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DOI: 10.1055/s-2001-16583
Morbus Whipple - eine seltene Systemerkrankung
Aktueller Stand der Diagnostik und TherapieWhipple's disease, a rare systemic disease: current diagnosis and treatmentPublication History
Publication Date:
23 August 2001 (online)

George H. Whipple (1878 - 1976) publizierte erstmals 1907 die Kasuistik eines 36-jährigen Arztes, der nach 5-jährigem Leiden an einer schleichend verlaufenden Erkrankung mit Polyarthritis, Bronchitis, Gewichtsverlust und chronischer Diarrhoe verstarb. Autoptisch fand sich eine Infiltration der Lamina propria des Dünndarms mit mononukleären Zellen, deren Zytoplasma durch eine große Zahl stäbchenförmiger Organismen schaumig wirkte. Die Ansammlung von Fett im intestinalen lymphatischen System führte zur Annahme einer Fettstoffwechselstörung als Ursache der Erkrankung und zur Prägung des Begriffs »Intestinale Lipodystrophie« [51]. 1949 entdeckte Black-Schaffer, dass sich die auffälligen Makrophagen mit Perjodsäure-Schiff (PAS) - Reagenz tiefrot anfärben ließen, - eine Methode, die bis heute bei der histologischen Sicherung der Diagnose angewandt wird [3]. Der erste Bericht einer erfolgreichen Therapie mit Antibiotika (Chloramphenicol) stammt aus dem Jahre 1952 [35]. Nach dem elektronenmikroskopischen Nachweis des Whipple-Bakteriums 1961 erfolgte 1992 seine molekularbiologische Charakterisierung und genetische Zuordnung zur Gattung der Aktinomyzeten unter dem Namen Tropheryma whipplei (bisher Tropheryma whippelii) [4] [39] [53].
Literatur
- 1
Bayless T M, Knox D L.
Whipple’s
disease: A multisystemic infection.
N Engl J Med.
1979;
300
920-921
Reference Ris Wihthout Link
- 2
Bjerknes R, Ødegaard S, Bjerkvig R, Børkje B, Lærum O D.
Whipple’s
disease. Demonstration of a persisting monocyte and makrophage dysfunction.
Scand
J Gastroenterol.
1988;
23
611-619
Reference Ris Wihthout Link
- 3
Black-Schaffer B.
Tinctoral
demonstration of glycoprotein in Whipple’s disease.
Proc
Soc Exp Biol Med.
1949;
72
225
Reference Ris Wihthout Link
- 4
Chears W C, Ashworth C T.
Electron
microscopic study of the intestinal mucosa in Whipple’s
disease.
Gastroenterology.
1991;
41
129-138
Reference Ris Wihthout Link
- 5
Cooper G S, Blades E W, Remler B F, Salata R A, Bennert K W, Jacobs G H.
Central nervous
system Whipple’s disease: Relapse during therapy with trimethoprim-sulfametoxazole
and remission with cefixime.
Gastroenterology.
1994;
106
782-786
Reference Ris Wihthout Link
- 6
Dobbins W O
III.
HLA-Antigens in Whipple’s disease.
Arthritis
Rheum.
1987;
30
102-105
Reference Ris Wihthout Link
- 7
Dobbins W O
III.
Is there an immune deficit in Whipple’s
disease?.
Dig Dis Sci.
1981;
26
247-252
Reference Ris Wihthout Link
- 8 Dobbins W O
III. Whipple’s disease. Lippinicott.
Philadelphia In Yamada T. (Ed): Textbook of Gastroenterology 1995: 1634-1640
Reference Ris Wihthout Link
- 9
Dobbins W O
III.
Whipple’s disease.
Mayo
Clin Proc.
1988;
63
623-624
Reference Ris Wihthout Link
- 10
Durand D V, Lecomte C, Cathébras P, Rousset H, Godeau P.
Whipple
Disease.
Medicine (Baltimore).
1997;
76
170-184
Reference Ris Wihthout Link
- 11
Enzinger F M, Helwig E B.
Whipple’s
disease - a review of the literature and report of fifteen
patients.
Virchows Arch.
1963;
336
238-269
Reference Ris Wihthout Link
- 12
Feldmann M.
Southern
Internal Medicine Conference: Whipple’s diesease.
Am
J Med Sci.
1986;
291
56-67
Reference Ris Wihthout Link
- 13 Fenoglio-Preiser C M, Noffsinger A E, Stemmermann G N, Lantz P E, Listrom M B. Gastrointestinal
pathology: an atlas and text,. Lippincott-RavenPublishers 1999: 382
Reference Ris Wihthout Link
- 14
Feuerle G E, Dörken B, Schöpf E, Lenhard V.
HLA B27 and defects
in the T-cell system in Whipple`s disease.
Eur
J Clin Invest.
1979;
9
385-389
Reference Ris Wihthout Link
- 15
Fleming J L, Wiesner R H, Shorter R G.
Whipple’s
disease: clinical, biochemical and histopathologic features and
assesment of treatment in 29 patients.
Moyo Clin Proc.
1988;
63
539-551
Reference Ris Wihthout Link
- 16
Girardin M FSM, Zafrani E S, Chaumette M T, Delchier J C, Métreau J M, Dhumeaux D.
Hepatic granulomas
in Whipple’s disease.
Gastroenterology.
1984;
86
753-756
Reference Ris Wihthout Link
- 17
Graham P M, Kelly C R, Booth J A.
Ultrasonic
appearance of abdominal lymphnodes in a case of Whipple’s
disease.
J Clin Ultrasound.
1983;
11
388-390
Reference Ris Wihthout Link
- 18
Gubler J GH, Kuster M, Dutly F. et al .
Whipple endocarditis without overt gastrointstinal
disease: report of four cases.
Ann Intern Med.
1999;
131
112-116
Reference Ris Wihthout Link
- 19
Hirsbrunner-Erni R, Altwegg M, Diener P A, Villiger P M.
Morbus Whipple
mit normaler Dünndarmhistologie: Rarität oder
Realität?.
Schweiz Med Wochenschr.
2000;
130
1820-1826
Reference Ris Wihthout Link
- 20
Kaufmann M, Risti B, Fried M.
Die
Whipple-Erkrankung.
Internist.
1996;
37
895-902
Reference Ris Wihthout Link
- 21
Keinath R D, Merrel D E, Vlietstra R, Dobbins W O III.
Antibiotic
treatment and relaps in Whipple’s Disease: long-term follow-up
of 88 patients.
Gastroenterology.
1985;
88
1867-1873
Reference Ris Wihthout Link
- 22
Kelly J J
III, Weisinger B B.
The
arthritis of Whipple’s disease.
Arthritis Rheum.
1963;
6
615-631
Reference Ris Wihthout Link
- 23
Kwee D, Kwee J, Fields P, King L E.
Subcutaneous
Whipple’s disease.
J Am Acad Dermatol.
1987;
16
188-190
Reference Ris Wihthout Link
- 24
Louie J S, Liebling M R.
The polymerase
chain reaction in ifectious and postinfectious arthritis. A review.
Rheum
Dis Clin North Am.
1998;
24
227-236
Reference Ris Wihthout Link
- 25
Louis E D, Lynch T, Kaufmann P, Fahn S, Odel J.
Diagnostic
guide-lines in central nervous system Whipple’s disease.
Ann
Neurol.
1996;
40
561-568
Reference Ris Wihthout Link
- 26
Lowsky R, Archer G L, Fyles G. et al .
Brief report: diagnosis of Whipple’s disease
by molecular analysis of peripheral blood.
N Engl J Med.
1994;
331
1343-1346
Reference Ris Wihthout Link
- 27
Maiwald M, Schumacher F, Ditton H J, von Herbay A.
Environmental occurence
of the Whipple’s disease bacterium (Tropheryma whippelii).
Appl
Environ Microbiol.
1998;
64
760-762
Reference Ris Wihthout Link
- 28
Maiwald M, von Herbay A, Persing D H. et al .
Tropheryma whippelii DNA is rare in the
intestinal mucosa of patients without other evidence of whipple
disease.
Ann Intern Med.
2001;
134
115-119
Reference Ris Wihthout Link
- 29
Maizel H, Ruffin J M, Dobbins W O
III.
Whipple’s disease: a review of 19 patients
from one hospital and a review of the literature since 1950.
Medicine
(Baltimore).
1970;
49
175-205
Reference Ris Wihthout Link
- 30
Marcial M A, Villafana M.
Whipple’s
disease with oesophageal and colonic involvement: endoscopic and
histopathologic findings.
Gastrointest Endosc.
1997;
46
263-266
Reference Ris Wihthout Link
- 31
Marth T, Neurath M, Cuccherini B A, Strober W.
Defects
of monocyte interleukin 12 production and humoral immunity in Whipple’s
disease.
Gastroenterology.
1997;
113
442-449
Reference Ris Wihthout Link
- 32
Martin F F, Vilseck J, Dobbins W O
III, Buckley C E III, Tyor M P.
Immunological
alterations in patients with treated Whipple’s disease.
Gastroenterology.
1972;
63
6-18
Reference Ris Wihthout Link
- 33
McAllister H A, Fenolglio J J.
Cardiac
involvement in Whipple’s disease.
Circulation.
1975;
52
152-156
Reference Ris Wihthout Link
- 34
Misra P S, Lebwohl P, Laufer H.
Hepatic
and appendiceal Whipple’s disease with negative jejunal
biopsies.
Am J Gastroenterol.
1981;
75
302-306
Reference Ris Wihthout Link
- 35
Paulley J W.
A
case of Whipple’s disease (intestinal Lipodystrophy).
Gastroenterology.
1952;
22
128-133
Reference Ris Wihthout Link
- 36
Petrides P E, Müller-Höcker J, Fredricks D N, Relman D A.
PCR analysis
of T. whippelii DNA in a case of Whipple’s disease: Effect
of antibiotics and correlation with histology.
Am J Gastroenterol.
1998;
93
1579-1582
Reference Ris Wihthout Link
- 37
Ramzan N N Jr., Loftus E, Burgart L J. et al .
Diagnosis and monitoring of Whipple’s
disease by polymerase chain reaction.
Ann Intern Med.
1997;
126
520-527
Reference Ris Wihthout Link
- 38
Raoult D, Birg M L, La Scola B. et al .
Cultivation of bacillus of Whipple’s disease.
N
Engl J Med.
2000;
342
620-625
Reference Ris Wihthout Link
- 39
Relman D A, Schmidt T A, MacDermott R P, Falkow S.
Identification of the uncultured
bacillus of Whipple’s disease.
N Engl J Med.
1992;
327
293-301
Reference Ris Wihthout Link
- 40
Rickman L S, Freeman W R, Green W R. et al .
Uveitis caused by Tropheryma whippelii
(Whipple’s bacillus).
N Engl J Med.
1995;
332
363-366
Reference Ris Wihthout Link
- 41
Scheib J S, Quinet R J.
Whipple’s
disease with axial and peripheral joint destruction.
South
Med J.
1990;
83
684-687
Reference Ris Wihthout Link
- 42
Schneider T, Stallmach A, von Herbay A, Marth T, Strober W, Zeitz M.
Treatment of refractory
Whipple’s disease with interferon-g.
Ann intern
Med.
1998;
129
875-877
Reference Ris Wihthout Link
- 43
Schnider P, Trattnig S, Kollegger H, Auff E.
MR of cerebral Whipple’s
disease.
Am J Neuroradiol.
1995;
16
1328-1329
Reference Ris Wihthout Link
- 44
Schnider P J, Reisinger E C, Gerschlager W. et al .
Long-term follow-up in cerebral Whipple
disease.
Eur J Gastroenterol Hepatol.
1996;
8
899-903
Reference Ris Wihthout Link
- 45
Sieracki J C.
Whipples
disease - observation on systemic involvement. Cytologic
observations.
Arch Pathol.
1958;
66
464-467
Reference Ris Wihthout Link
- 46
Singer R, von Herbay A, Willig F.
Erfolgreiche
Therapie eines zerebralen Morbus Whipple mit Rifampicin.
Med
Klin.
1995;
90
117-118
Reference Ris Wihthout Link
- 47
Singer R.
Diagnosis
and treatment of Whipple’s disease.
Drugs.
1998;
55
699-704
Reference Ris Wihthout Link
- 48
Valpolcelli N A, Salyer W R, Milligan F D, Bayless T M, Haedley J H.
The
endoscopic appearance of the duodenum in Whipple’s disease.
John
Hopkins Med J.
1976;
138
19-23
Reference Ris Wihthout Link
- 49
von Herbay A, Ditton H J, Schumacher F, Maiwald M.
Whipple’s disease: Staging
and monitoring by cytology and polymerase chain reaction analysis
of cerebrospinal fluid.
Gastroeneterol.
1997;
113
434-441
Reference Ris Wihthout Link
- 50
von Herbay A, Maiwald M, Ditton H J, Otto H F.
Histology of
intestinal Whipple’s disease revisited. A study of 48 patients.
Virchows
Arch.
1996;
429
335-343
Reference Ris Wihthout Link
- 51
Whipple G H.
A
hitherto undescribed disease characterized anatomically by deposits
of fat and fatty acids in the intestinal and mesenteric lymphatic
tissues.
John Hopkins Hosp Bull.
1907;
18
382-391
Reference Ris Wihthout Link
- 52
Wroe S J, Harding B, Youl B D, Shorvon S.
Whipple’s disease
confined to the CNS presenting with multiple intracerebral mass
lesions.
J Neurol Neurosurg Psychiatry.
1991;
54
989-992
Reference Ris Wihthout Link
- 53
Yardley J H, Hendrix T R.
Combined electron
and light microscopy in Whipple’s disease: demonstration
of ‘bacillary bodies’ in the intestine.
Bull
Johns Hopkins Hosp.
1961;
109
80-98
Reference Ris Wihthout Link
1 X Nachweis z. B. durch die »Arbeitsgruppe Tropheryma whippelii«, PD Dr. Axel von Herbay, Pathologisches Institut der Universität Heidelberg, Im Neuenheimer Feld 220, 69120 Heidelberg. http://www.whipplesdisease.net
Korrespondenz
Dr. Wolfgang Scharnke
Prof. Dr. Henryk Dancygier
Medizinische Klinik II, Klinikum Offenbach
Starkenburgring 66
63069 Offenbach
Phone: 069/8405-3971
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