Presentation
Recognizing that EMH may present as an epidural or paraspinal lesion is important since chemotherapy and radiotherapy are effective therapeutic options in the majority of patients who suffer few if any symptoms. [ncbi.nlm.nih.gov]
Extramedullary hematopoiesis presenting as an asymptomatic posterior mediastinal mass: An unusual presentation. Lung India 2016;33:569-70 How to cite this URL: Takhar R, Bunkar M, Jangid V, Saxena A. [lungindia.com]
Immune System
- Splenomegaly
These findings thus challenge our understanding of splenomegaly as a marker of disease. [ncbi.nlm.nih.gov]
1 Splenomegaly, in particular, is the result of extramedullary hematopoiesis. [haematologica.org]
Pseudotumoral masses as large as 12 cm in size have been described. 3 Associated splenomegaly was found in almost all the cases. [tropicalgastro.com]
Magnetic resonance imaging of the abdomen, performed without the administration of contrast material, revealed splenomegaly, a normal-appearing liver, ascites, and rimlike lesions on both sides of the perirenal space (Panel A, arrows). [nejm.org]
Gastrointestinal
- Epigastric Pain
We report a 64-year-old man with epigastric pain of 3-months' duration radiated to the back. Imaging workup showed multiple paravertebral, retrosternal and presacral masses. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
- Hepatosplenomegaly
It is characterized by bone marrow fibrosis, extramedullary hematopoiesis with hepatosplenomegaly and leukoerythroblastosis in the peripheral blood. [ncbi.nlm.nih.gov]
If extramedullary hematopoiesis develops, the liver and spleen become enlarged, a condition known as hepatosplenomegaly. Physical evidence of hepatosplenomegaly will be an individual who looks puffy and protrusive in the left upper abdominal area. [guwsmedical.info]
The usual manifestations of EMH include hepatosplenomegaly due to resumed red blood cell synthesis and loss of filtering capacity in the spleen and liver [ 2 ]. [omicsonline.org]
There was hepatosplenomegaly. A large hypodense lesion was seen in the spleen (Figure 3). The diagnosis of EMH was confirmed by fine-needle biopsy of the paraspinal masses. [appliedradiology.com]
- Hepatomegaly
Author information 1 Department of Pathology, Chulalongkorn University, Bangkok, Thailand. [email protected] Abstract Massive hepatomegaly is a common finding in hydrops fetalis (HF) arising from hemoglobinopathies. [ncbi.nlm.nih.gov]
Hodgkin disease 6 hemoglobinopathies sickle cell disease thalassemia hereditary spherocytosis 5 Radiographic features When involving an organ, extramedullary hematopoiesis causes visceromegaly occurring most often in the spleen (splenomegaly) and liver (hepatomegaly [radiopaedia.org]
Physical examination revealed a thin woman without lymphadenopathy, clear breath sounds bilaterally and hepatomegaly (10 cm below the costal margin). [oapublishinglondon.com]
On examination, he had pallor, jaundice, malar prominence; frontal bossing, splenomegaly (18 cm), and hepatomegaly (6 cm). [ijpmonline.org]
Neurologic
- Quadriplegia
Paraplegia or quadriplegia may ensue. [3] MRI is the diagnostic procedure which shows isointense lobulated mass lesions seen in both T 1 and T 2 weighted imaging involving extradural portion of spinal canal and in paravertebral portions. [asianjns.org]
Workup
Imaging workup showed multiple paravertebral, retrosternal and presacral masses. Cytology findings obtained by CT-guided FNAC were compatible with the diagnosis of EMH. Peripheral blood smear confirmed the presence of β-thalassemia. [ncbi.nlm.nih.gov]
Thorough and systematic preoperative workup is essential before treatment planning. [oapublishinglondon.com]
X-Ray
- Ground Glass Appearance
glass appearance, effusions, septal thickening on chest computerized tomography have been also described in PMF patients. 4 In patients with intermediate/high risk myelofibrosis, 5 allogeneic hematopoietic stem cell transplant (HSCT) is the only known [haematologica.org]
Other Pathologies
- Visceromegaly
Visceromegaly is best evaluated with ultrasound, CT or MRI. Lesions are typically hypermetabolic and are FDG-18 avid on PET-CT 4. [radiopaedia.org]
Treatment
In the English literature, there are a few cases with intracranial EH and thalassemia, which were treated with different treatment modalities, with different outcomes. There is no standard treatment approach in patients with symptomatic EH. [ncbi.nlm.nih.gov]
Prognosis
It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. [ncbi.nlm.nih.gov]
Prognosis The median length of survival for patients with primary myelofibrosis is 3.5-5.5 years. The 5-year survival rate is about half of that expected for age- and sex-matched controls. [emedicine.medscape.com]
Etiology
Otolaryngologists should consider this etiology in patients with chronic anemia and conductive hearing loss in the absence of otitis media. [ncbi.nlm.nih.gov]
[…] aims to a general approach on the condition, for a dedicated discussion for a particularly involved organ, please refer to the specific articles on: extramedullary hematopoiesis in the spleen extramedullary hematopoiesis in the adrenal gland Pathology Etiology [radiopaedia.org]
Due to the limited size of the nodules and the lack of symptoms suggestive of an infectious etiology, in 2 patients biopsy was not performed. [haematologica.org]
Epidemiology
Global epidemiology of hemoglobin disorders. Ann N Y Acad Sci. 1998. 850: 251-69 4. Ansari SH, Shamsi TS, Ashraf M, Bohray M, Farzana T, Khan MT. Molecular epidemiology of ß-thalassemia in Pakistan: Far reaching implications. [surgicalneurologyint.com]
However, exposure to radiation, Thorotrast contrast agents, and industrial solvents (eg, benzene, toluene) have been associated with an increased risk. [10, 11, 12, 13] Epidemiology Primary myelofibrosis is an uncommon disease, with an annual incidence [emedicine.medscape.com]
Pathophysiology
The current pathophysiology suggests that sacral fractures release hematopoietic stem cells, which in turn replicate to form an EMH mass. [ncbi.nlm.nih.gov]
Differentiate Extramedullary And Intramedullary Hematopoiesis Last Updated on Sat, 22 Jul 2017 | Bone Marrow Anticoagulant • Agent that prevents or delays blood coagulation Pathophysiology • Study of how normal processes are altered by disease Protocols [guwsmedical.info]
This pathophysiologic mechanism is not related to the amount of the tumour since small, early-stage solid tumours have been associated with extramedullary haematopoiesis [ 5 ]. [oapublishinglondon.com]
Pathophysiology In patients with primary myelofibrosis, the hematopoietic system is most affected. Other organ systems may be involved via extramedullary hematopoiesis. (See the image below.) [emedicine.medscape.com]
Prevention
The patient had not received preventive local chest radiation. Ten years later, he suffered from a life-threatening hemothorax complicated by acute respiratory failure without traumatic history. [ncbi.nlm.nih.gov]
We observed that loss of ATG5 in B cells significantly increased the survival of TLR7 tg mice, reduced serum IgG and anti-nuclear autoantibodies, thereby preventing glomerulonephritis. [jimmunol.org]
A neurosurgeon must be vigilant to recognize and treat such patients to prevent long-lasting disability. [surgicalneurologyint.com]
الصفحة 223 - American Thoracic Society/Centers for Disease Control and Prevention/Infectious Diseases Society of America: treatment of tuberculosis. Am J Respir Crit Care Med 2003;167:603-662. [books.google.com]