Internal medicine
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Fibrosing mediastinitis is rare. One type of this disease is idiopathic fibrosing mediastinitis. It is necessary to rule out malignancy in order to accurately diagnose fibrosing mediastinitis. ⋯ Immediately after initiating chemotherapy for lymphoma, the patient's symptoms improved dramatically and the mediastinal lesion decreased in size. Although few similar cases have been reported, hidden malignancy may present as fibrosing mediastinitis. Therefore, physicians should consider the probability of malignancy in patients with fibrosing mediastinitis because treatments may vary accordingly.
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Acute promyelocytic leukemia (APL) is characterized by the proliferation of blasts with distinct morphology and promyelocytic leukemia-retinoic acid receptor alpha (PML-RARA) transcripts. Although the treatment outcome is dramatically improved by all-trans retinoic acid (ATRA), life-threatening bleeding from enhanced fibrinolytic-type disseminated intravascular coagulation (DIC) remains a serious clinical problem, and a standard treatment has not been established. However, recent reports indicate that recombinant human soluble thrombomodulin (rTM) is effective against DIC. ⋯ Based on the present findings, rTM administration may be an effective, safe, and feasible therapeutic modality, producing a rapid resolution without progression of hemorrahage.
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Case Reports
Two cases of acute respiratory distress syndrome with high values of Chlamydophila pneumoniae-specific antibodies.
We herein report two cases of acute respiratory distress syndrome (ARDS) with high values of Chlamydophila pneumoniae-specific antibodies. In the first case (a 65-year-old man), high levels of anti-C. pneumoniae antibodies (IgG and IgA) were detected on admission, and the anti-C. pneumoniae IgA level rose by Day 30. The patient was successfully treated with quinolone and steroids. ⋯ The patient did not recover, despite receiving treatment with several antibiotics and anti-inflammatory agents. Neither of the patients displayed other pathogen-specific antigens or antibodies. Chlamydophila pneumonia is usually mild, although it can cause severe interstitial pneumonia and ARDS in reinfected patients and the elderly.
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Type 1 autoimmune pancreatitis (AIP) is characterized by a high serum IgG4 concentration and is closely associated with the HLA-DRB1(*)04:05-DQB1(*)04:01 haplotype, for which family studies may disclose its immunogenetic significance. In the present study, we encountered two male siblings with type 1 AIP who exhibited diffuse pancreatic swelling with a capsule-like rim and diffuse pancreatic duct stricture. The younger brother also displayed characteristic IgG4-related sialadenitis and retroperitoneal fibrosis. Contrary to our expectations, the siblings showed only normal or slightly elevated values of serum IgG4 and no HLA DRB1(*)04:05-DQB1(*)04:01 haplotype, suggesting that type 1 autoimmune pancreatitis is associated with multiple immunogenetic factors.
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Case Reports
Pulmonary tumor thrombotic microangiopathy with circulatory failure treated with imatinib.
Pulmonary tumor thrombotic microangiopathy is a lethal, yet difficult to diagnose, complication of gastrointestinal carcinoma. Even if properly diagnosed, there is no treatment, especially after a circulatory collapse. ⋯ Pulmonary hypertension was dramatically ameliorated and the patient was able to be weaned from percutaneous cardiopulmonary support within 20 days of treatment. Imatinib may be effective for ameliorating pulmonary hypertension that is caused by pulmonary tumor thrombotic microangiopathy.