1/7. histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) associated with antiphospholipid syndrome: case report and literature review.histiocytic necrotizing lymphadenitis (HNL), or Kikuchi-Fujimoto disease, is a benign, self-limited disease that predominantly occurs in women. The etiology remains undetermined, although a viral or autoimmune hypothesis has been suggested. The disease usually emerges with cervical lymphadenopathy with or without fever. The diagnosis can be confirmed only by histological findings of lymph node biopsy, characterized by necrosis and histiocytic infiltration without neutrophils. We report a case of a 28-year-old woman with a medical history of two episodes of unexplained pulmonary embolisms (3 and 2 years previously) who was admitted to our hospital because of unilateral cervical lymphadenopathy and mild fever that presented 1 week before admission. A diagnosis of HNL was performed by lymph node biopsy. In parallel, whereas the laboratory tests for inherited thrombophilia were negative, a progressive elevated titer of anti-beta(2) glycoprotein I (GPI) antibodies was established. Because of persistent fever, the patient received a short course of corticosteroid therapy and she recovered completely from the HNL after 2 months. It is noteworthy that to date the patient has displayed an elevated titer of anti-beta(2) GPI antibodies (18 months after the recovery from the HNL). Thus, considering the previous history of venous thrombosis and the presence of antiphospholipid antibodies, the diagnosis of primary antiphospholipid syndrome associated with HNL was made. To our knowledge, this is the first report in the literature describing antiphospholipid syndrome associated with HNL. Moreover, a brief literature review is provided with emphasis on the etiology, clinical course, and pathogenesis of this rare disease entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Cardiopulmonary complications in multicentric reticulohistiocytosis. Report of a case.Multicentric reticulohistiocytosis (MR) is a rare disease. Only recently was its systemic nature appreciated. It affects the skin, mucous membranes, joints, muscles, tendon sheaths, synovial membranes, bones, liver, kidney, lymph nodes, heart, and lungs. Our patient, a 50-year-old woman, had life-threatening cardiopulmonary complications of MR. The connection between the skin lesions, the arthritis, and the pathologic changes in the heart and lungs is still obscure.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Fatal acute pulmonary embolism in a patient with pelvic lipomatosis after surgery performed after transatlantic airplane travel.We describe a case of a 37-yr-old patient who traveled from europe to the United States and succumbed to a massive pulmonary embolism 6 days after elective pelvic surgery despite routine postoperative thrombotic prophylaxis. In retrospect, he was likely to have developed a deep venous thrombosis during the transatlantic trip to our hospital. Anesthesiologists and other physicians involved in perioperative management need to be aware of the prevalence of venous thromboembolism in patients with a history of recent prolonged air travel. This is particularly true in tertiary referral centers, where patients with rare diseases may have a major surgical intervention within days of prolonged air travel.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Primary extranodal nasal-type natural killer/T-cell lymphoma of the brain: a case report.Natural killer (NK)/T-cell lymphomas represent a rare type of lymphoma derived from either activated NK cells or, rarely, cytotoxic T cells. These lesions are most commonly extranodal and tend to present as destructive lesions within the midline facial structures. Other than the nasal cavity and paranasal sinuses, several other extranodal sites of involvement have been reported, including the pharynx, gastrointestinal tract, and testis. Although secondary involvement of the central nervous system has been reported, a convincing case of primary brain NK/T-cell lymphoma has not been previously reported. Here, we report a case of primary brain lymphoma of NK/T-cell type with a characteristic phenotype expressing CD3epsilon, CD56, granzyme B, Epstein-Barr virus-encoded small nuclear RNAs, with germline T-cell receptor gene configuration, and showing an unusual intravascular component. The patient underwent extensive imaging studies, revealing only the brain lesion. The lymphoma failed to respond to therapy and the patient eventually died after transfer to a hospice facility. This unusual case highlights an unusual presentation of a rare disease entity and highlights the need for a better understanding of the biology and treatment of T-cell lymphomas.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. A case of Sweet's syndrome following septic pulmonary emboli after high-dose chemotherapy for advanced testicular cancer.A 33-year-old man with advanced testicular cancer underwent high-dose chemotherapy combined with peripheral blood stem cell transplantation. After administration of granulocyte colony-stimulating factor (G-CSF), multiple infiltrative erythema was identified on the face, thigh, and lower leg. A dermatologist diagnosed this as Sweet's syndrome caused by G-CSF; consequently G-CSF administration was stopped. When the skin lesions had improved, phlebitis was found at the injection site of the peripheral vein catheter. The patient then suffered from sudden left chest pain and dyspnea. Chest computed tomography showed the characteristic findings of septic pulmonary emboli (SPE). He was treated by the administration of vancomycin, fluconazole, and pazufloxacin mesilate. Although Sweet's syndrome and SPE are rare diseases, the presence of these diseases must be considered when performing chemotherapy for urological malignancy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. Unusual survival after pulmonary artery sarcoma.A 46-year-old man with a primary sarcoma of the pulmonary artery is presented. The tumor was responsive to radiotherapy, and the patient is the longest reported survivor of this rare disease. The tumor spread distally in the pulmonary vasculature, with probable embolization to the central nervous system, and he died 27 months after the initial diagnosis, with sudden massive hemoptysis. pulmonary artery sarcomas are a rare cause of obstruction of the right ventricular outflow. The course of our patient suggests that early recognition and therapy may provide some of these patients with effective palliation and prolonged survival.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Pulmonary arteritis with pulmonary arterial thrombosis and recurrent endopulmonary embolization.A 41-year-old woman underwent medical examination for superficial thrombophlebitis of both lower legs. Incidentally a chronic myelogenous leukemia was diagnosed and chemotherapeutically treated. Three weeks after the first attendance the patient again suffered superficial thrombophlebitides of all extremities. Clinically she exhibited symptoms of recurrent mild pulmonary embolization. Contrast venography revealed no signs of deep venous thrombosis of legs, pelvis, or cava inferior. Despite continuous full-dose intravenous heparin administration the patient died, with signs of fulminant pulmonary embolization. Surprisingly, necropsy revealed a complete thrombotic occlusion of the pulmonary arterial system caused by pulmonary arteritis with signs of recurrent pulmonary embolization from a parietal truncus pulmonalis thrombosis. In addition, an appositional growth of parietal thrombi central from peripheral arterial ramifications had occurred. Simultaneously, superficial thrombophlebitis of all extremities was observed without any additional signs of general vasculitis. There was no strong evidence for a causal relationship between the chronic myelogenous leukemia and pulmonary arteritis nor for any other underlying systemic disorder. Therefore we consider the pulmonary arteritis a possibly primary one. This very rare disease is discussed with respect to the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |