Iliopsoas Hematoma in Gaucher's Disease

Selton, J.; Perrin, Julien; Ropion, Hélène; Abdelfatah, Manal; Siouala, Boualem; Pruna, L.; Kaminsky, P.

doi:10.2169/internalmedicine.50.5215

Cited by 4 publications

(3 citation statements)

References 6 publications

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“…(c) T1-weighted image shows iso-high-mixed masses extending into the surrounding soft tissues on both sides of the sacral roots, consistent with hematoma. (d) T2-weighted image shows the masses become slightly heterogeneous Jmoudiak et al 2003;Lesić et al 2006;Selton et al 2011). Horev et al reported that MRI examination revealed an unexpected high intramedullary as well as subperiosteal signal, suggesting a subacute hemorrhage or hematoma in five Gaucher disease patients (Horev et al 1991).…”

Section: Discussionmentioning

confidence: 99%

Extraosseous Extension Caused by Epidural Hematoma in Gaucher Disease Mimicking Malignant Bone Tumor

et al. 2013

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Gaucher disease is an inherited autosomal-recessive disorder caused by the defective hydrolysis of glucocerebroside. The resultant hepatosplenomegaly, hematological changes, and orthopedic complications are the predominant symptoms. However, extraosseous manifestation of Gaucher disease, mimicking malignant bone tumor, is supposed to be rare. No reports of extraosseous manifestation of Gaucher disease caused by epidural hematoma were identified in the English literature. A 64-year-old man visited a nearby clinic for low back pain and was referred to our tumor clinic on suspicion of malignant bone tumor on sacral MRI. MRI revealed a demarcated solid lesion extending into the surrounding soft tissues on both sides of the sacral roots. During preoperative examination, he suffered from pathologic fracture in right mid-femur. We performed internal fixation with intramedullary nailing, simultaneously harvesting tissue specimens. Histopathological analysis showed aggregates of Gaucher cells in the right femur and hematoma in the sacrum. Epidural hematoma in Gaucher disease, usually attributed to thrombocytopenia, is a rare manifestation of skeletal complication, mimicking malignant processes.

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Section: Discussionmentioning

confidence: 99%

Extraosseous Extension Caused by Epidural Hematoma in Gaucher Disease Mimicking Malignant Bone Tumor

et al. 2013

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“…However, post-operative [ 52 , 53 , 54 ], delivery, and post-partum bleeding [ 55 , 56 ] have been also reported. In addition to these typical bleeding manifestations of primary hemostasis defects, hemorrhages characteristics of coagulation defects, such as spontaneous Iliopsoas hematomas, have also been reported [ 57 ].…”

Section: Gd and Bleedingmentioning

confidence: 99%

Hemostatic Abnormalities in Gaucher Disease: Mechanisms and Clinical Implications

Linari

Castaman

2022

JCM

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Gaucher disease (GD) is a rare inherited lysosomal metabolism disorder, characterized by an accumulation into lysosomes of reticuloendothelial cells, especially in the bone marrow, spleen, and liver of β-glucosylceramide and glucosyl sphingosine, which is its deacylated product. Impaired storage is responsible for a chronic inflammatory state at the sites of accumulation and together represents the pathophysiological cause of GD. GD is a progressive, multi-organ chronic disorder. Type 1 GD is the most prevalent form, with heterogeneous multisystem involvement and different severity of symptoms at any age. Hematological involvement is consistent, and a bleeding tendency is frequent, particularly at diagnosis. Several coagulation and primary hemostasis abnormalities are observed in GD. Bleeding manifestations are rarely severe and usually mucocutaneous. Post-operative, delivery, and post-partum hemorrhages are also common. Thrombocytopenia, platelet function defects, and clotting abnormalities, alone or variably associated, contribute to increase the risk of bleeding in GD. Enzyme replacement therapy (ERT) or substrate reduction therapy (SRT) are the two specific available treatments effective in improving typical hematological symptoms and abnormalities, including those of hemostasis. However, the use of medication to potentiate hemostasis may be also useful in defined clinical situations: recent starting of ERT/SRT, surgery, delivery, and life-threatening bleeding.

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“…A rare subcategory of retroperitoneal hemorrhages is iliopsoas hemorrhage [2]. According to literature, spontaneous iliopsoas hemorrhage has been reported in patients with bleeding diathesis due to congenital or acquired pathology of coagulation, such as hemophilia and von Willebrand disease; in patients on anticoagulant or antiplatelet treatment and secondary to diseases that interfere with coagulation (Dengue hemorrhagic fever, Gaucher disease and Waldenström's macroglobulinemia) [3][4][5][6][7][8]. Hematomas are usually unilateral; never the less bilateral hematomas have been reported in patients on anticoagulant therapy [9][10][11].…”

Section: Introductionmentioning

confidence: 99%

Spontaneous Iliopsoas Hematoma After Total Hip Arthroplasty In A Patient On Rivaroxaban: A rare case report

Grammatikopoulou¹,

Vampertzis²,

Iosifidou³

et al. 2019

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Introduction: Pain in the inguinal region, frequently spreading to the femur, is a common symptom with a variable differential diagnosis. Spontaneous iliopsoas hematoma is a rarely reported adverse effect of anticoagulation therapy that can present with pain in the hip, with or without previous injury or surgery at the region. This case report aims to remind clinicians of this rare condition that can result in major complications. Case Presentation: An 83 year old male patient presented to our Emergency Department with pain at the right hip which within a week expanded to the lower back and anterior femur. During clinical examination restriction of movement due to soreness was noted, the hip joint was kept in slight flexion, while there were no abnormal vascular findings. Medical history included a right-sided total hip replacement 8 years ago, chronic atrial fibrilation, chronic cardiac failure and hypertension. He was on anticoagulant (rivaroxaban) and anti-hypertensive regimen. Laboratory results showed low hematocrit (HTC) and hemoglobin (HB), elevated prothrombin time (PT) and increased international normalized ratio (INR). Imaging showed a large iliopsoas hematoma. Conservative treatment was decided. Subsequently, the hematocrit normalized, the hematoma regressed and the pain receded. Conclusion: Despite being a frequent cause of complain, pain of the hip, lumbar or femur region could be the result of a more complex pathology. Such cases should therefore not be overlooked but examined thoroughly, especially if presenting with suspicious signs or symptoms or in a setting of co morbidities. When complains are with regard to an area that has been operated on, as in our case, clinicians should not attribute all symptoms on the presence of prior surgery. Keywords: Anticoagulation, spontaneous hematoma, iliopsoas, total hip arthroplasty.

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Iliopsoas Hematoma in Gaucher's Disease

Cited by 4 publications

References 6 publications

Extraosseous Extension Caused by Epidural Hematoma in Gaucher Disease Mimicking Malignant Bone Tumor

Extraosseous Extension Caused by Epidural Hematoma in Gaucher Disease Mimicking Malignant Bone Tumor

Hemostatic Abnormalities in Gaucher Disease: Mechanisms and Clinical Implications

Spontaneous Iliopsoas Hematoma After Total Hip Arthroplasty In A Patient On Rivaroxaban: A rare case report

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