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DOI: 10.1055/s-2008-1027628
© Georg Thieme Verlag KG Stuttgart · New York
Splenic Rupture in Systemic Amyloidosis – Case Presentation and Review of the Literature
Milzruptur bei systemischer Infiltrationskrankheit – ein Fallbericht und Übersicht der LiteraturPublication History
manuscript received: 6.12.2007
manuscript accepted: 17.6.2008
Publication Date:
11 March 2009 (online)

Zusammenfassung
Einleitung: Die Milzruptur und Blutung in den Bauchraum ist eine äußerst ungewöhnliche und seltene Komplikation einer internistischen Krankheit. Mögliche Ursachen für eine solche Komplikation sind Blut-, Entzündungs- oder Infiltrationskrankheiten, welche die Milz affizieren. Die Milzvergrößerung ist ein Faktor, der das Risiko einer Milzruptur signifikant erhöht. Fallbericht: Die Autoren stellen einen 52-jährigen Patienten mit festgestellter systemischer Amyloidose vor. Zuvor wurde er wegen einer undefinierten Leberkrankheit und Anämie behandelt, die bei der Behandlung pathologischer Wirbelbrüche festgestellt wurden. Leider willigte er während der ersten Behandlung in eine Leberbiopsie zur Bestimmung der Ätiologie der Krankheit nicht ein. Die systemische Infiltrationskrankheit wurde posthum bestimmt; die Milz sowie andere Organe waren betroffen. Es werden jene Formen der Amyloidose beschrieben, welche gewöhnlich einen ungünstigen Krankheitsverlauf bedeuten. Schlussfolgerung: Es wird ein Patient mit einer seltenen Form von Amyloidose und einer tödlichen Komplikation, Milzruptur, vorgestellt.
Abstract
Introduction: Splenic rupture and hemorrhage into the abdominal cavity is an extremely unusual and rare complication of internal disease. Hemopathies, inflammatory or infiltrative diseases affecting the spleen are possible causes for such a complication. Splenomegaly is a factor, which may significantly increase the risk of rupture. Case Report: The authors present the case of a 52-year-old patient with established systemic amyloidosis. In the past he had received treatment for undefined hepatic disease and anemia, which was established during the treatment of pathological vertebral fractures. Unfortunately, during the initial treatment he did not consent to a liver biopsy, which would have determined the etiology of the disease. Systemic infiltrative disease, affecting the spleen and other organs, was confirmed posthumously. Described are those forms of amyloidosis which usually represent an unfavorable course of the disease. Conclusion: Presented is a patient with a rare form of primary amyloidosis and fatal complication, spleenic rupture.
Schlüsselwörter
Milz - Ruptur - Infiltrationskrankheit - Leber - Amyloidose - Komplikationen
Key words
spleen - rupture - infiltrative diseases - liver - amyloidosis - complications
References
- 1
Autry J R, Weitzner S.
Hemangiosarcoma of spleen with spontaneous rupture.
Cancer.
1975;
35
534-539
Reference Ris Wihthout Link
- 2
Andrews D F, Hernandez R, Grafton W. et al .
Pathologic rupture of the spleen in non-Hodgkin’s lymphoma.
Arch Intern Med.
1980;
140
119-120
Reference Ris Wihthout Link
- 3
Von der Walde J, Mashiah A, Berrebi A.
Spontaneous rupture of the spleen in hairy cell leukemia.
Clin Oncol.
1981;
7
241-244
Reference Ris Wihthout Link
- 4
Okazaki K, Moriyasu F, Shiomura T. et al .
Spontaneous rupture of the spleen and liver in amyloidosis – a case report and review
of the literature.
Gastroenterol Jpn.
1986;
21
518-524
Reference Ris Wihthout Link
- 5
Simansky D A, Schiby G, Dreznik Z. et al .
Rapid progressive dissemination of hemangiosarcoma of the spleen following spontaneous
rupture.
World J Surg.
1986;
10
142-145
Reference Ris Wihthout Link
- 6
Gertz M A, Kyle R A.
Hepatic amyloidosis, primary (AL), immunoglobulin light chain: the natural history
in 80 patients.
Am J Med.
1988;
85
73-80
Reference Ris Wihthout Link
- 7
Gertz M A, Kyle R A.
Primary systemic amyloidosis – a diagnostic primer.
Mayo Clin Proc.
1989;
64
1505-1519
Reference Ris Wihthout Link
- 8
Hawkins P N, Lavender J P, Pepys M B.
Evaluation of systemic amyloidosis by scintigraphy with 123 I-labeled serum amyloid
P component.
N Engl J Med.
1990;
323
508-513
Reference Ris Wihthout Link
- 9
Patel S A, al-Haddadin D, Schopp J. et al .
Gastrointestinal manifestations of amyloidosis: a case of diverticular perforation.
Am J Gastroenterol.
1993;
88
578-582
Reference Ris Wihthout Link
- 10
Serra L, Poppi M C, Criscuolo M. et al .
Primary systemic amyloidosis with giant hepatomegaly and portal hypertension: a case
report and a review of the literature.
Ital J Gastroenterol.
1993;
25
435-438
Reference Ris Wihthout Link
- 11
Herrera G A, Sanders P W, Reddy B V. et al .
Ultrastructural immunolabeling: a unique diagnostic tool in monoclonal light chain-related
renal diseases.
Ultrastruct Pathol.
1994;
18
401-416
Reference Ris Wihthout Link
- 12
Gertz M A, Kyle R A.
Amyloidosis: prognosis and treatment.
Semin Arthritis Rheum.
1994;
24
124-138
Reference Ris Wihthout Link
- 13
Pascali E.
Diagnosis and treatment of primary amyloidosis.
Crit Rev Oncol Hematol.
1995;
19
149-181
Reference Ris Wihthout Link
- 14
Skinner M, Anderson J J, Simms R. et al .
Treatment of 100 patients with primary amyloidosis: A randomized trial of melphalan,
prednisone, and colchicine versus colchicine alone.
Am J Med.
1996;
100
290-298
Reference Ris Wihthout Link
- 15
Halm U, Berr F, Eggers E. et al .
Primary amyloidosis of the gastrointestinal tract and liver – two case reports.
Z Gastroenterol.
1997;
35
131-137
Reference Ris Wihthout Link
- 16
Kyle R A, Gertz M A, Greipp P R. et al .
A trial of three regimens for primary amyloidosis: Colchicine alone, melphalan and
prednisone, and melphalan, prednisone, and colchicine.
N Engl J Med.
1997;
336
1202-1207
Reference Ris Wihthout Link
- 17
Goss J A, Stribling R, Martin P.
Adult liver transplantation for metabolic liver disease.
Clin Liver Dis.
1998;
2
187-210
Reference Ris Wihthout Link
- 18
Kyle R A, Gertz M A, Greipp P R. et al .
Long-term survival (10 years or more) in 30 patients with primary amyloidosis.
Blood.
1999;
93
1062-1066
Reference Ris Wihthout Link
- 19
Sezer O, Niemoller K, Jakob C. et al .
Novel approaches to the treatment of primary amyloidosis.
Expert Opin Investig Drugs.
2000;
9
2343-2350
Reference Ris Wihthout Link
- 20
Anesi E, Palladini G, Perfetti V. et al .
Therapeutic advances demand accurate typing of amyloid deposits.
Am J Med.
2001;
111
243-244
Reference Ris Wihthout Link
- 21
Comenzo R L.
Primary systemic amyloidosis.
Curr Treat Options Oncol.
2000;
1
83-89
Reference Ris Wihthout Link
- 22
Gertz M A, Rajkumar S V.
Primary systemic amyloidosis.
Curr Treat Options Oncol.
2002;
3
261-271
Reference Ris Wihthout Link
- 23
Sanchorawala V, Wright D G, Seldin D C. et al .
Low dose continuous oral melphalan for the treatment of primary systemic (AL) amyloidosis.
Br J Haematol.
2002;
117
886-889
Reference Ris Wihthout Link
- 24
Bernstein C N.
Treatment of the extraintestinal manifestations of inflammatory bowel disease.
Curr Gastroenterol Rep.
2002;
4
513-516
Reference Ris Wihthout Link
- 25
Gertz M A, Lacy M Q, Dispenzieri A. et al .
Stem cell transplantation for the management of primary systemic amyloidosis.
Am J Med.
2002;
113
549-555
Reference Ris Wihthout Link
- 26
Park M A, Mueller P S, Kyle R A. et al .
Primary (AI) hepatic amyloidosis: clinical features and natural history in 98 patients.
Medicine (Baltimore).
2003;
82
291-298
Reference Ris Wihthout Link
- 27
Oran B, Wright D G, Seldin D C. et al .
Spontaneous rupture of the spleen in AL amyloidosis.
Am J Hematol.
2003;
74
131-135
Reference Ris Wihthout Link
- 28
Nilsson M R.
Techniques to study amyloid fibril formation in vitro.
Methods.
2004;
34
151-160
Reference Ris Wihthout Link
- 29
Solomon A, Weiss D T, Wall J S.
Immunotherapy in systemic primary (AL) amyloidosis using amyloid-reactive monoclonal
antibodies.
Cancer Biother Radiopharm.
2003;
18
853-860
Reference Ris Wihthout Link
- 30
Palladini G, Perfetti V, Obici L. et al .
Association of melphalan and high-dose dexamethasone is effective and well tolerated
in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation.
Blood.
2004;
103
2936-2938
Reference Ris Wihthout Link
- 31
Buxbaum J N.
The systemic amyloidoses.
Curr Opin Rheumatol.
2004;
16
67-75
Reference Ris Wihthout Link
- 32
Ekart R, Bevc S, Dajcman D. et al .
Sekundarna amiloidoza ob Crohnovi bolezni ter nefroticˇni sindrom: prikaz primera.
Zdrav Vestn.
2004;
73
177-179
Reference Ris Wihthout Link
- 33
Leung N, Dispenzieri A, Fervenza F C. et al .
Renal response after high-dose melphalan and stem cell transplantation is a favorable
marker in patients with primary systemic amyloidosis.
Am J Kidney Dis.
2005;
46
270-277
Reference Ris Wihthout Link
- 34
Shah K B, Inoue Y, Mehra M R.
Amyloidosis and the heart: a comprehensive review.
Arch Intern Med.
2006;
166
1805-1813
Reference Ris Wihthout Link
- 35
Sirohi B, Powles R.
Epidemiology and outcomes research for MGUS, myeloma and amyloidosis.
Eur J Cancer.
2006;
42
1671-1683
Reference Ris Wihthout Link
- 36
Palladini G, Perfetti V, Merlini G.
Therapy and management of systemic AL (primary) amyloidosis.
Swiss Med Wkly.
2006;
136
715-720
Reference Ris Wihthout Link
- 37
Schiodt I, Duun E, Fischer T K. et al .
Fatal rupture of the spleen caused by infiltration of T-cell lymphoma.
Ann Hematol.
2000;
79
158-160
Reference Ris Wihthout Link
- 38
Debnath D, Valerio D.
Atraumatic rupture of the spleen in adults.
J R Coll Surg.
2002;
47
437-445
Reference Ris Wihthout Link
- 39
Biswas S, Keddington J, McClanathan J.
Large B-cell lymphoma presenting as acute abdominal pain and spontaneous splenic rupture.
A case report and review of relevant literature.
World J Em S.
2006;
1
35
Reference Ris Wihthout Link
- 40
Aydinli B, Ozturk G, Balik A A. et al .
Spontaneous rupture of the spleen in secondary amyloidosis: A patient with rheumatoid
arthritis.
Amyloid.
2006;
13
160-163
Reference Ris Wihthout Link
- 41
Goddard S L, Chesney A E.
Pathological splenic rupture: a rare complication of chronic myelomonocytic leukemia.
Am J Hematol.
2007;
82
405-408
Reference Ris Wihthout Link
Prof. Pavel Skok
Gastroenterology and Endoscopy, University Clinical Centre Maribor
Ljubljanska 5
2000 Maribor
Slovenia
Phone: ++ 3 86/2/23 26 82
Fax: ++ 3 86/2/3 31 23 93
Email: pavel.skok@ukc-mb.si
