Clinical Case Summary
Woman, 51 years old, farmer, past history of pelviperitonitis when 46 years old and family history of gastric and breast neoplasm.
She was admitted to the Emergency Department for abdominal pain for 2 years and 4 months of diarrhoea (without blood or mucus), anorexia, weight loss (12%), arthralgias and asthenia.
Analysis results: Hb 8.2mg/dL, leukocytosis 11200x103/µL, thrombocytosis 63131x103/µL, abnormal liver tests, CRP 6mg/dL, iron deficiency and hypoalbuminemia (2.5g/dL).
Abdomen-pelvic CT and MRI revealed cystadenocarcinoma of the right ovary with bilateral supraclavicular, axillary and inguinal adenopathy.
After observation by Gynaecology, she was referred to Palliative Care and for evaluation in a tertiary hospital. There, after a multidisciplinary discussion, an etiological study of diarrhoea was carried out, and laboratory evaluation was negative.
The imaging exams were then re-assessed: the presence and morphology of the adenopathy, the mesenteric lymph nodes conglomerate and jejunal thickening suggested the diagnosis of Whipple's disease.
An enteroscopy was also performed: duodenum and proximal jejunum’s mucosa was hyperaemic, oedematous and friable. Biopsies showed expansion of the lamina propria by lymphangiectasias, fat droplets and dense and multifocal aggregates of foamy macrophages with positive PAS-D (periodic acid-schiff with diastase) cytoplasmic granules, Ziehl-Neelsen negative.
A malignant ovarian tumour was excluded: the multiloculated cystic tumour of the right ovary was actually a cystadenofibroma – a diagnosis additionally confirmed by suprapubic and transvaginal ultrasound.
After treatment with intravenous ceftriaxone, the patient showed clinical improvement with resolution of pain, normal intestinal transit and recovery of nutritional status. Eight months after, she maintains antibiotic therapy, with no evidence of recurrence.
This case highlights the need for a low suspicion threshold for diagnosing this rare and benign disease.
References
Boumaza A, Ben Azzouz E, Arrindell J, Lepidi H, Mezouar S, Desnues B. Whipple's disease and Tropheryma whipplei infections: from bench to bedside. Lancet Infect Dis. 2022 Oct;22(10):e280-e291. doi: 10.1016/S1473-3099(22)00128-1. Epub 2022 Apr 12. PMID: 35427488.