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Path Quiz Case-88 :
Diagnosis-
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Image Link4.Whipple's disease is a rare chronic, relapsing, and multisystem disease caused by bacterial infection. Causative organism: Gram-positive organism named Tropheryma whipplei. Age:
Patients in fourth
and fifth decade of life. Gross features:
The
affected bowel is usually edematous with yellow plaquelike lesions and
villiferous mucosa. Affected lymph nodes appear yellow with a spongy cut
surface. Other affected organs, such as lungs and heart, may show plaques
and edema Small intestinal mucosa filled with distended macrophages in the lamina propria. The macrophages contain diastase resistant PAS positive granules.
Image Link5 Microscopic findings in the CNS include PAS-positive intracellular and extracellular organisms surrounded by reactive astrocytes. The organisms may also violate the subarachnoid spaces and lead to the death of neurons, vacuolization, and demyelination. Diagnosis : The diagnosis is usually established by small intestinal biopsy, which shows the pathognomonic periodic acid Schiff-positive infiltrates in the lamina propria. Image5 ; Image6. The rod shaped bacilli may be demonstrated by electron microscopy. Image7 ; Image8 . Polymerase chain reaction is now available to aid in the diagnosis of WD, and this organism has been identified by polymerase chain reaction in many body fluids, including cerebrospinal fluid, aqueous humor, and synovial fluid. Detection in
cerebrospinal fluid and peripheral blood is less consistent than in
tissue.
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April
2008
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