Acquired protein S deficiency leads to obliterative portal venopathy and to compensatory nodular regenerative hyperplasia in HIV-infected patients

Abstract

Acquired autoimmune protein S paucity and secondary thrombophilia appear to be causes of obliterative portal venopathy and compensatory nodular regenerative hyperplasia in HIV-positive patients.

Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)—Newer Insights into Pathogenesis and Emerging Newer Treatment Options

“…Infection of hepatic stellate cells by HIV may contribute to the pathogenesis of portal venous and sinusoidal endothelial injury via stimulation of prothrombotic cytokines such as endothelin-l, inerleukins-l and 6 and platelet derived growth factor 46 and protein S deficiency. 47 Although untested it may also be that stellate cell infection by HIV may contribute by diminishing production of ADAMTS 13 (see later). NCIPH occurring in HIV infection has been associated with prolonged exposure to Didanosine or concomitant exposure to Stavudine or Tenofovir.…”

Human immunodeficiency virus and nodular regenerative hyperplasia of liver: A systematic review

“…Arey et al [21] Case report 1 Abdominal distention, abdominal, pain, EV Bihl et al [31] Case report 1 Abdominal pain, ALP, ascites, EV, GIB, splenomegaly Bissonnette et al [11] Case report 2 Ascites, EV, GIB Cachay et al [15] Case series 1 ALP Saifee et al [52] Case series 11 Ascites, EV, GIB Cesari et al [53] Case control 5 Ascites, EV, GIB, splenomegaly Cotte et al [12] Case control 13 Abdominal pain, ALP, ascites, EV, GIB, splenomegaly Alvarez Díaz et al [33] Case report 2 ALP, ascites, EV, GIB Ding et al [54] Case report 1 ALP, EV, GIB, ascites Dinh et al [55] Case control 3 Ascites, encephalopathy, EV Fernandez-Miranda et al [9] Case report 1 Unknown Garvey et al [19] Case report 2 EV, splenomegaly Hofmaenner et al [27] Case report 1 Epigastric pain, ALP Kochin et al [56] Case report 1 EV, GIB Kovari et al [23] Case control 1 EV Maida et al [8] Case series 2 Splenomegaly Mallet et al [4] Case control 13 ALP, EV, GIB Mallet et al [57] Case report 1 Portal hypertension Mallet et al [30] Case series 8 ALP, ascites, EV, splenomegaly Mendizabal et al [58] Case control 5 EV, GIB Podevin et al [59] Case report 1 Ascites, EV, splenomegaly Sandrine et al [20] Case report 1 ALP, ascites, EV, splenomegaly Santiago et al [28] Case report 1 Abdominal distention, splenomegaly Schiano et al [22] Case report 1 ALP, EV, splenomegaly Scourfield et al [60] Retrospective cohort 4 Unknown Schouten et al [61] Case report 3 EV, GIB Stebbing et al [62] Retrospective cohort 2 ALP Tateo et al [13] Case report 3 ALP, ascites, EV, GIB Vispo et al [29] Case report 3 ALP, ascites, EV, GIB ALP: Abnormal liver panel; EV: Esophageal varices; GIB: Gastrointestinal bleed. hypersplenism (Table 1).…”

“…The utility of anticoagulation in patients with NRH is a burgeoning concept. Mallet et al [30] described a series of 21 patients with clinical evidence of prothrombotic state in the setting of NCPH. The median protein S level in this group was nearly half of normal.…”

“…One theory is that it slows disease progression by preventing the micro-thrombosis thought to play a pathogenic role in NRH. In addition, given the noted thrombophilias in these patients, anticoagulation prevents portal vein thrombosis, which can lead to refractory manifestations of portal hypertension and endstage liver disease [30][31][32] . These findings suggest potential benefit of anticoagulation therapy and warrant further studies specifically in the context of NRH.…”

“…Patients with severe liver disease and intractable symptoms of portal hypertension should be considered for liver transplant, which has been successfully described in 7 cases of patients with HIV and NRH [13,30] . In most of these cases, the indication for liver transplant was severe and intractable symptoms portal hypertension, with or without complete portal vein thrombosis.…”