Severe complication during remission of Crohn's disease: hemophagocytic lymphohistiocytosis due to acute cytomegalovirus infection.

INTRODUCTION: Immunosuppressive therapy is today's standard treatment of patients with moderate to severe inflammatory bowel disease (IBD). The risk for opportunistic infections is increased due to this therapy and is a concern in the management of patient with IBD undergoing such a treatment. CASE REPORT: In this paper, we describe a case of an acute cytomegalovirus (CMV) infection in a 35-year-old male patient with Crohn's disease being in remission while receiving azathioprine therapy. His clinical presentation was high-grade fever, night sweats, skin rash, and abdominal pain.Laboratory findings showed pancytopenia, elevated liver enzymes, and high ferritin levels. Sonographic examination revealed splenomegaly and serological analysis proved an acute CMV infection. The severity of the acute illness and these results in the setting of immunosuppressive treatment with azathioprine were highly suspicious of hemophagocytic lymphohistiocytosis (HLH).Further investigations including bone marrow biopsy, analysis of natural killer cell function, and measurement of T-cell activity confirmed the suspected diagnosis. Treatment consisted of antiviral and symptomatic therapy. DISCUSSION AND CONCLUSION: HLH is a rare and severe condition triggered by uncontrolled stimulation of histiocytes and lymphocytes, resulting in abnormal cytokine production. The causes can be primary (genetic) or secondary due to acquired immunodeficiency or viral infections such as CMV. Several symptoms of this condition are unspecific, but the summary of clinical symptoms and signs are diagnostic. Treatment consists of specific intervention if possible and application of immunosuppressive drugs such as corticosteroids.

Sudden death after major head and neck cancer surgery due to undetected arrhythmogenic right ventricle cardiomyopathy (ARVC).

BACKGROUND: Oral squamous cell carcinoma is the sixth most frequent malignancy in Austria. The incidence of arrhythmogenic right ventricle dysplasia (ARVC), an important cause of sudden cardiac death, is estimated at 1:5000 to 1:1000. CASE REPORT: We present a case of a 75-year-old woman who underwent major oncologic surgery for T4aN0M0 maxillary squamous cell carcinoma and reconstruction with a scapular-latissimus dorsi microvascular flap. The patient died suddenly during her postoperative care. Autopsy revealed pericardiac tamponade due to rupture of the right ventricular wall. Histologic examination showed ARVC in a sample taken directly from the ruptured area. Cause of death was secondary arrhythmia originating from the ARVC. The arrhythmia had led to rupture of the right ventricular wall and sudden cardiac death. DISCUSSION: As per our current knowledge, no cases of maxillary cancer and ARVC as co-morbidities have been reported in the literature. The patient had been given anti-arrhythmia treatment for previously detected atrial fibrillation, which could have been why the arrhythmia was not apparent on the electrocardiogram. Thus, although the appropriate preoperative diagnostics were performed according to current oncologic and anesthesiology guidelines, the potentially lethal cardiac condition of the patient could not be detected.

Whipple's disease: misdiagnosed as sarcoidosis with further tricuspid valve endocarditis and pulmonary embolism - a case report.

GH Whipple described a 36-year-old physician in 1907 with gradual loss of weight and strength, stools consisting chiefly of neutral fat and fatty acids, indefinite abdominal signs and a peculiar multiple arthritis. The patient died of this progressive illness. Whipple called it intestinal lipodystrophy since he observed accumulation of large masses of neutral fats and fatty acids in the lymph spaces. It was renamed Whipple's disease in 1949. An infectious aetiology was suspected as early as Whipple's initial report. However, successful treatment with antibiotics was not reported until 1952, which resulted in dramatic clinical responses. The cause is now known to be Tropheryma whipplei. Light and electron microscopy of infected tissue identified a gram-positive, non-acid-fast, periodic acid-Schiff (PAS) positive bacillus with a characteristic trilamellar plasma membrane resembling that of gram-negative bacteria. Whipple's disease is extremely rare. It is a systemic infectious disorder affecting mostly middle-aged white men. The clinical presentation is often non-specific, which may make its diagnosis difficult. The four cardinal clinical manifestations are arthralgias, weight loss, diarrhoea and abdominal pain. The frequently vague articular symptoms can precede the diagnosis of Whipple's disease by an average of 6-8 years. Lymph nodes and other tissues may present diagnostic problems, since the changes in routinely stained sections may mimic those of sarcoidosis. The detection of PAS-positive histiocytes in the small intestine remains the mainstay of the diagnosis, although Whipple's disease without gastrointestinal involvement is described. We illustrate a case in which, retrospectively, the clinical presentation would have been typical for Whipple's disease. However, the clinical presentation and the histological examinations of lymph nodes, liver biopsies and ascites initially were misinterpreted as sarcoidosis with consecutive immunosuppressive therapy and progressive worsening of the patient's health presenting at least as sepsis with endocarditis.

Concomitant manifestation of pyoderma gangrenosum and colorectal carcinoma.

Pyoderma gangrenosum is an ulcerative skin disease of unknown origin and is commonly associated with inflammatory bowel disease, arthritis or lymphoproliferative disorders. Only sporadic cases of pyoderma gangrenosum in combination with malignant disease of the gastrointestinal tract have been reported until now. We report on a 53-year-old patient who suffered injury to the right scapula while gardening. Initially, the patient had only a superficial wound of the upper skin but in the subsequent weeks the lesion developed into an ulcerative defect and pyoderma gangrenosum was diagnosed. Laboratory test results, ultrasound of the abdomen and computed tomography of the chest and abdomen were normal. Immunosuppressive therapy with prednisolone and azathioprine was initiated. Four months later the patient was admitted to the gastroenterology department for further examination because of chronic fatigue, subfebrile temperature and a positive fecal occult blood test. Colonoscopy showed a semicircular carcinoma of the sigmoid colon measuring 3-4 cm. A left-sided hemicolectomy was performed. Microscopic examination revealed an adenocarcinoma (T3, N2, G3) and consequently the patient was given adjuvant chemotherapy. Nine months later the patient was asymptomatic and the pyoderma gangrenosum had recovered. Pyoderma gangrenosum is not only associated with inflammatory bowel disease or lymphoproliferative disorders. This case report demonstrates that colorectal carcinoma must also be considered as a possible differential diagnosis. The fast and complete remission of pyoderma gangrenosum following surgical treatment of colorectal carcinoma emphasizes a causal relationship.