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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.
Clinical
presentation:
Presents with malabsorption, diarrhoea and weight loss. May also present
with lymphadenopathy, hyperpigmentation and polyarthritis.
Sites
affected:
Intestine, central nervous system, joints, lymphnodes ,lungs and heart.
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
Histologic
features: Image1
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Small intestinal mucosa filled with distended macrophages in the lamina propria. The macrophages contain diastase resistant PAS positive granules.
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Villi may be distorted. Inflammation is usually absent.
Mesenteric lymphnodes may be involved and may show lymphatic dilatation
indicating lymphatic obstruction.
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.
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The rod shaped
bacilli may be demonstrated by electron microscopy.
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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.
Differential
diagnosis:
Includes AIDS
enteropathy caused by Mycobacterium avium intracellulare
(Ziehl-Neelsen
staining
positive).
Patients respond well to antibiotic therapy, but relapses occur.
Whipple's disease: a rare
disease
revisited.Curr Gastroenterol
Rep. 2010 Aug;12(4):263-9.
Since the
original postmortem diagnosis of "intestinal lipodystrophy" by Dr.
George H. Whipple
in 1907, the complexities of
Whipple's disease
have been elucidated through case reports. Universally fatal prior to
the advent of antibiotics, Tropheryma whipplei is increasingly
recognized as an organism that can be treated only if the clinician
seeks to identify it.
Whipple's disease
is primarily a gastrointestinal
disease
manifesting as a malabsorption syndrome, and is detected through
endoscopy and intestinal biopsy. Nongastrointestinal manifestations of
the disease,
although less common, are reported and have aided in its recognition
as a multiorgan
disease entity. Because of its rarity, treatment
recommendations are currently based on observational studies and on
one recent prospective study, which outlined induction therapy
followed by several months of suppressive maintenance therapy to
prevent relapse, which is often characterized by neurologic symptoms.
Tropheryma whipplei
infection. Colonization, self-limiting infection and
Whipple's disease]Pathologe.
2011
Sep;32(5):362-70.
Whipple's disease
is a multisystemic infection caused by the ubiquitous bacterium
Tropheryma whipplei. Immunological host factors enable classical
Whipple's disease;
however, T. whipplei can be found in three other clinical conditions:
healthy colonization, self-limiting infections, and isolated
endocarditis. The genetic predisposition of the host rather than the
genotype of the bacterium influences the infection. Modern diagnostic
methods elucidate the many facets of
Whipple's disease.
In particular, isolated T. whipplei-induced infective endocarditis can
only be diagnosed after valve resection. The sole treatment of
Whipple's disease
evaluated prospectively comprises intravenous induction therapy with
ceftriaxone or meropenem, followed by continuation therapy with oral
TMP-SMX. In the case of Immune reconstitution inflammatory syndrome
(IRIS) or inflammatory lesions of the CNS in the setting of
Whipple's disease,
additional treatment with corticosteroids should be considered to
avoid severe tissue damage. |
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