BACKGROUND: ADAMTS13 deficiency leading to excess ultralarge von Willebrand factor (VWF) multimers and platelet clumping is typically found in thrombotic thrombocytopenic purpura (a type of thrombotic microangiopathy). Idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH) is a microangiopathy of portal venules associated with significant thrombocytopenia and predisposing gut disorders. AIM: To determine whether the portal microangiopathy in NCIPH is associated with ADAMTS13 deficiency. METHODS: Plasma levels of ADAMTS13, anti-ADAMTS13 antibodies, and VWF were compared between cases (NCIPH patients) and controls (with chronic liver diseases of other etiology) matched for severity of liver dysfunction. Eighteen NCIPH patients [median (range) MELD score 12 (7-25)] and 25 controls [MELD score 11 (4-26)] were studied. RESULTS: ADAMTS13 activity was reduced in all 18 NCIPH patients and significantly lower than controls (median, IQR: 12.5%, 5-25% and 59.0%, 44-84%, respectively, P<0.0001) [normal range for plasma ADAMTS13 activity (55-160%)]. ADAMTS13 activity was <5% in 5/18 NCIPH patients (28%) and 0/25 controls (P=0.009). ADAMTS13 antigen levels were also decreased. Sustained low ADAMTS13 levels were seen in four NCIPH patients over 6 weeks to 11 months (highest ADAMTS13 level in each patient: <5%, 6%, 6%, and 25%), despite two patients having MELD score 12. Although nine cases had low titer anti-ADAMTS13 antibodies, there was no significant difference between cases and controls. Abnormally large VWF multimers were observed in 4/11 NCIPH patients (36%) and in 0/22 controls (P=0.008). CONCLUSIONS: Sustained deficiency of ADAMTS13 appears characteristic of NCIPH, irrespective of severity of liver disease.
BACKGROUND:ADAMTS13 deficiency leading to excess ultralarge von Willebrand factor (VWF) multimers and platelet clumping is typically found in thrombotic thrombocytopenic purpura (a type of thrombotic microangiopathy). Idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH) is a microangiopathy of portal venules associated with significant thrombocytopenia and predisposing gut disorders. AIM: To determine whether the portal microangiopathy in NCIPH is associated with ADAMTS13 deficiency. METHODS: Plasma levels of ADAMTS13, anti-ADAMTS13 antibodies, and VWF were compared between cases (NCIPH patients) and controls (with chronic liver diseases of other etiology) matched for severity of liver dysfunction. Eighteen NCIPH patients [median (range) MELD score 12 (7-25)] and 25 controls [MELD score 11 (4-26)] were studied. RESULTS:ADAMTS13 activity was reduced in all 18 NCIPH patients and significantly lower than controls (median, IQR: 12.5%, 5-25% and 59.0%, 44-84%, respectively, P<0.0001) [normal range for plasma ADAMTS13 activity (55-160%)]. ADAMTS13 activity was <5% in 5/18 NCIPH patients (28%) and 0/25 controls (P=0.009). ADAMTS13 antigen levels were also decreased. Sustained low ADAMTS13 levels were seen in four NCIPH patients over 6 weeks to 11 months (highest ADAMTS13 level in each patient: <5%, 6%, 6%, and 25%), despite two patients having MELD score 12. Although nine cases had low titer anti-ADAMTS13 antibodies, there was no significant difference between cases and controls. Abnormally large VWF multimers were observed in 4/11 NCIPH patients (36%) and in 0/22 controls (P=0.008). CONCLUSIONS:Sustained deficiency of ADAMTS13 appears characteristic of NCIPH, irrespective of severity of liver disease.
Authors: Hendrik B Feys; Maria T Canciani; Flora Peyvandi; Hans Deckmyn; Karen Vanhoorelbeke; Pier M Mannucci Journal: Br J Haematol Date: 2007-06-29 Impact factor: 6.998
Authors: Ashish Goel; P L Alagammai; Sukesh C Nair; Ian Mackie; Banumathi Ramakrishna; Jayaprakash Muliyil; Shyamkumar N Keshava; C E Eapen; Elwyn Elias Journal: Indian J Gastroenterol Date: 2014-04-24
Authors: K S Prasanna; Ashish Goel; G Jayakumar Amirtharaj; Anup Ramachandran; K A Balasubramanian; Ian Mackie; Uday Zachariah; K G Sajith; Elwyn Elias; C E Eapen Journal: Indian J Gastroenterol Date: 2016-11-08
Authors: Rakhi Maiwall; Ashish Goel; Anna B Pulimood; Sudhir Babji; J Sophia; Chaya Prasad; K A Balasubramanian; Banumathi Ramakrishna; Susy Kurian; G John Fletcher; Priya Abraham; Gagandeep Kang; B S Ramakrishna; Elwyn Elias; C E Eapen Journal: Indian J Gastroenterol Date: 2014-09-18
Authors: Ashish Goel; Banumathi Ramakrishna; Jayaprakash Muliyil; Kadiyala Madhu; K G Sajith; Uday Zachariah; Jeyamani Ramachandran; Shyamkumar N Keshava; R Selvakumar; George M Chandy; Elwyn Elias; C E Eapen Journal: Dig Dis Sci Date: 2012-08-24 Impact factor: 3.199
Authors: Ashish Goel; V Raghupathy; G J Amirtharaj; Aaron Chapla; Aparna Venkatraman; Banumathi Ramakrishna; Anup Ramachandran; Nihal Thomas; K A Balasubramanian; Ian Mackie; Elwyn Elias; Chundamannil E Eapen Journal: Indian J Gastroenterol Date: 2017-10-05