By Aydinli, B; Ozturk, G; Balik, A A; Aslan, S; Erdogan, F
Keywords: Amyloidosis, spleen, spontaneous rupture, rheumatoid arthritis
Abstract
A 63-year-old man who had had a history of rheumatoid arthritis presented with shock and hemoperitoneum, without a history of trauma. An emergency laparatomy revealed hemoperitioneum and splenic rupture with massive bleeding. Splenectomy was performed. Histopathological examination of the spleen revealed amyloid deposition in the wall of the vessels. Rectal biopsy revealed amyloid deposition in mucosa that indicating amyloidosis was systemic. Histochemical studies showed that amyloid was secondary or AA.
Introduction
Amyloidosis describes a group of diseases characterized by the extracellular deposition of fibrillar proteins. The disorders may be primary, secondary, or hereditary, and the deposits may be localized or systemic [1]. In secondary or AA amyloidosis, the acute phase reactant protein serum amyloid A is deposited in tissues. Among the causes of AA amyloidosis are rheumatic diseases (Rheumatoid and Juvenile arthritis), idiopathic diseases (Crohn’s disease and ulcerative colitis), inherited diseases (familial Mediterranean fever), and infectious diseases (tuberculosis and leprosy) [1,2]. AA accumulates predominantly in small vessel walls and parenchymal organs such as spleen, liver and kidney in human and various animal species [3]. Peritoneal hemorrhage following splenic rupture is rare, but there is an increased risk of this complication in patients with underlying systemic amyloidosis. Involvement of the spleen is common and, although this is usually of no clinical significance, it is recognized such abnormal spleens may rupture spontaneously [4].
Case
A 63-year-old man presented with shock and features of hemoperitoneum. The patient had no trauma history. The patient had abdominal pain and diffuse tenderness. On admission, the patients blood pressure was 70/30 mmHg and heart rate 120/min. Laboratory test revealed hemoglobin 5.3 g/dL, leucocytes 17.6 10^sup 9^/L, platelets 332 10^sup 9^/L and his PT and PTT levels were 77.4% and 45.2 s, respectively, INR was 1.2. The patient had splenomegaly with subcapsullary hematoma and free intraperitoneal fluid, which was visible on an emergency abdominal, computed tomographic (CT) scan (Figure 1). On emergency laparatomy, several liters of old and fresh blood in the upper abdomen and bleeding from a laceration of the anterior surface of the spleen were present (Figure 2). The spleen was removed. The liver was a little enlarged, there was no evidence of any other intra-abdominal pathology, and no lymphadenopathy was seen in the abdomen suggesting a lymphoproliferative disorder. Tru- cut biopsy was performed from the enlarged liver during the laparatomy.
Histopathological examination of the spleen showed deposits of amyloid, mainly in the wall of the vessels (Figure 3), with green birefringence after staining with Congo red in polarized light. Pretreatment with potassium permanganate resulted in the loss of congophilia, indicating that the amyloidosis was secondary. On biopsy, the architecture of the liver was normal, but fatty changes and mild cholestasis were also present. Endoscopic rectal biopsy was performed, and mucosal amyloid deposition was observed with Congo red staining. The patient had a long rheumatoid arthritis (RA) history, but he had received medical therapy for RA for the last 2 years. Postoperative laboratory findings showed no deficiency of factor X. The echocardiographic evaluation was normal. The patient was vaccinated against pneumoccoci and hemophilus within first day after splenectomy and was placed on prophilactic benzyl-penicillin application for 6 mondis thereafter. The postoperative course was uneventful, and the patient was discharged from the hospital on the fourth postoperative day. The patient has had no any problems during 4-year follow-up.
Figure 1. Abdominal-computed tomographic (CT) scan reveals subcapsular hematoma and free intraperitoneal fluid (arrow).
Figure 2. Appearance of the lacerated spleen.
Figure 3. Congo-red staining showed deposits of amyloid, mainly in wall of the vessels of the red pulp of the spleen (white arrow).
Discussion
Amyloidosis is a functional disorder marked by unusual depositions of amyloid protein in various organs such as liver, spleen and kidney [5]. Progressive accumulation of amyloid compresses and replaces normal tissue, and it leads to organ dysfunction. Amyloidosis is usually seen in a systemic form, although 10-20% of cases can be localized [6]. Systemic amyloidosis is subclasified by a particular precursor protein into two major forms; primary amyloidosis (AL amyloidosis) and secondary amyloidosis (AA amyloidosis) [6]. We determined systemic amyloidosis with endoscopic rectal biopsy in our patient. Amyloid A (AA) amyloidosis is a secondary disorder induced by chronic infection or inflammation, such as osteomyelitis, tuberculosis, rheumatoid arthritis, or granulomatous ileitis (5). Secondary amyloidosis results from defective metabolism of the inflammatory acute-phase reactant precursor protein, serum amyloid A (SAA), whose concentration strongly correlates with inflammatory disease activity [7]. The prevalence of AA amyloidosis among persons effected by ankylosing spondiylitis, rheumatoid arthritis and Crohn’s disease ranges from 0.5 to 13%, [2]. Secondary amyloidosis is well recognized as a severe complication in the late stages of RA. Nevertheless it may develop, even in patients with a short history of RA, when the disease is active [8]. Our patient had a long RA history, but he had received treatment only for the last 2 years.
Splenic involvement is common in amyloidosis, but splenomegaly is seen only 4-13% of all patients. Amyloid deposition in the spleen can be at three major sites; the red pulp, the white pulp, and the blood vessels. Clinical manifestations of splenic involvement are rare and include pain, hyposplcnism, infarction, and rupture [9]. In the present case, amyloid deposition was seen around the blood vessels.
Spontaneous rupture of the spleen, which has been associated with a number of different underlying conditions, is not common but associated with high mortality. The incidence of spontaneous splenic rupture is unclear [10]. It has been suggested that since the spleen is a friable vascular organ, it is particularly susceptible to parenchymal bleeding when disease, trauma, or vascular obstruction and infection, especially in the setting of coexisting coagulation disorders, damage the architecture. While infectious diseases including infectious mononucleosis and malaria have been the most commonly reported disorders associated with spontaneous splenic rupture, cases associated with sarcoidosis, Gaucher’s disease, hematological malignancies, connective disorders, and amyloidosis have also been reported [10].
The mechanism of spontaneous rupture of the spleen in amyloidosis is unknown. It is generally thought to be caused by rapid expansion of the splenic capsule, which has become rigid because of amyloid deposits. Fragility of the vascular red pulp from amyloid infiltration of the blood vessel walls, and coagulation abnormalities including factor X deficiency and protrombin time prolongation, may also be contributory factors [11].
Complications like spontaneous rupture are usually found postmortem in the amyloid disease, but the use of computed tomography makes antemortem diagnosis possible [12]. In our case, splenic rupture was diagnosed at the beginning and emergency splenectomy was successfully performed.
The postoperative diagnosis was performed with histopathological examination of spleen. Green birefringence after Congo red staining is highly specific for amyloid and a polarization facility is mandatory for diagnosing amyloid [13]. Following pretreatment with potassium permanganate, the loss of congophilia indicates tiiat amyloidosis is secondary, such as in our case [9]. Endoscopic rectal biopsy showed amyloid deposition in the mucosa, and this finding stated that the amyloidosis was systemic.
Splenic rupture has been reported in AL amyloidosis commonly [10,14-21]. Nevertheless spontaneous splenic rupture due to secondary amyloidosis has been reported in two cases (Table I) [9]. In our case histopamological findings supported that our patient who had a treatment history of RA for 2 years has developed secondary amyloidosis.
Modern surgical approach tends to preserve the spleen as long as possible, and this has been carried out successfully in spontaneous splenic ruptures of infections mononucleosis. Splenic preservation has not been attempted in the reported cases of splenic amyloidosis. Owing to the persistent intraperitoneal bleeding, the conservative treatment had to be abandoned [9].
Table I. Characteristics of ruptured amyloid spleen cases in selected literature.
In conclusion splenic amyloidosis must be kept in mind ins patient who have spontaneous splenic rupture, especially with chronic diseases such as RA.
References
1. Gillmore JD, Lovat LB, Persey MR, Pepys MB, Hawkins PN. Amyloid load and clinical outcome in AA amyloidosis in relation to circulating concentration of scrum amyloid A protein. Lancet 2001;358:24-29.
2. Rcken C, Radun D, Glasbrenner B, Malfertheiner P, Roessner A. Generalized AA-amyloidosis in a 58-year-old caucasion woman with an 18-monthhistory of gastrointestinal tuberculosis. Virchows Arch 199;434:95-100.
3. Wien TN, Omtvedt LA, Landsve T, Husby G. Characterization of proteoglycans and glycosaminoglycans in splenic AA amyloid induced in mink. Scand J Immunol 2000;52: 576-583.
4. Dedi R, Bhandari S, Sugar PM, Turney JH. Delayed splenic rupture as a cause of hemoperitoneum in a CAPD patient with amyloidosis. Nephrol Dial Transplant 2001;16:2446.
5. Hosaka N, Ito M, Taki Y, Iwai H, Toki J, Ikehara S. Amyloid A gastrointestinal amyloidosis associated with idiopathic retroperitoneal fibrosis. Are Pathol Lab Med 2003;127: 735-738.
6. Monzawa S, Tsukamota T, Omata K, Hosoda K, Araki T, Sugimura K. A case with primary amyloidosis of the liver and spleen: radiologic findings. Eur J Radiol 2002;41: 237-241.
7. Gottenberg JE, Merle-Vincent F, Bentabeny F, Allanore Y, Berenbaum F, Fautrel B et al. Anti-tumor necrosis factor 2 therapy in fifteen patients with AA amyloidosis secondary to inflammatory arthritides. Arthritis Rheum 2003;48: 2019-2024.
8. Nakamura T, Yamamura Y, Tomoda K, Tsukano M, Shono M, Baba S. Efficacy of cyclophosphamide combined with prednisolone in patients with AA amyloidosis secondary to rheumatoid arthritis. Clin Rheumatol 2003;22:371-375.
9. Rajesh G, Gurpreet S, Shashanka B, Kim V, Bishan R. Spontaneous rupture of the amyloid spleen: a report of two cases. J Clin Gastroenterol 1998;26:161.
10. Oran B, Wright DG. Seldin DC, McAnney D, Skinner M, Sanchorawala V. Spontaneous rupture of the spleen in AL amyloidosis. Am J Hematol 2003;74:131-131.
11. Nowak G, Westermark P, Wernerson A, Herlenius G, Sletten K, Ericzon BG. Liver transplantation as rescue treatment in a patient with primary AL kappa amyloidosis. Transpl Int 2000;13:92-97.
12. Sandberg-Gertzen H, Ericzon BG, Blomberg B. Primary amyloidosis with spontaneous splenic rupture, cholestasis, and liver failure treated with emergency liver tarnsplantation. Am J Gastroenterol 1998;93:2254-2256.
13. Rcken C, Sletten K. Amyloid in surgical pathology. Virchows Arch 2003;443:3-16.
14. Polliack A, Hershko C. Spontaneous rupture of the spleen in amyloidosis. Isr J Mod Sci 1972;8:57-60.
15. McMahon MJ, Lintott JD, Mair WS, Lee PW, Duthie JS. Occult rupture of the spleen. Br J Surg 1977;64:641-643.
16. Wilson H, Yawn DH. Rupture of the spleen in amyloidosis. J Am Med Assoc 1979;241:790-791.
17. Okazaki K, Moriyasu F, Shkimura 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.
18. Kozicky OJ, Brandt LJ, Lederman M, Milcu M. Splenic amyloidosis: a case report of spontaneous splenic rupture with a review of the pertinent literature. Am J Gastroenterol 1987;82:582- 587.
19. Festa V, Falcetto G, Garrone C, Simone P, Valente G, Morino M, Migliena C. Spontaneous rupture of the spleen caused by primary amyloidosis. Description of a clinical case. Minerva Chir 1989;31(44):1525-1528.
20. Russell TJ, Ferrera PC. Spontaneous rupture of an amyloid spleen in a patient on continuous ambulatory peritoneal dialysis. Am J Emerg Med 1998;16:279-280.
21. Sandberg-Gertzen H, Ericzon BG, Blomberg B. Primary amyloidosis with spontaneous splenic rupture, cholestasis, and liver failure treated with emergency liver transplantation. Am J Gastroenterol 1998;93:2254 2256.
B. AYDINLI1, G. OZTURK1, A. A. BALIK4, S. ASLAN2, & F. ERDOGAN3
1 Department of General Surgery, 2 Department of Emergency Medicine and 3 Department of Pathology, Ataturk University, School of Medicine, Erzurum, Turkey, and Department of General Surgery, Gaziantep University, School of Medicine, Gaziantep, Turkey
Correspondence: Bulent Aydinli, M.D., Department of General Surgery, Ataturk University School of Medicine, Erzurum, Turkey. Tel: +90 442 3166333,2247 (work). Fax: +90 442 3166340. E-mail: [email protected]
Copyright Taylor & Francis Ltd. Sep 2006
(c) 2006 Amyloid. Provided by ProQuest Information and Learning. All rights Reserved.
Comments