A Case of Atypical Thrombotic Microangiopathy

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.

A Case of Atypical Thrombotic Microangiopathy

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.

Disseminated Scedosporium apiospermum Infection Induced from Aspiration Pneumonia after Near-Drowning / 결핵및호흡기질환

Scedosporium apiospermum, the anamorph of Pseudallescheria boydii, is a ubiquitous saprophytic fungus. S. apiospermum can cause life-threatening infections usually in immunocompromised patients or after near-drowning incidents. Here, we report the first case of disseminated infection caused by S. apiospermum after near-drowning in Korea. A 44-year-old healthy man developed aspiration pneumonia, followed by multiple brain abscesses, and endopthalmitis, after a near-drowning incident in a septic tank. S. apiospermum infection was diagnosed on the 33rd day after the incident had occurred. The patient died from the progressive renal failure 255 days after incident, although he had been treated with voriconazole.

Two cases of acute fibrinous and organizing pneumonia / 대한내과학회지

Acute fibrinous and organizing pneumonia (AFOP) is a histological pattern consisting of intra-alveolar fibrin in the form of fibrin "balls" and organizing pneumonia, without hyaline membranes or prominent eosinophil infiltration. Some reports suggest that the clinical course and pathological findings of AFOP are different from typical findings of bronchiolitis obliterans organizing pneumonia (BOOP) or eosinophilic pneumonia (EP), and its prognosis can be better or similar to that of diffuse alveolar damage (DAD). We report two cases of pathologically demonstrated AFOP experienced recently at our institute. One fatal case revealed a rapid development of respiratory failure and the need for mechanical ventilation. Another nonfatal case revealed subacute diffuse bilateral lung infiltration without the need for mechanical ventilation. Judging from our experience, the patient who required a mechanical ventilator had a poorer prognosis than the one who did not need a mechanical ventilator.

Two cases of acute fibrinous and organizing pneumonia / 대한내과학회지

Acute fibrinous and organizing pneumonia (AFOP) is a histological pattern consisting of intra-alveolar fibrin in the form of fibrin "balls" and organizing pneumonia, without hyaline membranes or prominent eosinophil infiltration. Some reports suggest that the clinical course and pathological findings of AFOP are different from typical findings of bronchiolitis obliterans organizing pneumonia (BOOP) or eosinophilic pneumonia (EP), and its prognosis can be better or similar to that of diffuse alveolar damage (DAD). We report two cases of pathologically demonstrated AFOP experienced recently at our institute. One fatal case revealed a rapid development of respiratory failure and the need for mechanical ventilation. Another nonfatal case revealed subacute diffuse bilateral lung infiltration without the need for mechanical ventilation. Judging from our experience, the patient who required a mechanical ventilator had a poorer prognosis than the one who did not need a mechanical ventilator.

A Case of Mucosa-Associated Lymphoid Tissue Lymphoma in the Esophagus Accompanied by Bronchus-Associated Lymphoid Tissue Lymphoma / 대한소화기내시경학회지

Mucosa-associated lymphoid tissue (MALT) is specially adapted component of the immune system protecting the permeable surface of the gastrointestinal mucosa, bronchial mucosa and other mucosa. Chronic infection of the stomach by Helicobacter pylori, Hashimoto's thyroiditis, Sjogren syndrome in the salivary gland and other chronic inflammatory and autoimmune disease lead to the accumulation of MALT in the mucosa and MALT lymphoma arises from this acquired MALT. MALT lymphoma is histologically characterized by proliferation of centrocyte-like cells that invade the epithelium and lymphoepithelial lesion form. Gastrointestinal MALT lymphoma is clinically important because it is a localized, slow progressive disease and has a long survival and favorable clinical course compared with other lymphoma. Esophageal MALT lymphoma is extremely rare, so we report, radiologic, endoscopic and pathological findings and clinical course in a case of esophageal MALT lymphoma with brouchas-associated lymphoid tissue (BALT) lymphoma with a review of literature.

Diversion Colitis: Diversion Colitis A case report

Pseudomyxoma peritonei may result from implantation of benign or malignant tumor in peritoneal cavity and is filled with gelatinous material (termed "Jelly Belly") in abdominal cavity. Its origin is usually an appendiceal or ovarian mucinous adenoma or cystadenocarcinoma, but other primary origin such as uterus, intestine, pancreas and stomach umor have been reported. Generally, pseudomyxoma peritonei is slowly progressive and has low grade malignant potential. This report presents a unusual long term survival after evacuation of 15,000 cc of gelatinous material from abdominal cavity which was the low grade mucinous adenocarcinoma and a review of the current literature, management and new its concept. The origin of pseudomyxoma peritonei of this case was most likely from appendiceal mvcinous adenocarcinoma.