Splenomegaly in Primary Antiphospholipid Syndrome without Accompanying Portal Hypertension or Comorbidity

Abstract

Objective: To demonstrate the presence of splenomegaly in primary antiphospholipid syndrome (PAPS) patients without accompanying portal hypertension or comorbidity. Methods: Twelve patients (7 women) aged 23–65 years followed upon the diagnosis of PAPS were enrolled in the study. We documented the identified causes of splenomegaly in patients with PAPS, and searched for the potential causes of splenomegaly in patients with spleen enlargement. PAPS patients with or without splenomegaly were evaluated in terms of demographic and clinical findings. Results: Splenomegaly was present in 6 of the 12 patients. In these patients, there were no infections, hematological disorders, portal hypertension or malignancy that might lead to splenomegaly. The long axis of spleen was found to be in the range of 137–155 mm in patients with splenomegaly. Splenomegaly was more frequently determined in female PAPS patients. The splenomegaly group had a longer duration of disease (median 5.5 vs. 0.75 years) and a higher number of thrombotic events (median 3 vs. 1.5). The splenomegaly group was especially composed of patients who never received any anticoagulant and acetylsalicylic acid, or who used these agents irregularly for very short periods. Conclusion: Splenomegaly was observed in association with disease duration, frequency of thrombotic events and irregular antiaggregant or anticoagulant treatment in patients with PAPS, in the absence of comorbidity or portal hypertension.

Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary?

“…The pathogenesis of thrombocytopenia in antiphospholipid antibody-positive patients is not fully understood. However, based on currently available data [ 20 , 21 , 22 , 23 , 24 , 25 , 26 ], we here summarize four different mechanisms, reported in Table 2 .…”

The Significance and Management of Thrombocytopenia in Antiphospholipid Syndrome

“…In patients with APS, splenomegaly that develops after splenic or portal vein thrombosis may result in mild thrombocytopenia. Although splenomegaly is accepted as an unexpected finding in patients with APS, a recent study showed that splenomegaly can be found in patients with APS with no portal hypertension, infection, hematological disorders, or malignancy [82].…”

Significance of ramucirumab following atezolizumab plus bevacizumab therapy for hepatocellular carcinoma using real‐world data

“…Several studies have reported that ascites is a relatively common AE of RAM treatment 17,18,30,31 . Splenomegaly and portal hypertension are closely related 32 . The VEGFR‐2 signaling pathway is dependent on portal hypertensive syndromes, such as development of a hyperdynamic splanchnic circulatory state and formation of portal‐systemic collateral vessels 33 .…”

“…17,18,30,31 Splenomegaly and portal hypertension are closely related. 32 The VEGFR-2 signaling pathway is dependent on portal hypertensive syndromes, such as development of a hyperdynamic splanchnic circulatory state and formation of portal-systemic collateral vessels. 33 Previous reports have revealed that decreased portal venous inflow and increased splanchnic arteriolar resistance were observed by inhibiting VEGFR-2 significantly.…”