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Thread: Whipple's Disease

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    Default Whipple's Disease

    Whipple's Disease

    Definition
    Infectious disorder caused by the bacteria Tropheryma whippelii leading to accumulation of macrophages in the lamina propria and intestinal lipodystrophy.

    Pathogenesis

    * Organism:
    o cultured from bacilliform bodies
    o named Tropheryma whippelii
    o identified by sequencing largest part of a bacterial 16S ribosomal DNA, followed by PCR amplification1,2
    * Not clear whether:
    o a specific disease caused by a unique bacterium capable of intracellular survival and replication, or
    o a common organism in a host with impaired immunity:3
    + supported by demonstration of:
    # abnormal phagocytic capacity
    # increased numbers of intraepithelial lymphocytes
    # decreased CD4+/CD8+ cell ratio
    # increased IgM/IgA ratio in plasma cells of lamina propria4

    Histopathology


    * Jejunal biopsy:
    o typical appearance
    o empty spaces5 (Fig. 1
    Jejunal mucosa in Whipple's disease. The lamina propria is packed with histiocytes and empty round spaces. The latter contained lipid material that has been extracted during tissue processing.

    Fig. 1: Jejunal mucosa in Whipple's disease. The lamina propria is packed with histiocytes and empty round spaces. The latter contained lipid material that has been extracted during tissue processing.
    ):
    + may be surrounded by giant cells
    + also in mesenteric lymph nodes
    + contain neutral fat
    o large macrophages:
    + crowd lamina propria, distorting villi
    + occasionally restricted to submucosa6
    + also identified in:
    # rest of the alimentary tract
    # regional and peripheral lymph nodes (Figs 2 and 3
    Outer aspect of mesenteric lymph nodes massively involved by Whipple's disease.

    Fig. 2: Outer aspect of mesenteric lymph nodes massively involved by Whipple's disease.
    Cut surface of mesenteric lymph nodes massively involved by Whipple's disease.

    Fig. 3: Cut surface of mesenteric lymph nodes massively involved by Whipple's disease.
    )
    # heart
    # lung
    # liver
    # spleen
    # adrenal glands
    # nervous system
    # other sites7,8
    * Diastase-resistant, PAS-positive material:
    o large amounts in cytoplasm of macrophages
    o due to presence of bacilliform bodies
    o also:
    + in lumen
    + between epithelial cells
    o well demonstrated by:
    + electron microscopy9,10 (Fig. 4
    Electron micrograph of macrophage in submucosa of small intestine in Whipple's disease. Cytoplasm contains membrane-limited sacs (S) that are filled with dense spherical and rod-shaped bodies (b) intermixed with fine membranous profiles. These rods have been shown to be the organisms responsible for the disease. (15,000)

    Fig. 4: Electron micrograph of macrophage in submucosa of small intestine in Whipple's disease. Cytoplasm contains membrane-limited sacs (S) that are filled with dense spherical and rod-shaped bodies (b) intermixed with fine membranous profiles. These rods have been shown to be the organisms responsible for the disease. (15,000)
    )
    + immunohistochemistry11,12
    o some successful attempts at culture13
    * Electron microscopy:
    o useful in:
    + demonstrating diagnostic microorganisms
    + evaluating adequacy of therapy
    + identifying early reactivation11,14,15

    Special Stains and Immunohistochemistry

    * Immunohistochemistry:
    o useful in:
    + demonstrating diagnostic microorganisms
    + evaluating adequacy of therapy
    + identifying early reactivation11,14,15

    Diagnosis

    * Small bowel biopsy:
    o best method
    * Fine needle aspiration:16
    o suggests diagnosis
    o similar change (but not PAS-positive macrophages) common in normal intra-abdominal lymph nodes:
    + probably secondary to ingestion of mineral oil17
    * Biopsy of peripheral lymph nodes:
    o can be suggestive if typical macrophages
    o not pathognomonic18
    o immunohistochemistry and PCR can confirm diagnosis19,20
    * Rectal biopsy:
    o interpretation difficult because mucin-containing macrophages (muciphages) in lamina propria of 10% of normal individuals resemble histiocytes of Whipple's disease at light microscopic level21

    Management

    * Antibiotic therapy:
    o good response
    o relapses common23,24

    References

    1 Dobbins WO. The diagnosis of Whipple's disease (editorial). N Engl J Med. 1995;332:390392.

    2 Relman DA, Schmidt TM, MacDermott RP, Falkow S. Identification of the uncultured bacillus of Whipple's disease. N Engl J Med. 1992;327:293301.

    3 Dobbins WO. Whipple's disease. Springfield, Ill: Charles C Thomas; 1987;.

    4 Ectors N, Geboes K, De Vos R, Heidbuchel H, Rutgeerts P, Desmet V, et al. Whipple's disease. A histological, immunocytochemical and electron microscopic study of the immune response in the small intestinal mucosa. Histopathology. 1992;21:112.

    5 Von Herbay A, Maiwald M, Ditton HJ, Otto HF. Histology of intestinal Whipple's disease revisited. A study of 48 patients. Virchows Arch. 1997;429:335343.

    6 Kuhajda FP, Belitsos NJ, Keren DF, Hutchins GM. A submucosal variant of Whipple's disease. Gastroenterology. 1982;82:4650.

    7 Enzinger FM, Helwig EB. Whipple's disease. A review of the literature and report of fifteen patients. Virchows Arch [A]. 1963;336:238269.

    8 Gerard A, Sarrot-Reynauld F, Liozon E, Cathebras P, Besson G, Robin C, et al. Neurologic presentation of Whipple disease: report of 12 cases and review of the literature. Medicine (Baltimore). 2002;81:443457.

    9 Bhagavan BS, Hofkin GA, Cochran BA. Whipple's disease. Morphologic and immunofluorescence characterization of bacterial antigens. Hum Pathol. 1981;12:930936.

    10 Marsh MN editors. Immunopathology of the small intestine. New York: John Wiley & Sons; 1987.

    11 Baisden BL, Lepidi H, Raoult D, Argani P, Yardley JH, Dumler JS. Diagnosis of Whipple disease by immunohistochemical analysis. A sensitive and specific method for the detection of Tropheryma whipplei (the Whipple bacillus) in paraffin-embedded tissue. Am J Clin Pathol. 2002;118:742748.

    12 Lepidi H, Fenollar F, Gerolami R, Mege J-L, Bonzi M-F, Chappuis M, et al. Whipple's disease: immunospecific and quantitative immunohistochemical study of intestinal biopsy specimens. Hum Pathol. 2003;34:589596.

    13 Raoult D, Birg ML, La Scola B, Fournier PE, Enea M, Lepidi H, et al. Cultivation of the bacillus of Whipple's disease. N Engl J Med. 2000;342:620625.

    14 Denholm RB, Mills PR, More IAR. Electron microscopy in the long-term follow-up of Whipple's disease. Effect of antibiotics. Am J Surg Pathol. 1981;5:507516.

    15 Morningstar WA. Whipple's disease. An example of the value of the electron microscope in diagnosis, follow-up, and correlation of pathologic process. Hum Pathol. 1975;6:443454.

    16 Saleh H, Williams TM, Mimda JM, Gupta PK. Whipple's disease involving the mesenteric lymph nodes diagnosed by fine-needle aspiration. Diagn Cytopathol. 1992;8:177180.

    17 Boitnott JK, Margolis S. Mineral oil in human tissues. Part II. Oil droplets in lymph nodes of the porta hepatis. Bull Hopkins Hosp. 1966;118:414422.

    18 Maizell H, Ruffin JM, Dobbins WO. Whipple's disease. A review of 19 patients from one hospital and a review of the literature since 1950. Medicine Baltimore. 1970;49:175205.

    19 Alkan S, Beals TF, Schnitzer B. Primary diagnosis of whipple disease manifesting as lymphadenopathy. Use of polymerase chain reaction for detection of Tropheryma whippelii. Am J Clin Pathol. 2001;116:898904.

    20 Lepidi H, Costedoat N, Piette JC, Harle JR, Raoult D. Immunohistological detection of Tropheryma whipplei (Whipple bacillus) in lymph nodes. Am J Med. 2002;113:334336.

    21 Ekuan JH, Hill RB. Colonic histiocytosis. Clinical and pathological evaluation. Gastroenterology. 1968;55:619625.

    22 Maliha GM, Hepps KS, Maia DM, Gentry KR, Fraire AE, Goodgame RW. Whipple's disease can mimic chronic AIDS enteropathy. Am J Gastroenterol. 1991;86:7981.

    23 Comer GM, Brandt LJ, Abissi CJ. Whipple's disease. A review. Am J Gastroenterol. 1983;78:107114.

    24 Keinath RD, Merrell DE, Vlietstra R, Dobbins WO. Antibiotic treatment and relapse in Whipple's disease. Long-term followup of 88 patients. Gastroenterology. 1985;88:18671873.


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    Fig. 1: Jejunal mucosa in Whipple's disease. The lamina propria is packed with histiocytes and empty round spaces. The latter contained lipid material that has been extracted during tissue processing.



    Fig. 2: Outer aspect of mesenteric lymph nodes massively involved by Whipple's disease.


    Fig. 3: Cut surface of mesenteric lymph nodes massively involved by Whipple's disease.


    Fig. 4: Electron micrograph of macrophage in submucosa of small intestine in Whipple's disease. Cytoplasm contains membrane-limited sacs (S) that are filled with dense spherical and rod-shaped bodies (b) intermixed with fine membranous profiles. These rods have been shown to be the organisms responsible for the disease. (15,000)


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    If U Think Ur Life Getting Necrosed,Ur Dreames Inflammed,Ur Thoughts Thrombosed,Then Try This Out.Spread D Neoplasia Of Love Around U.
    V.H.SHAH

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