1-antitrypsin clearance. Treatment consisting of a low-fat diet and MCT supplementation was successful. The therapy has resulted in the disappearance of duodenal lesions previously observed endoscopically.