Hydroxyurea and colonic ulcers: a case report.

BACKGROUND: Hydroxyurea at a relatively low dose is frequently prescribed to induce hemoglobin F production in patients with sickle cell and ß-thalassemia diseases because of its good efficacy and safety profiles. However, a potentially fatal gastrointestinal ulceration was recently found and herein reported. CASE PRESENTATION: A thirty-seven-year-old man with transfusion dependent hemoglobin E/ß-thalassemia disease was treated with hydroxyurea to induce hemoglobin F production since 2007 without incident. From 2008 to April 2010, episodes of hematochezia, mucous diarrhea and epigastric pain intermittently manifested. Four colonoscopies done during the period repeatedly showed ulcerative lesions from the terminal ileum to the ascending colon with a non-specific histo-pathologic finding. Subsequently, ulcerative lesions also developed at the pharynx, histo-pathologic findings of which were not different from those in the colon. These ulcerative lesions resolved within a month after discontinuing hydroxyurea in April 2010 and have not recurred since. CONCLUSION: The findings suggested role of hydroxyurea in the pathogenesis of these ulcers, and that it must be immediately discontinued to prevent further damage to the digestive mucosa.

Laryngeal sarcocystosis accompanying laryngeal squamous cell carcinoma: case report and literature review.

Laryngeal sarcocystosis is an uncommon zoonotic coccidian protozoal infestation of human beings. The authors reviewed the pathology of 1,063 laryngeal biopsies over the past 10 years (2000 to 2009). Only one case of laryngeal sarcocystosis accompanying laryngeal squamous cell carcinoma was identified. The overall prevalence of laryngeal sarcocystosis was 0.094%. The case was a 66-year-old man who presented with voice hoarseness for six months. Physical examination and computed tomography revealed an ulcerative exophytic mass on the right true vocal cord, suggestive of laryngeal carcinoma. He underwent a right frontolateral partial laryngectomy. Histopathology showed a nonkeratinizing squamous cell carcinoma with Sarcocystis spp in the vocalis muscle. He was followed up and enrolled in speech therapy. The authors briefly review the clinicopathologic features and pathogenesis of muscular sarcocystosis and concurrent laryngeal sarcocystosis and squamous cell carcinoma.

Infection-associated hemophagocytic syndrome among patients with dengue shock syndrome and invasive aspergillosis: a case series and review of the literature.

The authors report four autopsy cases of previously healthy children with dengue shock syndrome complicated with infection-associated hemophagocytosis and invasive aspergillosis. Hemophagocytosis is confirmed by histopathology of autopsied reticuloendothelial organs. All four children were identified to have invasive aspergillosis by histopathology and three cases were positive on fungal culture for Aspergillus spp. Regarding the cause of death among the four children without pre-existing underlying disease, three cases were directly ascribable to invasive aspergillosis and the remaining case was ascribed to dengue shock syndrome. The transmigration of preexisting fungi from the respiratory mucosa damaged by the dengue shock process is postulated as the pathogenesis of invasive aspergillosis. The main predisposing factor was found to be prolonged dengue shock syndrome. We reviewed the clinicopathologic features and therapeutic management of infection-associated hemophagocytic syndrome in patients with dengue shock syndrome and invasive aspergillosis.

Adrenal histoplasmosis: a case series and review of the literature.

Adrenal histoplasmosis is an uncommon mycotic disease typically caused by Histoplasma capsulatum. The objective was to determine the clinicopathological findings in adrenal histoplasmosis. Pathological records were searched from the database at the Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University from 1993 to 2008 for cases of adrenal histoplasmosis. The keywords were "histoplasmosis" and "adrenal gland". Adrenal histoplasmosis was diagnosed by histopathology and Gomori-Grocott methenamine silver staining. Histoplasma capsulatum was confirmed by tissue culture and/or serology. The authors report seven cases of adrenal histoplasmosis in immunocompetent patients. The mean age at diagnosis was 67 years. All patients presented as chronic fatigue syndrome. The onset of symptoms ranged from one to three months. Addison's disease was found in adrenal histoplasmosis in one case (14.3%). The computed tomography revealed adrenal nodules measuring 1.2 to 7.8 cm in diameter. The histopathology showed granulomatous inflammation with caseous necrosis. Culture of adrenal tissue from two patients revealed Histoplasma capsulatum. Serum Histoplasma antibodies were positive in four cases. A cure was accomplished in 6 out of 7 cases (85.7%). The patients were followed up for 2.5 to 16.5 years.

Eosinophilic appendicitis caused by Schistosoma japonicum: a case report and review of the literature.

Parasitic appendicitis is uncommon. The authors reviewed the pathology of 4,130 appendices resected over the past 10 years (2000 to 2009). Only one case of eosinophilic appendicitis caused by Schistosoma japonicum was identified. The overall prevalence of schistosomal appendicitis was 0.024%. The case was a 61-year-old woman who presented with right lower quadrant abdominal pain. She had been a farmer in Chumphon and Surat Thani Provinces, which are endemic for schistosomiasis in Thailand. Physical, laboratory and ultrasound examinations were suggestive of acute appendicitis. She underwent emergency appendectomy. Intraoperative findings revealed a ruptured appendix with a fecalith in the appendiceal lumen. The histopathologic diagnosis was suppurative eosinophilic appendicitis with schistosomal ova in the mucosa, submucosa, muscular layer and vascular lumens, identified as S. japonicum eggs. The patient was treated for the parasite with praziquantal. We briefly review the clinicopathologic features and pathogenesis of schistosomal appendicitis.

Primary hepatic clear cell myomelanocytic tumor. Case report and review of the literature.

A case of hepatic clear cell myomelanocytic tumor in a 31-year-old woman presenting clinically with abdominal pain is reported. Histopathologic examination showed a lesion characterized by a population of large epithelioid cells with clear or eosinophilic granular cytoplasm, rich in glycogen. Immunohistochemically, the tumor cells were positive for HMB-45, Melan-A and muscle-specific actin, but negative for epithelial markers, desmin, S-100 protein, and neuroendocrine markers. Ultrastructurally, the tumor cells had abundant glycogen, well-developed rough endoplasmic reticulum, microtubules and aberrant melanosomes. Clinical and pathologic features with a brief review of the relevant literature for hepatic CCMMT as a variant of perivascular epithelioid cell tumor (PEComa) are discussed.

Hepatic fascioliasis due to Fasciola hepatica: a two-case report.

Two cases of hepatic fascioliasis due to Fasciola hepatica were retrieved from our surgical-pathology file since the hospital's foundation in 1969 up to 2005. The diagnosis of hepatic fascioliasis was based on detection of one live fluke in a large cystic lesion in the lobectomized liver specimen in one case and of deposited eggs in the large liver specimen obtained from open biopsy in the other Hepatic fascioliasis is rather rare and almost worldwide in distribution including Thailand. The diagnosis should be considered in the patient from endemic areas consisting of the northern, northeastern and upper-central regions of the country, with a history of ingesting fresh water plants or drinking untreated water and having fever right-upper-quadrant pain or intrahepatic cystic lesion(s) together with absolute peripheral blood eosinophilia.

Clinical and epidemiological analyses of human pythiosis in Thailand.

BACKGROUND: Pythiosis is an emerging and life-threatening infectious disease in humans and animals that is caused by the pathogenic oomycete Pythium insidiosum. Human pythiosis is found mostly in Thailand, although disease in animals has been increasingly reported worldwide. Clinical information on human pythiosis is limited, and health care professionals are unfamiliar with the disease, leading to underdiagnosis, delayed treatment, and poor prognosis. METHODS: To retrospectively study the clinical and epidemiological features of human pythiosis, we analyzed clinical data from patients with pythiosis diagnosed during the period of January 1985 through June 2003 at 9 tertiary care hospitals throughout Thailand. RESULTS: A total of 102 cases of human pythiosis were documented nationwide. A substantial proportion (40%) of cases occurred in the last 4 years of the 18-year study interval. Clinical presentations fell into 4 groups: cutaneous/subcutaneous cases (5% of cases), vascular cases (59%), ocular cases (33%), and disseminated cases (3%). Almost all patients with cutaneous/subcutaneous, vascular, and disseminated pythiosis (85%) had underlying thalassemia-hemoglobinopathy syndrome. Most ocular cases (84%) were associated with no underlying disease. A majority of the patients were male (71%), were aged 20-60 years (86%), and reported an agricultural occupation (75%). Regarding treatment outcomes, all patients with disseminated infection died; 78% of patients with vascular disease required limb amputation, and 40% of these patients died; and 79% of patients with ocular pythiosis required enucleation/evisceration. CONCLUSIONS: Here, we report, to our knowledge, the largest case study of human pythiosis. The disease has high rates of morbidity and mortality. Early diagnosis and effective treatment are urgently needed to improve clinical outcomes. Because P. insidiosum is distributed worldwide and can infect healthy individuals, an awareness of human pythiosis should be promoted in Thailand and in other countries.