Cushing's Syndrome Patient with Renal Vein Thrombosis

Presentation Number: SUN-0785
Date of Presentation: June 22nd, 2014

Saleha Babli*
Prince Sultan Military Medical City, Riyadh, Saudi Arabia

Abstract

Introduction:

Cushing’s Syndrome (CS) is associated with an increased cardiovascular morbidity and mortality. Chronic endogenous and exogenous hypercortisolism frequently induce a hypercoagulable and thrombotic condition. Little is known about haemostatic features of patients with CS.

 Venous thromboembolic complication have frequently been reported in Cushing’s Syndrome especially after surgery, its involved several site mainly lower limps vein, pulmonary embolism, jugular vein thrombosis and cerebral sinus thrombosis.

This is a case of CS with right renal vein thrombosis found incidentally in CT scan.

Clinical case:

44 years old gentleman who presented in September 2013 with proximal myopathy and new onset diabetes and hypertension which were difficult to be controlled. His physical examination was consistent with CS which was proved biochemically to be ACTH dependant.

Cushing’s disease was ruled out by negative IPSS.

Extensive work up were failed to identify the ectopic source so the patient was managed medically with Octreotide, cabergoline and Ketokonazole  this lead to normalization of his 24 hours urine cortisol ( 24 hours urine cortisol at presentation was 4374 nmole/d  ( normal  25- 146  nmole/day) drop to 136 nmole/day ) . And over the time required dexamethasone replacement.

Repeated radiological imaging 6 months later in effort to identify the source of ectopic cortisol production was again failed. However his CT abdomen showed right renal Vein thrombosis (RVT) , patient was a symptomatic from this aspect.

Extensive work up for RVT including coagulopathy, homocystien level, connective tissue disease and nephrotic syndrome screen were all within normal limit ( protein S (free) 1.3 u/ml (N 0.5-1.3 u/ml), protein S (total) 1.25 u/ml (N 1.0-1.3 u/ml), protein S functional 0.94 u/ml (N 0.7-1.4 u/ml). protein C functional 1.4 u/ml (0.7-1.4 u/ml). anti thrombin III 1.2 u/ml (N 0.8-1.2 u/ml) . activated protein C resistance 1.0  ( N 0.7-1.1). plasminogen 1.3 IU/ml (0.75-1.4 IU/ml) . anticardiolipin  and antiphospholipid were negative. ANA , anti double strand DNA both were negative. 24 hours urine protein   was 0.15 g/day (normal 0-0.15 g/day), 24 hours albumin was 18 mg/d (N less than 30). Homocystien level 12.5 micro mole/l (n 0-15 micro mole/l ).

The only abnormality in his coagulation profile was low aPTT which was 23 (normal 27-40) whic was confirmed in more than one occasion. The aPT and INR were normal.

This decrease in aPTT most likely related to CS, as increase in thrombitic risk in these patient might be in part due to increase platelets, fibrinogen, tissue plasminogen activator and decrease in aPTT levels, which represent a potential hyper coagulable and hypofibrinolytic state .

Conclusion:

Cushing’s syndrome is associated with increase thrombotic risk . This is the first case demonstrating the occurrence of renal vein thrombosis in patient with cushing’s syndrome.

 

Nothing to Disclose: SB