Extramedullary Hemopoiesis with Multiple Tumor-Simulating Mediastinal Masses in Hemoglobin E-Thalassemia Disease

doi:10.1378/chest.65.2.210

Chest

Volume 65, Issue 2, February 1974, Pages 210-212

https://doi.org/10.1378/chest.65.2.210 Get rights and content

Intrathoracic tumor-simulating heterotopic marrow in hemoglobin E-thalassemia is described for the first time. The characteristic feature of these extramedullary hemopoietic masses is the uniform radiologic picture of rounded, often lobulated, paravertebral soft tissue opacities in the posterior mediastinum usually below the 7th thoracic vertebra. When this picture is associated with chronic hemolytic anemia, preoperative diagnosis is possible making surgical intervention unnecessary.

Section snippets

Case Report

An Indonesian Chinese housewife, aged 55, was first seen in this department on May 10, 1961, with cough and breathlessness of one month's duration. Direct questioning revealed that she had had yellow sclerae for more than 20 years. She had had six pregnancies, three ending in miscarriages. Two children died during the neonatal period, and only the third child, aged 25, is alive. No details concerning the patient's family were available as she had lost contact with them during World War II.

Discussion

EMH is most commonly seen in the liver and spleen and may occur in myelofibrosis, various types of leukemia and in hemolytic anemias.⁴ Lymph node involvement is rare but has been reported in myelofibrosis and osteitis fibrosa cystica.¹⁶ Intrathoracic heterotopic marrow has also been reported in congenital spherocytic anemia,1, 2, 3^,5, 6, 7 thalassaemia9, 10, 11 and HbS-S disease.¹³

The origin of EMH in the paravertebral regions is obscure but it has been thought to develop by extension from

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Cited by (36)

Invasive methods for the diagnosis and management of intrathoracic extramedullary hematopoiesis: A literature review
2021, Respiratory Medicine and Research
Citation Excerpt :
Fine needle aspiration biopsy with radiological assistance has emerged as an essential procedure to rule out or establish malignancy in superficial as well as deep-seated lesions by obtaining adequate samples [28]. Image-guided needle biopsy has been used for the diagnosis of EMH in a paravertebral mass of the right hemithorax [19]. Ultrasound imaging has also been used for obtaining biopsy and establishing the diagnosis of intrathoracic EMH [29–31].
Extramedullary hematopoiesis is defined as hematopoiesis occurring outside of the bone marrow. It usually compensates insufficient bone marrow function or ineffective erythropoiesis and is observed mostly in hematological disorders. Most common locations of extramedullary hematopoiesis are the spleen, the liver and the lymph nodes. Intrathoracic extramedullary hematopoiesis is rare presenting as bilateral lobulated masses of lower paravertebral regions. This review summarizes the role of invasive techniques in the diagnosis and management of intrathoracic EMH and its complications.
An electronic search in PubMed and Google Scholar was conducted with the keywords “intrathoracic extramedullary hematopoiesis” AND “surgery” OR “video-assisted thoracic surgery (VATS)” OR “medical thoracoscopy” OR “biopsy” OR “thoracotomy” OR “image-guided biopsy” OR “median sternotomy”, within 1970 to 2020 with the limitation of English language to include those articles reporting data on invasive techniques in intrathoracic extramedullary hematopoiesis.
Overall, 93 articles were originally identified using our search criteria and from the reference list of the previously identified documents. Following elimination of duplicates, 29 were excluded after title, abstract or full text screening, since they did not report the use of invasive techniques in the diagnosis and management of intrathoracic extramedullary hematopoiesis.
Although in some cases radiological features are typical for the diagnosis of intrathoracic extramedullary hematopoeisis, invasive methods such as bronchoscopy with transbronchial biopsy, ιmage-guided fine needle aspiration, endobronchial ultrasound-guided fine needle aspiration of the mass and mediastinoscopy, medical thoracoscopy, median sternotomy, video-assisted thoracoscopic surgery and thoracotomy, are essential for definite diagnosis and management.
Marrow outside marrow: imaging of extramedullary haematopoiesis
2020, Clinical Radiology
Citation Excerpt :
Most cases of paraspinal EMH are not apparent on radiographs unless they are large. Cross-sectional imaging shows multiple well-defined paravertebral and subpleural paracostal masses having sharp interface (outer margin) with the pleura and lung15 The masses are often bilateral and multifocal, occurring in a segmental distribution (Fig 3a–d).31 The larger masses often have lobulate contours.
Extramedullary haematopoiesis (EMH) refers to the formation of non-neoplastic blood cell lines outside the bone marrow and is a common incidental finding when patients with haematological disorders are imaged. EMH presenting as mass (tumefactive EMH) has long been a radiological conundrum as it resembles neoplasms. Several imaging findings have been described in EMH, and these vary depending on the activity of the underlying haematopoiesis. The older lesions are easier to diagnose as they often demonstrate characteristic findings such as haemosiderin and fat deposition. In comparison, the newer, actively haematopoietic lesions often mimic neoplasms. Molecular imaging, particularly ^99mTc labelled sulphur colloid scintigraphy, may be helpful in such cases. Although imaging is extremely useful in detecting and characterising EMH, imaging alone is often non-diagnostic as no single mass shows all the typical findings. Hence, a judgement based on the clinical background, combination of imaging findings, and slow interval growth may be more appropriate and practical in making the correct diagnosis. In every case, an effort has to be made in providing an imaging-based diagnosis as it may prevent a potentially risky biopsy. When confident differentiation is not possible, biopsy has to be resorted to. This article describes the causes, pathophysiology, and theories underlying the genesis of EMH, followed by the general and location-specific imaging findings. The purpose is to provide a thorough understanding of the condition as well as enable the clinical radiologist in making an imaging-based diagnosis whenever possible and identify the situations where biopsy has to be performed.
Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia
2002, Journal of Clinical Neuroscience
Extramedullary haemopoiesis causing spinal cord compression is a rare manifestation of thalassemia. We describe a 17 year old male with thalassemia intermedia who presented with progressive paraplegia and sphincter disturbance. Magnetic resonance imaging revealed an epidural lesion extending from T5 to T8 compressing the spinal cord. The patient recovered completely after surgical decompression with postoperative radiation therapy. Histological examination of the lesion confirmed the diagnosis of extramedullary haemopoiesis. Clinical awareness of this phenomenon with early treatment is essential for optimizing the neurological outcome.
Schwannoma of posterior mediastinum: A case report and concise review
1998, Heart and Lung: Journal of Acute and Critical Care
In the vast majority of cases, schwannoma (neurilemmoma) of the posterior mediastinum arises from 1 of the intercostal nerves and most often is manifested by an asymptomatic solitary mass on a radiograph. This case report emphasizes the importance of new imaging studies in the diagnosis of this classic tumor of the mediastinum, with special regard to the differential diagnosis of such a tumor. (Heart Lung^® 1998;27:344-7)
Extramedullary hematopoiesis of the middle ear in a patient with thalassemia
1998, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Intrathoracic extramedullary hematopoiesis simulating post-traumatic intrathoracic hemorrhage
1989, The American Journal of Medicine

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Chest

Selected ReportsExtramedullary Hemopoiesis with Multiple Tumor-Simulating Mediastinal Masses in Hemoglobin E-Thalassemia Disease

Section snippets

Case Report

Discussion

Extramedullary hematopoiesis in anemias

John Hopkins Med J

Paravertebral heterotopia of bone marrow in case of acholuric jaundice

J Pathol

Hume Lectures on haemolytic icterus

Br Med J

Clinical Hematology

Massive paravertebral heterotopia of bone marrow in a case of acholuric jaundice

J Pathol

Massive thoracic extramedullary hemopoiesis

N Engl J Med

Roentgen findings in extramedullary hematopoiesis

Am J Roentgenol Radium Ther Nucl Med

Aspetti insoliti dell'iperplasia midollare nelle anemie emolitiche

Haematologica

Extramedullary hematopoiesis presenting as intrathoracic tumour: report of case in patient with thalassaemia minor

Cancer

Tumour simulating intrathoracic extramedullary hemopoiesis: report of case

Acta Radiol

Selected Reports
Extramedullary Hemopoiesis with Multiple Tumor-Simulating Mediastinal Masses in Hemoglobin E-Thalassemia Disease