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Paraspinal ekstramedüller hematopoez

Paravertebral extramedullary hematopoiesis

Paraspinal ekstramedüller hematopoez: yayımlanmış olgu görüntüsü, aksiyel kesit

5 adım

Görüntü: Barakos GP, Papazafiropoulou A, Mihos K ve ark., “A case of intrathoracic extramedullary hematopoiesis-associated pleural effusion in a beta-thalassemia intermedia patient and a short literature review.”, 2024, Figure 2.. PMC11615976 · doi:10.1177/2050313x241305169 · CC BY-NC 4.0. Değişiklik: kırpıldı, yeniden boyutlandırıldı, odak işaretleri eklendi.

Özet

Kronik hemolitik anemi, talasemi veya miyelofibrozda kemik iliği dışındaki hematopoetik doku paraspinal kitleler oluşturabilir. Kontrastsız BT, alt torakal düzeyde düzgün sınırlı veya lobüle paravertebral yumuşak doku kitlelerini, içlerinde heterojen yağ odaklarını ve komşu vertebra/kaburga korteksinde erozyonun bulunmadığını gösterir. Görünümün hematolojik öyküyle birlikte değerlendirilmesi olası tanıyı öne çıkarır; atipik bulgularda doku tanısı gerekebilir. Epidural uzanımın veya spinal kanal basısının atlanması nörolojik riskin fark edilmesini geciktirebilir.

Faz ve pencere

Kontrastsız tanısal
Toraks BT'de alt torakal paravertebral oluklarda düzgün sınırlı, sıklıkla bilateral lobüle yumuşak doku kitleleri görülür; etkin hematopoetik doku yumuşak doku atenüasyonundadır, kronik lezyonlarda yağ odakları bulunabilir. Kontrastsız BT kitle dağılımını ve makroskobik yağ varlığını gösterebilir; mikroskobik yağ deteksiyonu veya intraspinal uzanım değerlendirmesi için MR daha uygun olabilir.

Önerilen pencereler: Mediasten (G 350 / M 50), Yumuşak doku (G 400 / M 40), Kemik (G 1800 / M 400).

BT bulguları

  • Bilateral paraspinal kitleler — alt torakal düzeyde, düzgün sınırlı veya lobüle yumuşak doku dansitesinde oluşumlar.
  • Parakostal uzanım — kitle posterior kaburga komşuluğuna oturabilir; kontur iyi sınırlı kalır.
  • İç yağ odakları — daha kronik ya da yağlı dönüşüm gösteren hematopoetik dokuda heterojen düşük atenüasyon alanları görülebilir.
  • Vertebra gövdesinin ince korteksinden hematopoetik dokunun çıkışı, bu lokalizasyonun mekanizmasından biri olarak düşünülebilir.
  • Kemik destrüksiyonunun olmaması — posterior kostovertebral kortekste erozyon ve agresif kemik yıkımı beklenen tipik bulgu değildir.
  • Çok odaklılık — paraspinal kitlelere perirenal, retroperitoneal, presakral veya hepatosplenik hematopoetik odaklar eşlik edebilir.
  • Epidural uzanım — spinal kanala doğru doku geçişi ve kanal daralması varsa omurilik basısı olasılığı ayrıca belirtilir.

Normalde

Posterior mediastin normalde öncelikli olarak paravertebral yağ planları ve nörojenik yapılardan oluşur; patolojik kitleler bulunmaz.

Normal BT ile kıyasla

Aynı bölgenin, kaynak veri setinde patoloji içermediği belirtilen bir BT incelemesini kesit kesit kaydırın; organ sınırlarını açarak anatomiyi hasta görüntüsüyle karşılaştırın. Küçük rastlantısal bulgular tümüyle dışlanmamıştır.

Kaynak: TotalSegmentator v2.01 veri seti (Wasserthal ve ark., Radiology: Artificial Intelligence 2023), CC BY 4.0, vaka s1278; organ sınırları veri setinin otomatik segmentasyonundan, birleştirilerek sadeleştirildi. Görüntüler 2,5 mm kesit aralığına yeniden örneklendi.

Ayırıcı tanı

Nörojenik tümör
çoğunlukla tek taraflı, vertebral foramene veya spinal kanala uzanan ve forameni genişletebilen kitle şeklindedir.
Lenfoma
paravertebral kitlelerle birlikte sistemik lenfadenopati veya farklı nodal bölge tutulumu düşünülür.
Paravertebral apse
vertebra son plaklarında destrüksiyon, disk aralığında değişiklik veya çevre yağda inflamatuvar bulanıklık eşlik edebilir.
Lateral meningosel
kitle BOS atenüasyonundadır ve genişlemiş nöral foramenle spinal kanal ya da meninkslerle devamlılık gösterebilir.

Tuzaklar

  • Düzgün sınırlı her posterior mediastinal kitleyi nörojenik tümör saymayın; bilateral alt torakal dağılım ve kronik anemi öyküsünü eşleştirin.
  • Yağ içeriği hematopoetik lezyonun yaşını yansıtabilir; yalnızca yağ var diye lipom veya liposarkom olarak sınıflamayın.
  • BT'de belirgin kanal tutulumu görünmese bile epidural hematopoez omurilik basısı yapabilir; nörolojik belirti varsa MR ile kanal değerlendirmesi gerekir.

Kendini dene

  1. Talasemili hastada alt torakal düzeyde iki taraflı, lobüle paravertebral kitleler var; komşu korteks korunmuş. En olası tanı hangisidir?

    Cevabı göster

    Ekstramedüller hematopoez. Kronik anemiyle birlikte bilateral alt torakal kitleler ve kemik destrüksiyonunun olmaması ekstramedüller hematopoezi güçlü biçimde destekler. Diğer seçeneklerde tipik olarak tek taraflı foraminal ilişki, inflamatuvar kemik-disk bulgusu veya BOS devamlılığı beklenir.

  2. Paraspinal yumuşak doku kitlesi vertebral forameni genişletiyor ve tek taraflı spinal kanala uzanıyor. En olası alternatif nedir?

    Cevabı göster

    Schwannom. Foramen genişlemesi ve tek taraflı kanal uzanımı sinir kılıfı kökenli schwannomu destekler. Ekstramedüller hematopoez çoğunlukla iki taraflı lobüle paraspinal kitleler şeklindedir; diğer seçenekler bu foraminal paterni oluşturmaz.

Kaynaklar

Bu sayfadaki 35 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 8 cümle bu karşılaştırmada düzeltildi (2026-10-08).

  1. A case of intrathoracic extramedullary hematopoiesis-associated pleural effusion in a beta-thalassemia intermedia patient and a short literature reviewpmc.ncbi.nlm.nih.gov
  2. Thoracic Paravertebral Extramedullary Hematopoiesispmc.ncbi.nlm.nih.gov
  3. The variable manifestations of disease in pyruvate kinase deficiency and their managementpmc.ncbi.nlm.nih.gov
  4. Intrathoracic Extramedullary Hematopoiesis Arising in the Anterior Mediastinumpmc.ncbi.nlm.nih.gov
  5. A Complex Case of Glucose-Phosphate Isomerase Deficiency With an Indeterminate Adrenal Incidentalomapmc.ncbi.nlm.nih.gov
  6. Presacral and intrathoracic extramedullary hematopoiesis in a patient with β thalassemia minor.pubmed.ncbi.nlm.nih.gov
  7. A case of intrathoracic extramedullary hematopoiesis-associated pleural effusion in a beta-thalassemia intermedia patient and a short literature review.pubmed.ncbi.nlm.nih.gov
  8. Intrathoracic extramedullary hematopoiesis mimicking intrathoracic tumors: A case report.pubmed.ncbi.nlm.nih.gov
  9. Extramedullary Hematopoiesis Mimicking Mediastinal Tumor in a Patient with Hereditary Spherocytosis: Case report.pubmed.ncbi.nlm.nih.gov
  10. [Lesions of the paravertebral mediastinum].pubmed.ncbi.nlm.nih.gov
  11. Intracranial extramedullary hematopoiesis: a rare cause of headaches.pubmed.ncbi.nlm.nih.gov
  12. [Posterior mediastinal hemangioma with extramedullary haematopoieisis].pubmed.ncbi.nlm.nih.gov
  13. Paravertebral pseudotumor in patient with hereditary spherocytosis.pubmed.ncbi.nlm.nih.gov
  14. Recurrent spontaneous massive hemothorax from intrathoracic extramedullary hematopoiesis resulting in respiratory failure.pubmed.ncbi.nlm.nih.gov
  15. A Curious Case of Suspicious Lymphadenopathy in a Hereditary Spherocytosis Patient Reported as Extramedullary Hematopoiesis.pubmed.ncbi.nlm.nih.gov
  16. [Video-assisted thoracoscopic surgery for intrathoracic extramedullary hematopoiesis].pubmed.ncbi.nlm.nih.gov
  17. Extramedullary hematopoiesis in the facial sinus in a patient homozygous for Hemoglobin Lepore: the first case in literature.pubmed.ncbi.nlm.nih.gov
  18. PET CT imaging in extramedullary hematopoiesis and lung cancer surprise in a case with thalassemia intermedia.pubmed.ncbi.nlm.nih.gov
  19. Extramedullary Hematopoiesis in the Cerebral Dura Resulting in Fatal Brain Herniation in a Patient With Secondary Myelofibrosis: A Case Report.pubmed.ncbi.nlm.nih.gov
  20. Periportal Extramedullary Hematopoiesis With Bile Duct Dilatation: A Case Report of a Rare Condition.pubmed.ncbi.nlm.nih.gov
  21. Hereditary spherocytosis concomitant with JAK2V617F-positive primary myelofibrosis: a case report.pubmed.ncbi.nlm.nih.gov
  22. Intrathoracic Extramedullary Hematopoiesis Arising in the Anterior Mediastinum.pubmed.ncbi.nlm.nih.gov
  23. Spinal granulocytic sarcoma with both extradural and intradural involvement: a case report.pubmed.ncbi.nlm.nih.gov
  24. A systematic review of 47 published cases of extramedullary hematopoiesis in myelofibrosis: clinical patterns, treatment responses, and prognostic implications.pubmed.ncbi.nlm.nih.gov
  25. Extramedullary hematopoiesis presenting as a large adrenal mass mimicking adrenocortical carcinoma in a primary myelofibrosis patient: A case report.pubmed.ncbi.nlm.nih.gov
  26. Beyond the Bone Marrow: A Case Report of Extramedullary Hematopoiesis in Advanced Primary Myelofibrosis.pubmed.ncbi.nlm.nih.gov
  27. Case Report: CBFA2T3::GLIS2-positive myeloid sarcoma with focal bone marrow involvement mimicking Ewing sarcoma in an infant.pubmed.ncbi.nlm.nih.gov
  28. Primary intraosseous anastomosing hemangioma of the sacrum: illustrative case.pubmed.ncbi.nlm.nih.gov
  29. Neurological Complications in a Patient With Thalassemia Intermedia and Chronic Myeloid Leukemia: A Case of Extramedullary Hematopoiesis-Induced Spinal Cord Compression.pubmed.ncbi.nlm.nih.gov
  30. Renal extramedullary hematopoiesis as an epiphenomenon of bone marrow dysfunction in a patient with primary myelofibrosis: A rare case report.pubmed.ncbi.nlm.nih.gov
  31. Unraveling Anastomosing Hemangioma: A Case Report.pubmed.ncbi.nlm.nih.gov
  32. The diagnostic value of magnetic resonance imaging compared to computed tomography in the evaluation of fat-containing thoracic lesions.pubmed.ncbi.nlm.nih.gov
  33. Imaging of Central Nervous System Involvement in Pediatric Hematologic Disorders.pubmed.ncbi.nlm.nih.gov
  34. Febrile episode unmasking neuropsychiatric systemic lupus erythematosus with lytic lesions caused by secondary autoimmune myelofibrosis: Case report.pubmed.ncbi.nlm.nih.gov
  35. Pictorial essay of radiological features of benign intrathoracic masses.pubmed.ncbi.nlm.nih.gov
  36. Purely extradural cervical cavernous hemangioma presenting with quadriparesis and urinary retention: a case report.pubmed.ncbi.nlm.nih.gov
  37. Retrospective Review Is the Best View: A Primer for Residents and Radiologists on Missed Findings in Chest Radiography.pubmed.ncbi.nlm.nih.gov
  38. Multimodal imaging features to diagnose abdominal complications of sickle cell disease.pubmed.ncbi.nlm.nih.gov
  39. Osteoblastic skeletal metastases mimicking high-bone-turnover metabolic bone disease in gastric adenocarcinoma.pubmed.ncbi.nlm.nih.gov
  40. Mandibular Plasmacytoma as the Initial Manifestation of Non-secretory Multiple Myeloma: A Rare Diagnostic Challenge.pubmed.ncbi.nlm.nih.gov
  41. Case Report: Gastrointestinal cancer initially presenting as postmenopausal osteoporosis.pubmed.ncbi.nlm.nih.gov
  42. An Unusual Presentation of Philadelphia Chromosome Positive B-Cell Acute Lymphoblastic Leukemia With Isolated Osteolytic Lesions at Diagnosis: A Case Report.pubmed.ncbi.nlm.nih.gov
  43. Primary Splenic Classic Hodgkin Lymphoma with Immune Thrombocytopenic Purpura and Extramedullary Hematopoiesis.pubmed.ncbi.nlm.nih.gov
  44. Fetal Hepatosplenomegaly: Stepwise Diagnostic Framework, Diagnostic Approach to Fetal Hepatosplenomegaly.pubmed.ncbi.nlm.nih.gov
  45. An extramedullary hematopoietic lesion causing acute lumbar stenosis in the setting of sickle cell anemia: a case report and review of the literature.pubmed.ncbi.nlm.nih.gov
  46. Effective non-surgical management of spinal cord compression from extra-medullary hematopoiesis with radiotherapy in thalassemia intermedia: a case report.pubmed.ncbi.nlm.nih.gov
  47. Skeletal manifestations in lymphatic diseases: nonenhanced magnetic resonance lymphography.pubmed.ncbi.nlm.nih.gov
  48. Pediatric endocrine disorders: a review of intra-abdominal findings and appropriate imaging.pubmed.ncbi.nlm.nih.gov
  49. The role of thoracoplasty in modern thoracic surgery: a narrative review with three illustrative cases.pubmed.ncbi.nlm.nih.gov
  50. Brucellar spondylitis is associated with disturbance in gut microbiota and histamine metabolism associated inflammation.pubmed.ncbi.nlm.nih.gov
  51. Preliminary Retrospective Analysis of X-Ray, CT, and MRI Imaging Features of Brucellar Spondylodiscitis Diagnosis.pubmed.ncbi.nlm.nih.gov
  52. The spinal lymphatic system: an emerging pathway bridging fluid homeostasis, immunity, and disease.pubmed.ncbi.nlm.nih.gov
  53. Incidentally Detected Thoracic Dumbbell Schwannoma With Intraspinal Extension and Cord Compression: A Case Report.pubmed.ncbi.nlm.nih.gov
  54. Preoperative Intravenous Indocyanine Green Injection Demarcates Tumor Border and Adjacent Nerves in Surgical Resection of Posterior Mediastinal Neurogenic Tumors.pubmed.ncbi.nlm.nih.gov
  55. Resection of a giant paravertebral retroperitoneal schwannoma via the weaver approach: a case report on technical refinements and surgical considerations.pubmed.ncbi.nlm.nih.gov
  56. Posterior mediastinal soft-tissue Rosai-Dorfman disease mimicking a neurogenic tumor.pubmed.ncbi.nlm.nih.gov
  57. Primary testicular diffuse large B-cell lymphoma with gonadal vein tumor thrombus: A case report and review of the literature.pubmed.ncbi.nlm.nih.gov
  58. CT, MRI, and FDG PET/CT in the Assessment of Lymph Node Involvement in Pediatric Hodgkin Lymphoma: An Expert Consensus Definition by an International Collaboration on Staging Evaluation and Response Criteria Harmonization for Children, Adolescent, and Young Adult Hodgkin Lymphoma (SEARCH for CAYAHL).pubmed.ncbi.nlm.nih.gov
  59. Post-vertebroplasty lumbar spondylodiscitis due to <i>Cutibacterium modestum</i>.pubmed.ncbi.nlm.nih.gov
  60. Differentiating Tuberculous and Pyogenic Spondylodiscitis: Part II: MRI/CT Features, Prediction Models, and the Role of PET/CT-A Narrative Review.pubmed.ncbi.nlm.nih.gov
  61. Delayed diagnosis of spinal tuberculosis mimicking spondyloarthritis: a case report.pubmed.ncbi.nlm.nih.gov
  62. A diagnostic approach to mediastinal masses in clinical practice.pubmed.ncbi.nlm.nih.gov
  63. Prenatal diagnosis of infratentorial anomalies: ultrasound, fetal MRI, and implications for counseling.pubmed.ncbi.nlm.nih.gov
  64. The Differential Diagnosis of Congenital Developmental Midline Nasal Masses: Histopathological, Clinical, and Radiological Aspects.pubmed.ncbi.nlm.nih.gov
  65. Case Report: Successful treatment of mixed-phenotype acute leukemia with cauda equina syndrome as the initial manifestation.pubmed.ncbi.nlm.nih.gov
  66. Case Report: Two cases of adrenal anastomosing hemangioma misdiagnosed as angiosarcoma.pubmed.ncbi.nlm.nih.gov
  67. Radiological concern for liposarcoma unmasked as extra-adrenal myelolipoma: A case report.pubmed.ncbi.nlm.nih.gov
  68. Approach to Imaging of Mediastinal Masses.pubmed.ncbi.nlm.nih.gov
  69. Modified Transverse in-Plane Costotransverse Foramen Block for Thoracic Postherpetic Neuralgia: A Prospective Case Series.pubmed.ncbi.nlm.nih.gov
  70. ARDS management in trauma patients.pubmed.ncbi.nlm.nih.gov
  71. Preoperative CT-Based Planning for Unilateral Pedicle Approach PVP Reduces Cement Leakage and Improves Radiological Outcomes in Osteoporotic Vertebral Compression Fractures: A Retrospective Cohort Study.pubmed.ncbi.nlm.nih.gov
  72. Artificial intelligence-driven assessment of sarcopenia in orthopedic geriatrics: technical progress and clinical implications.pubmed.ncbi.nlm.nih.gov
  73. Differential diagnosis of benign and malignant vertebral compression fractures based on CT radiomics model.pubmed.ncbi.nlm.nih.gov
  74. Quantification of Skeletal Muscle at the First Lumbar Level for Prognosis in Amyotrophic Lateral Sclerosis.pubmed.ncbi.nlm.nih.gov
  75. Long-term safety and effectiveness of the "OptEase" vena cava filter.pubmed.ncbi.nlm.nih.gov
  76. Cementoplasty to cryoablation: review and current status.pubmed.ncbi.nlm.nih.gov
  77. Understanding a mass in the paraspinal region: an anatomical approach.pubmed.ncbi.nlm.nih.gov
  78. Comparison of surgical efficacy between O-arm combined with CT 3D real-time navigation system and Tinavi robot-assisted treatment of adolescent congenital scoliosis.pubmed.ncbi.nlm.nih.gov
  79. Paraspinal Extrarenal Wilms Tumor Case Report and Review of Literature.pubmed.ncbi.nlm.nih.gov
  80. The Rising Power of Electrochemotherapy in Musculoskeletal Oncology.pubmed.ncbi.nlm.nih.gov
  81. Multisystem involvement in hydatid disease: a case report of disseminated echinococcosis.pubmed.ncbi.nlm.nih.gov
  82. Surgery After Microcoil Embolization in a Patient With Large Bilateral Intrathoracic Extramedullary Hematopoiesis and Thalassemia.pubmed.ncbi.nlm.nih.gov
  83. Paraspinal Extramedullary Haematopoiesis in A Patient with Sickle Cell Disease: A Rare Clinical Presentation.pubmed.ncbi.nlm.nih.gov
  84. A case series and literature review on extramedullary hematopoiesis in patients with thalassemia treated with conformal external beam radiation treatment.pubmed.ncbi.nlm.nih.gov
  85. Mediastinal tumors: why, when, and how to biopsy?pubmed.ncbi.nlm.nih.gov
  86. Pediatric mediastinal tumors.pubmed.ncbi.nlm.nih.gov
  87. Tips and Tricks in Thoracic Radiology for Beginners: A Findings-Based Approach.pubmed.ncbi.nlm.nih.gov
  88. Lumbar Spinal Stenosis: Pathophysiology, Biomechanics, and Innovations in Diagnosis and Management.pubmed.ncbi.nlm.nih.gov
  89. Clinical Outcomes of Full-Endoscopic Visualized Foraminoplasty and Discectomy for Lumbar Disc Herniation with Bilateral Radiculopathy.pubmed.ncbi.nlm.nih.gov
  90. The value of magnetic resonance imaging and computed tomography in the study of spinal disorders.pubmed.ncbi.nlm.nih.gov
  91. Dumbbell-shaped thoracic epidural capillary hemangioma mimicking schwannoma: a case report.pubmed.ncbi.nlm.nih.gov
  92. Extradural spinal arteriovenous fistula mimicking schwannoma: Diagnostic pitfalls and surgical implications.pubmed.ncbi.nlm.nih.gov
  93. Case Report: Giant L1 dumbbell-shaped spinal schwannoma with osteolytic destruction and intense [<sup>18</sup>F]FDG uptake on PET/CT mimicking a malignant tumor.pubmed.ncbi.nlm.nih.gov
  94. Clinical and Imaging Phenotype of Schwannomatosis in Children, Adolescents and Young Adults.pubmed.ncbi.nlm.nih.gov
  95. Jugular Foramen Syndrome: Concurrent Neurological Deficits, Advanced Imaging Findings, Underlying Diagnoses, and Outcomes in 14 Dogs (2016-2024).pubmed.ncbi.nlm.nih.gov
  96. Task force of the Brazilian Society of Otology - evaluation and management of peripheral facial palsy.pubmed.ncbi.nlm.nih.gov
  97. Spontaneous bacteremia and spontaneous bacterial peritonitis share similar prognosis in patients with cirrhosis: a cohort study.pubmed.ncbi.nlm.nih.gov
  98. Progressive cervicothoracic meningocele with neurovascular compression and spinal deformity in neurofibromatosis type 1: a decade-long radiological and clinical evolution with operative repair. Illustrative case.pubmed.ncbi.nlm.nih.gov
  99. A Review of Posteromedial Lesions of the Chest Wall: What Should a Chest Radiologist Know?pubmed.ncbi.nlm.nih.gov
  100. Infantile Presentation of Lehman Syndrome with Multiple Lateral Meningoceles, Dural Ectasias, and Herniation of Conus: A Rare Case Report.pubmed.ncbi.nlm.nih.gov
  101. Preperitoneal Myelolipoma Occurring after Video-Assisted Thoracoscopic Surgery for Thymoma: A Case Report.pubmed.ncbi.nlm.nih.gov
  102. When Marrow Mimics Malignancy: Tumefactive Hepatic Extramedullary Hematopoiesis Presenting as a Hypervascular Liver Mass.pubmed.ncbi.nlm.nih.gov
  103. Multiparametric MRI to quantify disease and treatment response in mice with myeloproliferative neoplasms.pubmed.ncbi.nlm.nih.gov
  104. Repeatability of Quantitative Magnetic Resonance Imaging Biomarkers in the Tibia Bone Marrow of a Murine Myelofibrosis Model.pubmed.ncbi.nlm.nih.gov
  105. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach From the Writing Committee of the American Society for Apheresis: The Tenth Special Issue.pubmed.ncbi.nlm.nih.gov
  106. Case Report: Thigh anastomotic hemangioma.pubmed.ncbi.nlm.nih.gov
  107. Pelvic extramedullary hematopoiesis with life-threatening hemorrhage: a case report.pubmed.ncbi.nlm.nih.gov

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