ClinicalRadiology(1991) 44, 60-61
Case Report: Hepatic and Splenic Calcification Due to Amyloid N. M . K E N N A N
a n d C. E V A N S
Department of Radiology, Cardiff Royal Infirmary, Newport Road, Cardiff A case of marked hepatic and splenic calcification due to primary amyloidosis is presented. Although the patient had been treated with continuous ambulatory peritoneal dialysis, there was no evidence of a causal relationship with the calcification. Amyloid is known to have an affinity for calcium, but this radiographic manifestation has not been previously reported. K e n n a n , N . M . & E v a n s , C. (1991). Clinical Radiology C a l c i f i c a t i o n D u e to A m y l o i d
44, 6 0 - 6 1 .
Case Report: Hepatic and Splenic
In p r i m a r y a m y l o i d o s i s , h e p a t i c i n f i l t r a t i o n is p r e s e n t in u p to o n e t h i r d o f p a t i e n t s ( K y l e a n d G r e i p p , 1983). H e p a t o m e g a l y is t h e o n l y f e a t u r e u s u a l l y seen o n p l a i n r a d i o g r a p h s in s u c h p a t i e n t s . W e d e s c r i b e a c a s e o f m a s s i v e h e p a t i c a n d splenic c a l c i f i c a t i o n d u e to h i s t o l o g i cally p r o v e n a m y l o i d disease, d e m o n s t r a t e d o n p l a i n abdominal and chest radiographs. T o o u r k n o w l e d g e , this is the first such r e p o r t .
CASE REPORT A 63-year-old male presented in early 1985 with marked hepatomegaly, renal insufficiency and proteinuria. Primary amyloidosis was diagnosed on liver biopsy. Continuous ambulatory peritoneal dialysis (CAPD) was started in March 1986. The patient had an episode of clinical peritonitis in August 1988, though no organism was isolated. Four years after his initial presentation he was found on routine chest radiograph to have marked abdominal calcification which was also shown on supine abdominal radiograph (Fig. 1) and lateral abdominal radiograph (Fig. 2) and outlined the dome of the right hepatic lobe. At no time did he show evidence of renal osteodystrophy or hypercalcaemia. Serum inorganic phosphate had been maintained below 2.2 mmol/1 throughout his illness.
Fig. 2 Lateral abdominal radiograph showing the heavily calcified liver, in particular the dome of the right lobe. Four months after these radiographic findings he was admitted with a general deterioration, hypotension and cachexia. He was found to have candida peritonitis and died despite intensive supportive therapy. The post-mortem findings were of marked calcification of the right lobe of liver with less marked involvement of the left lobe (Fig. 3) and of the spleen. No renal calcification was present. Histology confirmed primary amyloid which had extensively calcified in the liver and spleen (Fig. 4). Fig. 1 Abdominal radiograph showing the calcification of the enlarged liver extending across the middle of the abdomen and the more punctate calcification of the spleen. Correspondence to: Dr C. Evans, Department of Radiology, Cardiff Royal Infirmary, Newport Road, Cardiff CF2 ISZ
DISCUSSION Hepatic and generalized abdominal calcification both h a v e a w i d e differential d i a g n o s i s o f w h i c h a m y l o i d has
HEPATIC AND SPLENIC CALCIFICATION DUE TO AMYLOID
Fig. 3 Post-mortem radiograph of the liver.
61
factors include the acetate buffer of the diatysate and secondary hyperparathyroidism. Amyloid is known to have an affinity for calcium and radiographically detectable calcification of amyloid deposits in several soft tissues has been described, such as the lung in tracheobronchopathia osteoplastica (Alroy et al., 1972), and in nodular pulmonary amyloidosis (Weiss, 1960). It has also been described in subcutaneous tissue (Bottomley et al., 1974) and in the renal pelvis and ureter in renal amyloidosis of the organ limited variety (Gardner et al., 1971). It has been shown that amyloidotic livers have a much increased calcium content compared to non-amyloidotic livers (Yood et al., 1981), with a range of 131.5 458.5 mg calcium/g dry weight liver tissue in three patients with amyloid compared to 6.8 mg/g liver in controls. Furthermore this increased calcium content is likely to be one of the factors involved in the uptake of bone seeking radionuclides by amyloid infiltrated livers, which is often seen.
Acknowledgements. Dr S. Leadbeaner, Department of Pathology, Cardiff Royal Infirmary.
REFERENCES
Fig. 4 - Histological section of liver showing replacement of hepatoeytes by amyloid with focal calcification (lower right-hand corner). H & E. ×13.
not been a recognized cause up to now (Darlak et al.,
1980). Another recently recognized cause, originally considered in our patient, is progressive calcifying peritonitis (Marichal et aL, 1987). These authors described two patients on CAPD with 'egg shell' peritoneal calcification involving pelvic small bowel loops in particular, but later extending to involve the whole peritoneum in one patient. The aetiology remains unknown, though probable risk
Alroy, GG, Lichtig, C & Kaftori, JK (1972). Tracheobronchopathia osteoplastica: End stage of primary lung amyloidosis? Chest, 61, 465-468. Bottomley, JP, Bradley, J & Whitehouse, GH (1974). Waldenstrom's maeroglobulinaemia and amyloidosis with subcutaneous calcification and lymphographic appearances. British Journal of Radiology, 47, 232-235. Darlak, JJ, Moskowitz, M & Kattan, KR (1980). Calcifications in the liver. Radiologic Clinics of North America, 18, 209-219. Gardner, KD, Castellino, RA, Kempson, R, Young, BW & Stamey, TA (1971). Primary amyloidosis of the renal pelvis. New England Journal of Medicine, 284, 1196-1198. Kyle, RA & Greipp, PR (1983). Amyloidosis (AL)-clinical and laboratory features in 229 cases. Mayo Clinic Proceedings, 58, 665683. Marichal, JF, Faller, B, Brignon, P, Wagner, D & Straub, P (1987). Progressive calcifying peritonitis: A new complication of CAPD? Report of two cases. Nephron, 45, 229-232. Weiss, L (1960). Isolated multiple nodular pulmonary amyloidosis. American Journal of Clinical Pathology, 33, 318-329. Yood, RA, Skinner, M, Cohen, AS & Lee, VW (1981). Soft tissue uptake of bone seeking radio nuclide in amyloidosis. The Journal of Rheumatology, 8, 760-766.
Case Report: Hepatic and Splenic Calcification Due to Amyloid N. M . K E N N A N
a n d C. E V A N S
Department of Radiology, Cardiff Royal Infirmary, Newport Road, Cardiff A case of marked hepatic and splenic calcification due to primary amyloidosis is presented. Although the patient had been treated with continuous ambulatory peritoneal dialysis, there was no evidence of a causal relationship with the calcification. Amyloid is known to have an affinity for calcium, but this radiographic manifestation has not been previously reported. K e n n a n , N . M . & E v a n s , C. (1991). Clinical Radiology C a l c i f i c a t i o n D u e to A m y l o i d
44, 6 0 - 6 1 .
Case Report: Hepatic and Splenic
In p r i m a r y a m y l o i d o s i s , h e p a t i c i n f i l t r a t i o n is p r e s e n t in u p to o n e t h i r d o f p a t i e n t s ( K y l e a n d G r e i p p , 1983). H e p a t o m e g a l y is t h e o n l y f e a t u r e u s u a l l y seen o n p l a i n r a d i o g r a p h s in s u c h p a t i e n t s . W e d e s c r i b e a c a s e o f m a s s i v e h e p a t i c a n d splenic c a l c i f i c a t i o n d u e to h i s t o l o g i cally p r o v e n a m y l o i d disease, d e m o n s t r a t e d o n p l a i n abdominal and chest radiographs. T o o u r k n o w l e d g e , this is the first such r e p o r t .
CASE REPORT A 63-year-old male presented in early 1985 with marked hepatomegaly, renal insufficiency and proteinuria. Primary amyloidosis was diagnosed on liver biopsy. Continuous ambulatory peritoneal dialysis (CAPD) was started in March 1986. The patient had an episode of clinical peritonitis in August 1988, though no organism was isolated. Four years after his initial presentation he was found on routine chest radiograph to have marked abdominal calcification which was also shown on supine abdominal radiograph (Fig. 1) and lateral abdominal radiograph (Fig. 2) and outlined the dome of the right hepatic lobe. At no time did he show evidence of renal osteodystrophy or hypercalcaemia. Serum inorganic phosphate had been maintained below 2.2 mmol/1 throughout his illness.
Fig. 2 Lateral abdominal radiograph showing the heavily calcified liver, in particular the dome of the right lobe. Four months after these radiographic findings he was admitted with a general deterioration, hypotension and cachexia. He was found to have candida peritonitis and died despite intensive supportive therapy. The post-mortem findings were of marked calcification of the right lobe of liver with less marked involvement of the left lobe (Fig. 3) and of the spleen. No renal calcification was present. Histology confirmed primary amyloid which had extensively calcified in the liver and spleen (Fig. 4). Fig. 1 Abdominal radiograph showing the calcification of the enlarged liver extending across the middle of the abdomen and the more punctate calcification of the spleen. Correspondence to: Dr C. Evans, Department of Radiology, Cardiff Royal Infirmary, Newport Road, Cardiff CF2 ISZ
DISCUSSION Hepatic and generalized abdominal calcification both h a v e a w i d e differential d i a g n o s i s o f w h i c h a m y l o i d has
HEPATIC AND SPLENIC CALCIFICATION DUE TO AMYLOID
Fig. 3 Post-mortem radiograph of the liver.
61
factors include the acetate buffer of the diatysate and secondary hyperparathyroidism. Amyloid is known to have an affinity for calcium and radiographically detectable calcification of amyloid deposits in several soft tissues has been described, such as the lung in tracheobronchopathia osteoplastica (Alroy et al., 1972), and in nodular pulmonary amyloidosis (Weiss, 1960). It has also been described in subcutaneous tissue (Bottomley et al., 1974) and in the renal pelvis and ureter in renal amyloidosis of the organ limited variety (Gardner et al., 1971). It has been shown that amyloidotic livers have a much increased calcium content compared to non-amyloidotic livers (Yood et al., 1981), with a range of 131.5 458.5 mg calcium/g dry weight liver tissue in three patients with amyloid compared to 6.8 mg/g liver in controls. Furthermore this increased calcium content is likely to be one of the factors involved in the uptake of bone seeking radionuclides by amyloid infiltrated livers, which is often seen.
Acknowledgements. Dr S. Leadbeaner, Department of Pathology, Cardiff Royal Infirmary.
REFERENCES
Fig. 4 - Histological section of liver showing replacement of hepatoeytes by amyloid with focal calcification (lower right-hand corner). H & E. ×13.
not been a recognized cause up to now (Darlak et al.,
1980). Another recently recognized cause, originally considered in our patient, is progressive calcifying peritonitis (Marichal et aL, 1987). These authors described two patients on CAPD with 'egg shell' peritoneal calcification involving pelvic small bowel loops in particular, but later extending to involve the whole peritoneum in one patient. The aetiology remains unknown, though probable risk
Alroy, GG, Lichtig, C & Kaftori, JK (1972). Tracheobronchopathia osteoplastica: End stage of primary lung amyloidosis? Chest, 61, 465-468. Bottomley, JP, Bradley, J & Whitehouse, GH (1974). Waldenstrom's maeroglobulinaemia and amyloidosis with subcutaneous calcification and lymphographic appearances. British Journal of Radiology, 47, 232-235. Darlak, JJ, Moskowitz, M & Kattan, KR (1980). Calcifications in the liver. Radiologic Clinics of North America, 18, 209-219. Gardner, KD, Castellino, RA, Kempson, R, Young, BW & Stamey, TA (1971). Primary amyloidosis of the renal pelvis. New England Journal of Medicine, 284, 1196-1198. Kyle, RA & Greipp, PR (1983). Amyloidosis (AL)-clinical and laboratory features in 229 cases. Mayo Clinic Proceedings, 58, 665683. Marichal, JF, Faller, B, Brignon, P, Wagner, D & Straub, P (1987). Progressive calcifying peritonitis: A new complication of CAPD? Report of two cases. Nephron, 45, 229-232. Weiss, L (1960). Isolated multiple nodular pulmonary amyloidosis. American Journal of Clinical Pathology, 33, 318-329. Yood, RA, Skinner, M, Cohen, AS & Lee, VW (1981). Soft tissue uptake of bone seeking radio nuclide in amyloidosis. The Journal of Rheumatology, 8, 760-766.
