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Key Clinical Message: Hemophilic pseudotumors are rare complications occurring in individuals with severe hemophilia, characterized by progressive cystic swellings in muscles and/or bones due to recurrent bleeding. Timely initiation of factor VIII replacement is crucial. Hemophilic pseudotumors are rare complications occurring in individuals with severe hemophilia, characterized by progressive cystic swellings in muscles and/or bones due to recurrent bleeding. Although their incidence has decreased with the advent of factor VIII replacement therapy, they still create challenges, particularly in regions with limited access to medical care. Here, we present a case report of a hemophilic pseudotumor of the knee joint in a 15‐year‐old male with hemophilia A. The patient presented with severe left knee pain, swelling, and restricted range of motion, prompting further investigation. Imaging studies revealed lytic lesions, and MRI bone signal changes consistent with hemophilic pseudotumors. Prompt initiation of factor VIII replacement therapy and supportive management led to a significant improvement in symptoms and joint functionality. Follow‐up after 2 months showed that the swelling had significantly reduced in size, with marked improvement in the functionality of the knee joint. This case confirms what is already known in the hemophilia literature: how important it is to prevent, diagnose, and treat pseudotumors early in hemophilia. However, longer clinical and imaging follow‐up of this case is necessary to determine whether the complaints associated with pseudotumors resolve with hematologic treatment or will require surgical treatment. [ABSTRACT FROM AUTHOR]

Key Clinical Message Hemophilic pseudotumors are rare complications occurring in individuals with severe hemophilia, characterized by progressive cystic swellings in muscles and/or bones due to recurrent bleeding. Timely initiation of factor VIII replacement is crucial. Abstract Hemophilic pseudotumors are rare complications occurring in individuals with severe hemophilia, characterized by progressive cystic swellings in muscles and/or bones due to recurrent bleeding. Although their incidence has decreased with the advent of factor VIII replacement therapy, they still create challenges, particularly in regions with limited access to medical care. Here, we present a case report of a hemophilic pseudotumor of the knee joint in a 15‐year‐old male with hemophilia A. The patient presented with severe left knee pain, swelling, and restricted range of motion, prompting further investigation. Imaging studies revealed lytic lesions, and MRI bone signal changes consistent with hemophilic pseudotumors. Prompt initiation of factor VIII replacement therapy and supportive management led to a significant improvement in symptoms and joint functionality. Follow‐up after 2 months showed that the swelling had significantly reduced in size, with marked improvement in the functionality of the knee joint. This case confirms what is already known in the hemophilia literature: how important it is to prevent, diagnose, and treat pseudotumors early in hemophilia. However, longer clinical and imaging follow‐up of this case is necessary to determine whether the complaints associated with pseudotumors resolve with hematologic treatment or will require surgical treatment.

Introduction:Mandibular pseudotumors, also known as blood cysts, are rare complications which occur more frequently in patients with an associated bleeding disorder such as hemophilia.Case Report: We present a case of a 2-year and 6-month-old patient with a hemophilic pseudotumor associated with Von Willebrand's disease, who consulted the emergency room due to spontaneous increase in volume of the left maxillary region, with no previous relevant medical history.Conclusions:Different imaging studies were carried out to characterize the lesion, providing the necessary information for the correct approach. Due to the low prevalence of this complication, we believe it is of vital importance to understand the adequate management in this patient population.

Abstract Background Cerebral infarction (CI) is an unusual complication in patients with bleeding disorders. To our knowledge, this is the first case of postoperative internal border-zone infarction (I-BZI) from Hemophilia A. Case presentation We present a case of Hemophilia A developing I-BZI, after surgical treatment of giant hemophilic pseudotumor. A 36-year-old man was introduced from other hospital by Hemophilia with giant hemophilic pseudotumor in his left thigh. Patient and his relatives did not have a history of thrombophilia. After excluding the relevant surgical contraindications, we performed the operation of pseudotumor resection. Prior to surgery, blood tests revealed hemoglobin of 137 g/L. FVIII activity was 1.5%. Activated partial thromboplastin time (APTT) was 71.50 s and D-dimer was 3.33 mg/L FEU. Immediately before surgery, the patient received an intravenous infusion of FVIII products (Xyntha®) at a dose of 3500 IU for his body weight of 80 kg. Post-operative day two (POD2), patient developed vomiting, decreased response, and dysarthria. Hemoglobin was 54 g/L with blood pressure of 110/70 mmHg. Magnetic resonance imaging of the brain showed there were multiple acute cerebral infarctions in bilateral lateral ventricles (internal border zone) and multiple ischemic foci in the white matter areas and basal ganglia of the bilateral cerebral hemispheres. This case suggested that acute severe anemia can be one of the causes of I-BZI. Conclusions For the treatment of I-BZI caused by acute anemia from Hemophilia A, volume expansion, red blood cell supplement and continuous improvement of coagulation with suitable dose of factor VIII (FVIII) should be considered to improve prognosis.

Introduction: Mandibular pseudotumors, also known as blood cysts, are rare complications which occur more frequently in patients with an associated bleeding disorder such as hemophilia. Case Report: We present a case of a 2-year and 6-month-old patient with a hemophilic pseudotumor associated with Von Willebrand's disease, who consulted the emergency room due to spontaneous increase in volume of the left maxillary region, with no previous relevant medical history. Conclusions: Different imaging studies were carried out to characterize the lesion, providing the necessary information for the correct approach. Due to the low prevalence of this complication, we believe it is of vital importance to understand the adequate management in this patient population. [ABSTRACT FROM AUTHOR]

Background: Cerebral infarction (CI) is an unusual complication in patients with bleeding disorders. To our knowledge, this is the first case of postoperative internal border-zone infarction (I-BZI) from Hemophilia A.Case Presentation: We present a case of Hemophilia A developing I-BZI, after surgical treatment of giant hemophilic pseudotumor. A 36-year-old man was introduced from other hospital by Hemophilia with giant hemophilic pseudotumor in his left thigh. Patient and his relatives did not have a history of thrombophilia. After excluding the relevant surgical contraindications, we performed the operation of pseudotumor resection. Prior to surgery, blood tests revealed hemoglobin of 137 g/L. FVIII activity was 1.5%. Activated partial thromboplastin time (APTT) was 71.50 s and D-dimer was 3.33 mg/L FEU. Immediately before surgery, the patient received an intravenous infusion of FVIII products (Xyntha®) at a dose of 3500 IU for his body weight of 80 kg. Post-operative day two (POD2), patient developed vomiting, decreased response, and dysarthria. Hemoglobin was 54 g/L with blood pressure of 110/70 mmHg. Magnetic resonance imaging of the brain showed there were multiple acute cerebral infarctions in bilateral lateral ventricles (internal border zone) and multiple ischemic foci in the white matter areas and basal ganglia of the bilateral cerebral hemispheres. This case suggested that acute severe anemia can be one of the causes of I-BZI.Conclusions: For the treatment of I-BZI caused by acute anemia from Hemophilia A, volume expansion, red blood cell supplement and continuous improvement of coagulation with suitable dose of factor VIII (FVIII) should be considered to improve prognosis. [ABSTRACT FROM AUTHOR]

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A 14-year-old inhibitor-positive male patient with severe hemophilia A and severe psychomotor disability was admitted due to left buccal swelling and impaired downward movement of the left eye. He had been on noninvasive positive-pressure ventilation (NPPV) through a nasal mask for upper airway obstruction. The patient began to have repeated epistaxis 16 months after initiation of treatment with 6 mg/kg Q4W of emicizumab, and was thus administered a bypassing agent. Left buccal swelling and impaired downward movement of the left eye appeared during the subsequent month. Imaging examination revealed a mass in the left maxillary sinus and bone destruction. Endoscopic tumor resection and tracheostomy were performed using recombinant activated factor VII. NPPV was discontinued thereafter. Pathological examination revealed that the mass was a hemophilic pseudotumor (HP). After discharge the emicizumab-regimen dose was changed to 3 mg/kg, Q2W to increase serum emicizumab levels. No recurrent HP or bleeding requiring treatment was observed. Pressure applied to the damaged nasal mucosa by NPPV was suspected as the main cause of HP development. If a mass is observed in a patient with hemophilia, HP should be considered as a possible diagnosis even if the patient is receiving emicizumab. [ABSTRACT FROM AUTHOR]

An 11-year-old boy presented with a lesion of the right orbit that was thought to be a hemophilic pseudotumor. Excisional biopsy revealed an unexpected diagnosis of mesenchymal chondrosarcoma. Both mesenchymal chondrosarcoma and hemophilic pseudotumor of the orbit are exceedingly rare. To the best of our knowledge, this is the first reported case of orbital mesenchymal chondrosarcoma masquerading as hemophilic pseudotumor. [ABSTRACT FROM AUTHOR]

Abstract Background Cranial hemophilic pseudotumor (cHPT) is a very rare disease, which is easy to misdiagnose. It is also difficult to manage such patients. We reported the first case of occipital cHPT. Case presentation Here, we presented a rare case of an occipital bone mass in a 3-year-old boy who was diagnosed with hemophilia A. The mass was misdiagnosed as an aneurysmal bone cyst by pathological examination. After resection, the patient underwent one-stage cranioplasty. However, the patient was admitted again for hematoma caused by an invasive procedure. A second surgery and one-stage cranioplasty were performed at the same time. A follow-up 3 months after discharging showed the patient was uneventful, and the titanium mesh was well fixed. Conclusion The diagnosis of cHPT requires the combining of history, radiological examination, and pathological examination. Resection is the best choice for symptomatic cHPT. Replacement treatment and less invasive treatment can make perioperative management safer. One-stage cranioplasty for resection of an occipital cHPT can improve the quality of life.

INTRODUCTION Hemophilic pseudotumor is a rare complication that occurs in patients with severe hemophilia. Results from multiple episodes of bleeding into the bones and soft tissues. PRESENTATION OF CASE A 31 years old male patient, with severe hemophilia A. Diagnosed with an abdominal tumor 10 years ago during routine screening, that progressively grew to encompass the entire abdominal area, with symptoms of intestinal obstruction. DISCUSSION Hemophilic pseudotumor appears as a painless tumor of slow growth that can compress vital organs producing bone destruction, muscle and skin necrosis. The tumor may have fistulas or break spontaneously. CONCLUSION The abdominal hemophilic pseudotumor is a rare pathological entity, with few reports worldwide, but must be considered in hemophilic patients with a well documented abdominal tumor. [ABSTRACT FROM AUTHOR]

Hemophilic pseudotumor is a rare and serious complication of hemophilia, often occurring in the long bones and muscle tissue of the extremities, with an incidence of about 1–2%. However, there is no effective surgical treatment for massive hemophilia pseudotumors of the extremities. Therefore, we would like to report this case to provide new ideas and methods for the treatment of massive hemophilic pseudotumors of the extremities through resection and total femoral prosthesis reconstruction. After admission, the patient first underwent a month-long coagulation factor replacement therapy to maintain the patient's factor IX level at approximately 100%. Then, extensive resection of hemophilic pseudotumor and total femoral prosthesis reconstruction was performed in collaboration with several departments, and we collected the patient's preoperative, intraoperative and postoperative data for analysis. After coagulation factor replacement therapy the patient's factor IX level was significantly increased and approached 100%. The surgical procedure was uneventful, and postoperative X-ray suggested a well-positioned prosthesis with postoperative pathological features consistent with a hemophilic pseudotumor. The patient was able to move around with the assistance of a walker 14 days after surgery, and there was no recurrence after one year of follow-up. For treat the massive hemophilic pseudotumors of the extremities, extensive resection and prosthetic replacement with coagulation factor replacement therapy is a safe and effective treatment that can significantly improve the prognosis and quality of life of patients. • This is the first report of such a large hemophilic pseudotumor of the proximal extremities with severe pathological fractures. • This is the first report of the application of extensive resection and total femoral prosthetic reconstruction for the treatment of hemophilic pseudotumors of the extremities. • Good prognosis achieved by standardized coagulation factor replacement therapy in combination with surgical treatment. • We hope that this case report can provide new ideas for the treatment of hemophilia pseudotumors of the extremities and functional reconstructive surgery of the extremities. [ABSTRACT FROM AUTHOR]

Hemophilic pseudotumor is a rare but serious complication that occurs less frequently in the jaw, and it may lead to bleed uncontrollably and even death. This paper reported a case of hemophilic pseudotumor with extensive osteo-destruction in the right mandible, treated by factor Ⅷ replacement and with no recurrence after 10 years of follow-up.

Hemophilic pseudotumors can occur in patients with hemophilia because of recurrent bleeding and poor hemostasis. A man in his 30s with hemophilia B and human immunodeficiency virus/hepatitis C virus co-infection complicated by liver cirrhosis presented with a large pseudotumor in the left iliopsoas muscle. However, resting to stop bleeding was difficult with his daily work. Osteolytic changes in the left ilium progressed over 8 years. A large osteolytic pseudotumor in the pelvis was also incidentally identified in his younger brother during his 30s. The same mutations in F9 (p. Arg294Gln, hemizygous mutation) associated with a non-severe phenotype were detected in both brothers. The clinical courses of the brothers suggested that large pseudotumors can occur in patients with non-severe hemophilia and underline the importance of patient education.

We report the computed tomography (CT) and magnetic resonance imaging (MRI) aspects of a rare case of a patient with a large abdominal hemophilic pseudotumor, a chronic, encapsulated, slowly expanding hematoma occurring in severe hemophilia, without involvement of iliopsoas muscles and iliac bones.