Whipple’s disease infection surgical treatment: presentation of a rare case and literature review

The Whipple’ Disease (W.D.) is a very rare disease with an incidence of 1 per 1.000.000 inhabitants; it is a systemic infection that may mimic a wide spectrum of clinical disorders, which may have a fatal outcome and affects mainly male 40-50 years old. The infective agent is an actinomycete, Tropheryma Whipplei (T.W.) that was isolated 100 years after first description by Wipple, and identified in macrophages of mucosa of the small intestine by biopsy which is characterized by periodic acid-Schiff-positive, products of the inner membrane of his polysaccharide bacterial cell wall. The multisystemic clinical manifestations evolve rapidly towards an organic decay characterized by weight loss, malabsorption, diarrhea, polyathralgia, opthalmoplegia, neuro-psychiatric disorders and sometimes associated to endocarditis. Early antibiotic treatment with trimethoprim and sulfometathaxazole reduces the fatal evolution of the disease. The authors present a rare experience about a female subject in which the clinical gastrointestinal signs were preceded by neuro-psychiatric disorders, and evolved into obstruction and intestinal perforation which required an emergency surgery with temporary ileostomy, recanalized only after adequate medical treatment with a full dose of antibiotic and resolution of clinical disease for the high risks of fistulae for the edema and lymphadenopathy of mucosa. The diagnosis was histologically examined by intestinal biopsy performed during surgery, which showed PAS-positive histiocytes, while PRC polymerase RNA was negative, which confirms the high sensibility of PAS positive and low specificity of RNA polymerase for T.W.

HIV Infection Presenting as Haemophagocytic Syndrome

The authors describe a case of a 48-year-old man who presented with four weeks of fever, generalized malaise, weight loss, right upper quadrant abdominal pain and hepatosplenomegaly. He evolved with pancytopenia, bone marrow haemophagocytosis and hyperferritinaemia. Recent diagnosis of HIV infection, with the exclusion of other plausible causes, prompted the diagnosis of haemophagocytic syndrome (HPS) secondary to HIV. Despite intensive care support and initiation of antiretroviral therapy, the patient died. HPS diagnosis secondary to HIV alone demands the exclusion of all the other secondary causes. The best approach includes early diagnosis and specific treatment of the associated cause, whenever possible.