Idiopathic portal hypertension with transfusion-associated hemolytic anemia: a case report
Highlight box
Key findings
• Transjugular intrahepatic portosystemic shunt (TIPS) combined with pharmacotherapy was observed to be an effective treatment strategy in this patient with idiopathic portal hypertension (IPH) complicated by transfusion-associated hemolytic anemia.
What is known and what is new?
• IPH is a rare non-cirrhotic cause of portal hypertension, and autoimmune hemolytic anemia (AIHA) is an uncommon hematological disorder. TIPS is established for portal hypertension complications, and glucocorticoids are standard for AIHA. This clinical sequence is rarely reported with no management guidelines.
• First reported case of transfusion-associated hemolytic anemia during TIPS in an IPH patient.
• Demonstrates that covered stents effectively reduce portal pressure in this patient and were associated with improvement in hypersplenism parameters. Suggests that glucocorticoids may be safely administered postoperatively in this specific case.
What is the implication, and what should change now?
• TIPS with covered stents was observed to improve hypersplenism in this patient, though causality cannot be established from a single case. However, transfusion-associated AIHA is a recognizable risk requiring prompt management.
• Maintain high suspicion for AIHA in transfused IPH patients. Use washed red blood cells when transfusion is necessary. Multidisciplinary collaboration among hepatologists, hematologists, and interventional radiologists is essential for optimal outcomes.
Introduction
Background
Idiopathic portal hypertension (IPH) is a rare type of portal hypertension (1), which often occurs in young patients and has clinical manifestations of portal hypertension but rarely progresses to cirrhosis and even more rarely to liver cancer if the patient has no hepatic dysfunction, such as jaundice or hepatic encephalopathy (2). IPH manifests as obstruction or stenosis of the branches surrounding the portal vein in the liver without stenosis or occlusion of the hepatic vein or main portal vein. Currently, there is controversy regarding the diagnosis and treatment of this disease stated in the guidelines for cirrhotic portal hypertension. Autoimmune hemolytic anemia (AIHA) is caused by immune system dysfunction, impaired autoantibodies, and red blood cell damage that exceeds the compensatory ability of bone marrow and is rarely caused by transfusion of the same blood type. Transfusion-associated hemolytic reactions may occur in sensitized patients. Therefore, IPH combined with AIHA is extremely rare in clinical practice, causing significant difficulties in terms of clinical diagnosis and treatment.
Rationale and knowledge gap
IPH is a rare vascular disorder characterized by portal hypertension in the absence of cirrhosis, while AIHA is an uncommon hematological condition caused by autoantibody-mediated red blood cell destruction. Although both entities have been individually described in the literature, the occurrence of transfusion-associated hemolytic anemia in an IPH patient undergoing TIPS has not been previously reported to our knowledge.
Current management guidelines for IPH are extrapolated from cirrhotic portal hypertension due to the absence of disease-specific evidence, and the optimal treatment strategy for patients with concurrent hematological abnormalities remains undefined. Furthermore, the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in IPH patients with severe hypersplenism have not been established, and the risk of transfusion-associated AIHA in this specific population undergoing invasive procedures has not been previously recognized.
This case report documents the first instance of transfusion-associated hemolytic anemia complicating TIPS in an IPH patient, demonstrating the feasibility of covered stent TIPS for managing portal hypertension and hypersplenism, and illustrating the safe postoperative administration of glucocorticoids despite recent variceal bleeding.
Objective
This report describes a patient who was admitted to our department with upper gastrointestinal bleeding combined with AIHA caused by IPH. TIPS surgery was performed, and the patient was followed up on glucocorticoid therapy for a year. The treatment was clinically effective and there was significant improvement in her hypersplenism. We present this article in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0052/rc).
Case presentation
All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient and her family members for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
The patient was a 20-year-old nulliparous woman who was admitted to a local hospital with a 3-year history of repeated episodes of hematemesis and melena, which recurred within 17 hours after treatment. Three years earlier, she had noted a small amount of hematemesis with no obvious cause that was accompanied by melena but with no abdominal pain or jaundice. Gastroscopy at another hospital had revealed moderate esophageal varices and portal hypertensive gastropathy. Therefore, portal hypertension, esophageal variceal bleeding, and hypersplenism were considered. The patient had received approximately 5–6 units of red blood cell transfusions over the preceding 3 years during episodes of gastrointestinal bleeding. No acute transfusion reactions were documented previously.
The patient’s condition stabilized after medical treatment (including blood transfusion) without endoscopic ligation. The above-mentioned symptoms occurred multiple times and improved after medical treatment. Seventeen hours before presentation, the patient had experienced a further episode of hematemesis (approximately 200 mL) and melena with no abdominal pain or jaundice. On physical examination, she had an anemic appearance and the spleen was found to be crossing the anterior midline on palpation. There was no yellowing of the sclera. Routine blood investigations included a white blood cell (WBC) count of 1.53×109/L, a hemoglobin of 56 g/L, and a platelet count of 35×109/L. Liver function tests showed alanine aminotransferase (ALT) 22 U/L, aspartate aminotransferase (AST) 25 U/L (reference: 13–35 U/L), albumin 42 g/L, total bilirubin 15.2 µmol/L, and international normalized ratio (INR) 1.0, confirming preserved hepatic synthetic function. Contrast-enhanced abdominal computed tomography (CT) revealed portal hypertension, splenomegaly, local tumor-like dilatation of the main portal vein and its right branch, and splenic vein dilation, without any features of cirrhosis such as nodular liver surface, right lobe atrophy, left lobe hypertrophy, or regenerative nodules (Figure 1). Unfortunately, lactate dehydrogenase (LDH), haptoglobin, and reticulocyte count were not measured during the initial workup, which represents a limitation in confirming the hemolytic process. A cross-matching test was positive for a single specific antibody, indicating alloimmunization from prior transfusions. Antibody screen results showed irregular antibodies, though comprehensive antibody identification panel testing was not available at our institution. Blood transfusion, medication for acid reflux, and other treatments were administered after consultation with the patient and family members.
Four years earlier, the patient had been concerned about pallor and sought medical attention from the local hospital and then our hospital. There had been no hematemesis or melena at that time. Routine blood tests revealed a WBC count of 2.1×109/L, a hemoglobin of 59 g/L, and a platelet count of 38×109/L with normal liver function and coagulation. Two separate bone marrow puncture biopsies revealed significant erythroid hyperplasia with a small amount of mild megaloblastic transformation, the cause of which was considered to be hypersplenism related to portal hypertension and not hematological disease. After consultation with the Department of Gastroenterology and Hepatobiliary Surgery, we concluded that gastroscopy would be of limited benefit in this patient in view of the history of chronic anemia, the mild tortuosity of the gastric coronary vein on abdominal CT, and the high risk of re-bleeding after the procedure. Surgery to reduce portal hypertension was recommended with splenectomy if necessary. Further investigations were performed to exclude alternative causes of portal hypertension, as summarized in Table 1. These included comprehensive testing for viral hepatitis, autoimmune liver diseases, vascular disorders (Budd-Chiari syndrome, portal vein thrombosis), myeloproliferative neoplasms, and cirrhosis. All results were negative or normal, supporting the diagnosis of IPH. The diagnoses were IPH, esophageal varices, splenomegaly, and hypersplenism.
Table 1
| Condition excluded | Investigation performed | Result |
|---|---|---|
| Viral hepatitis | Hepatitis B surface antigen (HBsAg) | Negative |
| Hepatitis B core antibody (anti-HBc) | Negative | |
| Hepatitis C antibody (anti-HCV) | Negative | |
| Autoimmune hepatitis | Antinuclear antibody (ANA) | Negative |
| Anti-smooth muscle antibody (ASMA) | Negative | |
| Anti-liver kidney microsomal antibody | Negative | |
| Serum immunoglobulin G (IgG) level | Normal | |
| Primary biliary cholangitis | Anti-mitochondrial antibody (AMA) | Negative |
| Primary sclerosing cholangitis | Magnetic resonance cholangiopancreatography (MRCP) | No biliary strictures or beading |
| Budd-Chiari syndrome | Doppler ultrasound of hepatic veins | No hepatic vein occlusion or stenosis |
| Contrast-enhanced computed tomography (CT) venography | Hepatic veins patent, no thrombosis | |
| Portal vein thrombosis | Doppler ultrasound of portal vein | Portal vein patent, no thrombus |
| Contrast-enhanced CT angiography | No portal vein thrombosis or cavernous transformation | |
| Myeloproliferative neoplasm | JAK2 V617F mutation analysis | Negative |
| BCR-ABL fusion gene | Negative | |
| Bone marrow biopsy (morphology) | No features of myeloproliferative neoplasm | |
| Cirrhosis | Abdominal contrast-enhanced CT | No nodular liver surface; no atrophy-hypertrophy pattern; no regenerative nodules |
| Liver stiffness measurement (FibroScan) | Normal liver stiffness (≤7 kPa) | |
| Serum albumin | 42 g/L (reference: 35–50 g/L) | |
| International normalized ratio (INR) | 1.0 (reference: 0.8–1.2) | |
| Hepatic veno-occlusive disease/sinusoidal obstruction syndrome | Clinical history | No exposure to pyrrolizidine alkaloids or busulfan |
| Liver histology (available in subset) | No sinusoidal dilatation or centrilobular necrosis | |
| Congenital hepatic fibrosis | CT imaging | No biliary cysts or Caroli disease features |
TIPS surgery was performed in our department using a covered stent (Gore Viatorr; Gore Medical Flagstaff, AZ, USA) (Figure 2). The pressure in the main portal vein decreased from 41 to 30 cmH2O after surgery. There was no hematemesis or melena postoperatively, and 3 IU of washed red blood cells were infused. The next day, the patient reported fatigue and abdominal distension with dark brown urine. Physical examination revealed an anemic appearance with yellowing of the skin and sclera. Routine blood tests showed a WBC count of 1.4×109/L, a hemoglobin of 46 g/L, and a platelet count of 36×109/L. Liver function tests showed total bilirubin of 90.7 µmol/L, direct bilirubin of 18.7 µmol/L, and indirect bilirubin of 72.00 µmol/L with positive indirect and direct anti-human globulin tests. After consultation with the Department of Hematology and Transfusion, in view of the patient’s history of multiple transfusions and relevant test results, transfusion-associated hemolytic anemia with autoimmune features was considered. The patient was started on methylprednisolone 40 mg intravenously once daily for 5 days. Upon clinical improvement, she was transitioned to oral prednisone 40 mg once daily. The dose was tapered by 5 mg every week until reaching 20 mg at week 4, then tapered by 2.5 mg every 2 weeks until complete discontinuation at 3 months. After treatment with methylprednisolone, a choleretic agent for jaundice (ursodeoxycholic acid 250 mg orally twice daily as a choleretic agent), a urine alkalizing agent (sodium bicarbonate 1 g orally three times daily), and hydration, the patient’s urine reverted to a normal color 3 days later with no further symptoms of fatigue, hematemesis, melena, abdominal distension, or jaundice. Routine blood tests at this time included a WBC count of 3.49×109/L, hemoglobin of 36 g/L, and a platelet count of 35×109/L. Liver function tests included a total bilirubin of 30.50 µmol/L, direct bilirubin of 8.2 µmol/L, and indirect bilirubin of 22.30 µmol/L. Prednisone and hepatoprotective (polyene phosphatidylcholine 456 mg orally three times daily for hepatoprotection), anti-hyperammonemic (lactulose 15 mL orally twice daily and rifaximin 400 mg orally twice daily), and rivaroxaban 10 mg orally once daily for anticoagulation were taken continuously after discharge.
The findings at postoperative months 1, 3, 6, and 12 are shown in Figures 3-5. After 3 months, gastroscopy confirmed no esophageal varices, and the vascular stents remained patent at the 6-month and 12-month follow-ups.
Discussion
Key findings
This case report presents three principal findings. First, we describe an exceptionally rare clinical sequence of IPH complicated by transfusion-associated hemolytic anemia in a young woman.
Second, we demonstrate that covered stent TIPS effectively controlled recurrent variceal bleeding, and improvement in hypersplenism parameters was observed with platelet counts normalizing during one-year follow-up. Third, we observed that postoperative glucocorticoid therapy was administered without recurrent bleeding in this patient, though this finding requires confirmation to manage acute hemolysis without precipitating recurrent hemorrhage.
Strengths and limitations
Strengths
This is the first reported case of IPH complicated by transfusion-associated AIHA during TIPS placement, addressing a previously unrecognized clinical scenario. The diagnosis of IPH was rigorously established by excluding all alternative causes of portal hypertension, including viral hepatitis, autoimmune liver disease, Budd-Chiari syndrome, and myeloproliferative neoplasms. Comprehensive follow-up data over 12 months with serial laboratory monitoring and imaging confirms the durability of treatment outcomes. The case also highlights the value of multidisciplinary collaboration among hepatologists, hematologists, and interventional radiologists in managing complex patients.
Limitations
As a single case report, the findings cannot be generalized to all IPH patients. The mechanism underlying hypersplenism improvement following covered stent TIPS remains speculative and requires further investigation. Additionally, the temporal relationship between blood transfusion and AIHA onset, while highly suggestive, cannot definitively establish causation. Longer-term follow-up beyond one year is needed to assess sustained benefits and potential late complications such as stent dysfunction or hepatic encephalopathy. The absence of LDH, haptoglobin, and reticulocyte count data limits the completeness of our hemolytic workup. Future cases should include these parameters to better characterize the hemolytic mechanism.
Comparison with similar research
The pathogenesis of IPH is still unknown but may be related to heredity, autoimmune disorders, infection, portal vein thrombosis around the liver, a splenic source, and other factors (3-5) . IPH lacks evidence-based guidelines and is treated according to cirrhotic portal hypertension protocols. No randomized trials exist for TIPS in IPH with bleeding or transfusion-associated hemolytic complications. Given our patient’s recurrent bleeding and severe hypersplenism, we performed TIPS with possible splenic embolization. During one-year follow-up, no bleeding or encephalopathy occurred. While recent studies show covered stents improve thrombocytopenia in cirrhosis (6), our patient demonstrated improvement in hypersplenism parameters during follow-up after TIPS, which may potentially obviate the need for splenectomy, though this requires confirmation in larger studies.
AIHA has an annual incidence of 0.8–3.0 per 100,000 and is classified as primary or secondary (7). Our patient developed acute post-transfusion hemolysis confirmed by positive anti-human globulin tests after excluding other causes. For chronically anemic patients with hemoglobin 50–70 g/L, washed red blood cells are recommended. When cross-matching is incompatible, slow infusion with the weakest reaction should be chosen, and glucocorticoids (e.g., methylprednisolone 40 mg daily) may be given preemptively. Our patient, with hemoglobin below 50 g/L, historically avoided transfusions to prevent reactions. Given her upper gastrointestinal bleeding, glucocorticoids were deferred until after surgery. Following glucocorticoid therapy, urinary alkalinization, and choleretic agents, her hemoglobin normalized, and steroids were tapered over three months.
Explanations of findings
The observed improvement in hypersplenism parameters following covered stent TIPS may potentially be explained by several mechanisms, though causality cannot be established from this single case. Unlike bare stents, covered stents create a more controlled shunt that effectively decompresses the portal system while preserving sinusoidal perfusion, potentially reducing splenic congestion and sequestration. Additionally, the absence of cirrhosis in IPH means that hepatic synthetic function and regenerative capacity remain intact, allowing for more complete hemodynamic compensation.
The development of transfusion-associated hemolytic anemia may reflect underlying immune sensitization from prior transfusions. IPH has been associated with autoimmune disorders, and our patient’s history of chronic anemia may have primed the immune system for alloimmunization. The positive direct and indirect anti-human globulin tests confirm antibody-mediated hemolysis, while the rapid response to glucocorticoids supports an immune-mediated mechanism.
The steroid regimen began with methylprednisolone 40 mg intravenously daily, followed by oral prednisone tapering over 3 months. This gradual tapering approach was chosen to minimize relapse risk while monitoring for bleeding complications. The safe administration of postoperative glucocorticoids despite recent variceal bleeding can be attributed to the effective portal decompression achieved by TIPS. By reducing portal pressure from 41 to 30 cmH2O, the risk of rebleeding was minimized, creating a window for safe immunosuppressive therapy. This highlights the importance of sequential treatment—first addressing the acute surgical risk, then managing the hematological complication.
We acknowledge that the differentiation between AIHA and alloimmune transfusion reaction requires antibody identification panel testing, which was not available at our institution. The positive DAT and IAT support an immune-mediated mechanism, but definitive classification as AIHA versus alloimmune hemolysis cannot be established without further antibody characterization. This represents a limitation of our diagnostic workup.
Implications and actions needed
Implications
This case demonstrates that covered stent TIPS may be an effective intervention for IPH patients with recurrent variceal bleeding. The observed improvement in hypersplenism parameters in this case suggests a potential additional benefit requiring further investigation—an observation supported by objective laboratory data showing normalization of platelet counts during follow-up. The findings also reveal that transfusion-associated hemolytic anemia is a potential complication in chronically anemic IPH patients undergoing invasive procedures, requiring prompt recognition and management. Furthermore, the successful postoperative use of glucocorticoids without recurrent bleeding in this case suggests that adequate portal decompression may potentially create a safe window for immunosuppressive therapy, though this observation requires validation in larger cohorts.
Actions needed
Clinicians should maintain a high index of suspicion for AIHA in IPH patients receiving transfusions during or after TIPS. Pre-procedural cross-matching should be performed with particular care, and washed red blood cells should be utilized when transfusion is necessary. Close postoperative monitoring for signs of hemolysis—including jaundice, dark urine, and falling hemoglobin—is essential for early detection. Multidisciplinary collaboration among hepatologists, hematologists, and interventional radiologists is critical for optimal outcomes. Future studies should investigate the mechanisms underlying hypersplenism improvement following covered stent TIPS in IPH and establish registries to better define management algorithms for this rare clinical scenario.
Conclusions
This case report describes a rare clinical sequence of IPH complicated by transfusion-associated hemolytic anemia in a 20-year-old woman. The data demonstrate that covered stent TIPS effectively controlled recurrent variceal bleeding, with no hemorrhagic episodes during 12-month follow-up and endoscopic confirmation of variceal resolution. Portal pressure decreased from 41 to 30 cmH2O post-procedure, and stent patency was maintained at 6 and 12 months.
The data also show significant improvement in hypersplenism parameters was observed following TIPS, with hemoglobin increasing from 36 g/L post-hemolysis to normal range, WBC count rising from 1.43×109/L to normal values, and platelet count normalizing during follow-up. These objective laboratory improvements suggest that covered stent TIPS may be associated with reduced portal hypertension-related cytopenias in IPH patients, potentially obviating the need for splenectomy, though this requires confirmation in larger studies. Regarding AIHA management, the data support that prompt recognition of transfusion-associated hemolysis (evidenced by acute jaundice, dark urine, indirect bilirubin elevation to 72.0 µmol/L, and positive anti-human globulin tests) followed by glucocorticoid therapy resulted in rapid clinical improvement within three days and complete hematological recovery by three months. Despite recent variceal bleeding, no rebleeding occurred during or after corticosteroid treatment, indicating that postoperative glucocorticoid administration can be safe when portal pressure has been adequately reduced.
In conclusion, this case provides observations suggesting that: (I) covered stent TIPS is effective for managing portal hypertension and was associated with improvement in hypersplenism parameters in this IPH patient; (II) transfusion-associated hemolytic anemia can occur in this setting and requires prompt diagnosis; and (III) sequential treatment with TIPS followed by glucocorticoids achieved favorable outcomes in this specific case.
These observations are specific to this single case and cannot be generalized; they require confirmation in prospective studies with larger sample sizes.
Acknowledgments
None.
Footnote
Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0052/rc
Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0052/prf
Funding: This study was funded by
Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0052/coif). Both authors report this study was funded by the Chongqing Jiangbei District Science & Health Joint Medical Research Project (grant number 2025JBKWLH006). The authors have no other conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient and her family members for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
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Cite this article as: Yang Z, Zhang B. Idiopathic portal hypertension with transfusion-associated hemolytic anemia: a case report. AME Case Rep 2026;10:145.
