A 37-year-old man presents to the Emergency Department with abdominal pain exacerbation of 3 hours of evolution associated diarrhea with melena. The patient had a documented medical history of intermittent abdominal pain and diarrhea over the 6 months prior to the current admission. Upon admission, the patient presented febrile and in anasarca, with major edema in lower limbs. Initial blood work showed moderate microcytic anemia (Hb 10.3 g/dl, Hct. 23.7%, and MCV 75 fL), severe hypoalbuminemia (1.7 g/dL), inversion of albumin/globulin ratio, and CBC did not show signs of infection.
The patient was started on protein replenishment with human albumin which resolved partially the edema.
An exhaustive workout was performed to identify the underlying cause of the edema and low protein levels. Liver disease was ruled out due to normal liver profile (total bilirubin 0.21 mg/dL, indirect bilirubin 0.11 mg/dL, direct bilirubin 0.10 mg/dL, AST 9 IU/L, and ALT 8 IU/L) and the negative liver ultrasound. Nephrotic syndrome was also ruled out as a possible diagnosis.
Due to the persistence of abdominal pain, structural damage or tumor processes were investigated; however, abdominal CT only showed edema in the small intestine wall suggesting chronic enteropathy.
Although the patient was considered initially immunocompetent, there was a significant drop in complement C3 0.5 g/L (negative <0.8 g/L) and C4 0.03 g/L (negative <0.10 g/L). Further laboratory testing showed hypogammaglobulinemia (22 mg/dL IgA (normal 22-149 mg/dL), 150 mg/dL IgG (normal 615-1530 mg/dL), 20 mg/dL IgM (normal 31-272 mg/dL)), and 20 mL/24 hrs alpha-1 antitrypsin clearance.
An upper digestive endoscopy was performed showing a whitish lace pattern and erythematous walls in the second portion of the duodenum. In addition, a colonoscopy showed hemorrhoidal packages, multiple ulcerated lesions in sigma, and elevated, pseudopolypoid, infiltrative lesions in cecum and ileocecal valve.
Since our patient lived in a tuberculosis endemic area and that the lesions simulated intestinal tuberculosis, empirical antituberculosis therapy (isoniazid 75 mg, rifampicin 150 mg, ethambutol 275 mg, and pyrazinamide 400 mg) was started until biopsy results came back.
Multiple cryptococci were found in samples taken from the cecum, sigma, and ileocecal valve using the techniques of immunohistochemistry CD68, Grocott stain, PAS stain, and Alcian blue stain (Figure 1). In addition, the presence of intestinal lymphangiectasia was confirmed with D240 staining (Figure 2).
After receiving the pathology report, antituberculosis therapy was suspended and a regimen consisting of fluconazole 800 mg/day IV for 2 weeks, and octreotide 1 ml subcutaneously every 8 hours was initiated. Due to the severity of the presentation octreotide was started along with dietary treatment. Octreotide and oral fluconazole 600 mg were maintained for 90 days after discharge with favorable symptomatic evolution, additionally, following laboratory controls shown that albumin levels went back to normal.