However, little is known about its hematopoietic activity during malignant development and progression. Intracranial or spinal compressive lesions due to extramedullary hematopoiesis have been reported in the medical literature. Spleen, red pulp extramedullary hematopoiesis, increased in a treated male b6c3f1n mouse from a chronic study higher magnification of figure 1. Diagnosis was made by tissue biopsy, fineneedle aspiration biopsy, or radionuclide bone marrow scanning. In one patient, acute spinal cord compression developed secondary to epidural hematopoiesis. Extramedullary hematopoiesis emh occurs under various. Hcc was highly considered in the differential diagnosis based on the behavior during the.
Observational studies, case series, and case reports confirm that both transfusion and hydroxyurea therapy may have a role in the prevention and management of extramedullary hematopoietic pseudotumors 3, 1012. Extramedullary hematopoiesis in a subdural hematoma. Normal conditions originate in the bone marrow some components e. Extramedullary hematopoiesis emh is the expansion and differentiation of hematopoietic stem and progenitor cells outside of the bone. Definition hematopoiesis is the proliferation of progenitor cells, which are maintained by the stem cells and their differentiation into all the cellular components of blood. Niches for extramedullary hematopoiesis in the spleen pages 1. The spleen is a major site for extramedullary hematopoiesis and adult mouse and human spleens retain low numbers of hematopoietic stem. Primary extramedullary hematopoiesis manifesting as massive.
Download fulltext pdf download fulltext pdf paravertebral extramedullary hematopoiesis article pdf available in diagnostic and interventional imaging 954. Synonyms for extramedullary hematopoiesis in free thesaurus. Extramedullary hematopoiesis radiology reference article. Mammary emh is a rare condition either in human and veterinary medicine and can be associated with. Pdf homeostatic and pathogenic extramedullary hematopoiesis. Extramedullary hematopoiesis is a response to the failure of erythropoiesis in the bone marrow. Extramedullary hematopoiesis presenting as a compressive. Hematopoiesis, or the process of blood formation, has been extensively studied at both basic and clinical levels. We retrospectively identified 44 patients with emh. A beneficial role of janus kinase 2 jak2 inhibitors on extramedullary hematopoiesis in the spleen is suggested by animal studies, and further. Niches for extramedullary hematopoiesis in the spleen.
Imaging and clinical implications department of radiology, tulane university school of medicine purposes process of hematopoiesis introduction methods pathophysiology head discussion treatment conclusion reference pelvis musculoskeletal spine emh occurs when bone marrow is no longer able to support normal. Extramedullary hematopoiesis emh refers to hematopoiesis outside of the medulla of the bone. Extramedullary hematopoiesis definition of extramedullary. Extramedullary hematopoiesis article about extramedullary. Thoracic extramedullary hematopoiesis emh is a rare disease entity that is usually associated with hematologic disorders, such as myelodysplastic or hemolytic disease. Intracranial extramedullary hematopoiesis in betathalassemia. Increased extramedullary hematopoiesis also includes increased numbers of erythroid arrow and myeloid arrowhead precursor cells. Feb 03, 2015 spleen, red pulp extramedullary hematopoiesis, increased in a treated male b6c3f1n mouse from a chronic study higher magnification of figure 1. Extramedullary hematopoiesis presented as cytopenia and massive. The involvement of other parenchymatous organs is rare and there are only sporadic reports concerning the kidney 17, 8. We present a case of a 58 yearold male, with a history of thalassemia, which presented with a paravertebral mass composed of hematopoietic tissue.
Thoracic cord compression by extramedullary hematopoiesis in. Spinal cord compression secondary to extramedullary. Until 20 weeks of prenatal life, bloodcell production occurs in the yolk sac and in the reticuloendothelial re system. Previous reports of successful treatment have included red blood cell transfusions,51 surgical excision,52 decompressive laminectomy,53 and chemotherapy including hydroxyurea54,55. If the inline pdf is not rendering correctly, you can download the pdf file here. Extramedullary hematopoiesis emh implies the production of erythroid and myeloid progenitor cells outside of the bone. Extramedullary hematopoiesis occurs in bone marrow disorders and is most commonly seen in thalassemia and myelofibrosis. Scintigraphic diagnosis of intrathoracic extramedullary. It has been done in many different locations, depending on the moment or the kind of hematopoiesis that is needed in that moment. Emh arises from multipotential cells in any tissue, such as the liver, spleen, lymph. Extramedullary hematopoiesis jama dermatology jama. What are synonyms for extramedullary hematopoiesis.
Extramedullary hematopoiesis in fetus is a physiological process which consists of two steps. Hematopoiesis is the formation and maturation of blood elements. Extramedullary hematopoiesis is defined as the formation of blood cells outside the bone marrow. Extramedullary hematopoiesis synonyms, extramedullary hematopoiesis pronunciation, extramedullary hematopoiesis translation, english dictionary definition of extramedullary hematopoiesis. Extramedullary hematopoiesis at the posterior mediastinum in. Pdf extramedullary hematopoiesis emh in laboratory animals. Extramedullary hematopoiesis refers to hematopoiesis occurring outside of the medulla of the bone. Hydroxyurea as a firstline treatment of extramedullary hematopoiesis in patients with beta thalassemia. Extramedullary hematopoiesis is the production of blood. Although extramedullary hematopoiesis is rarely encountered within the spinal canal, it should be considered among the differential diagnoses when a posterior compressive thoracic lesion contributes to myelopathy in a patient with a history of thalassemia intermedia and the accompanying chronic hemolytic anemia. Case report of the extramedullary hematopoiesis presented as. Sagittal graphic of extramedullary hematopoiesis emh depicts hematopoietic marrow extending into prevertebral and. One of the primary hemic organs is the spleen, yet the mechanisms and.
Extramedullary hematopoiesis in a subdural hematoma published on aug 1966 by journal of neurosurgery publishing group. Extramedullary hematopoiesis is the production of blood elements outside the bone marrow cavity. Extramedullary hematopoiesis in a subdural hematoma in. Extramedullary hematopoiesis in lymph nodes springerlink. Case report of the extramedullary hematopoiesis presented. Massive splenomegaly and extramedullary hematopoiesis in. Gaffney1 1 department of radiation oncology, huntsman cancer institute and university of utah, salt lake city, utah 2department of radiology, huntsman cancer institute and university of utah, salt lake city, utah. Typical morphological features consist of small aggregates of cells with intensely basophilic nuclei erythroid or small collections of immature and mature myelocytic cells myelopoiesis located in the sinusoids and, in severe cases, in. A case of perirenal extramedullary hematopoiesis in a. Nov 20, 2011 to report the imaging appearances of masslike extramedullary hematopoiesis emh, to identify those features that are sufficiently characteristic to allow a confident diagnosis, and to recognize the clinical conditions associated with emh and the relative incidence of masslike disease. Extramedullary hematopoiesis in a case of benign mixed. Ebook hematopoiesis as pdf download portable document format. Extramedullary hematopoiesis an overview sciencedirect. Thalassemia, extramedullary hematopoiesis, and spinal cord.
To report the imaging appearances of masslike extramedullary hematopoiesis emh, to identify those features that are sufficiently characteristic to allow a confident diagnosis, and to recognize the clinical conditions associated with emh and the relative incidence of masslike disease. Extramedullary hematopoiesis emh is a rare cause of spinal cord compression scc. A beneficial role of janus kinase 2 jak2 inhibitors on extramedullary hematopoiesis in the spleen is suggested by animal studies, and further clinical evaluation is underway 1, 4548. Spleen extramedullary hematopoiesis nonneoplastic lesion. Pdf extramedullary hematopoiesis emh is a pathological process secondary to underlying bone marrow bm insufficiency in adults.
Extramedullary hematopoiesis is a significant cause of splenomegaly primarily in bone marrow disorders e. Extramedullary haematopoiesis in the kidney clinical kidney. Extramedullary hematopoiesis mimicking hepatocellular carcinoma. Tumorderived vegf modulates hematopoiesis vascular cell. Extramedullary hematopoiesis emh refers to the presence of immature hematopoietic cells in extramedullary nonbone marrow sites. Human diseases such as thalassemia, immunodeficiency, and leukemia represent defects in this process.
This emh definition excludes myeloid sarcoma a tumor mass of immature cells of myeloid or monocytic lineage. This is a temporary file and hence do not link it from a website, instead link the url of this page if you wish to link the pdf file. It is a common manifestation of many chronic hemolytic anemias, and typically involves the liver, spleen, and lymph nodes. Extramedullary hematopoiesis extramedullary hematopoiesis emh. Vegfinduced angiogenesis significantly contributes to tumor growth, invasion and metastasis. Extramedullary hematopoiesis emh represents tumorlike proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. For faster navigation, this iframe is preloading the wikiwand page for extramedullary hematopoiesis. This article aims to a general approach on the condition, for a dedicated discussion for a particularly involved organ, please refer to the specific articles on. Normal haematopoiesis the bone marrow is the only site of blood production in extrauterine life. Extramedullary hematopoiesis, mediastinal masses, and spinal. Extramedullary hematopoiesis eh is defined as hematopoiesis occurring in organs outside of the bone marrow. Aside from blood transfusions and hydroxyurea, management options of paraspinal extramedullary hematopoietic pseudotumors may also include radiotherapy or.
The ability of hematopoietic stem cells hscs to mobilize from the bone marrow. Extramedullary hematopoiesis in a thalassemia patient this case was donated to by used in the following article. A case of perirenal extramedullary hematopoiesis in a patient. Extramedullary hematopoiesis at the posterior mediastinum. In the embryo and fetus it takes place in a variety of sites including the liver, spleen, thymus, lymph nodes, and bone marrow. Usually two or three cell lineages are present in emh. However, when the bm microenvironment is stressed or diseased, hematopoiesis takes. The yolk sac and the liver produce blood in the intrauterine life. Haematopoietis in the liver in utero and subsequently extrauterine haematopoiesis in the bone marrow are known as definitive haematopoiesis. Extramedullary hematopoiesis in the absence of myeloproliferative. Here we show that in a mouse tumor model, tumorderived vegf acts as an endocrinelike hormone to induce extramedullary hematopoiesis by targeting distal organs in the host. Extramedullary hematopoiesis emh is defined as the presence of hematopoietic stem cells such as erythroid and myeloid lineage plus megakaryocytes in extramedullary sites like liver, spleen and lymph nodes and is usually associated with either bone marrow or hematological disorders. Extramedullary hematopoiesis refers to hematpoiesis in locations other than the bone marrow medullary space. Chronic anemia states such as thalassemia can cause hematopoietic tissue to expand in certain locations.
Hashmi ma, guha s, sengupta p, basu d, baboo s, neha. How do i diagnose extramedullary hematopoiesis diagnostic. It is particularly prone to occur with anemia due to myelophthisis or myelosclerosis, and is usually thought of as a compensatory mechanism whereby extramedullary tissue attempts to. Because thoracic emh is usually encountered as a mass during radiologic examinations, it should be differentiated from posterior mediastinal neurogenic tumors.
Pdf in the right clinical setting, ct scans can indicate a diagnosis of extramedullary hematopoiesis. It has been done in many different locations, depending on the moment or the kind of hematopoiesis that. Extramedullary hematopoiesis in this case includes increased numbers of erythroid arrow and myeloid arrowhead precursor cells. Extramedullary hematopoiesis emh is commonly observed in rodents as a normal component of the splenic red pulp. Extramedullary hematopoiesis, mediastinal masses, and. Several sites of extramedullary hematopoiesis emh have been reported in the literature.
Extramedullary hematopoiesis synonyms, extramedullary. A case of perirenal extramedullary hematopoiesis in a patient with primary myelofibrosis kentaro imai 0 1 2 tomonori aoi 0 1 2 hiroki kitai 0 1 2 nobuhide endo 0 1 2 masahiko fujino 0 1 2 shizunori ichida 0 1 2 0 department of pathology, japanese red cross nagoya daiichi hospital, nagoya, japan 1 department of nephrology, japanese red cross nagoya daiichi hospital, 335 michishitacho. Long segment spinal epidural extramedullary hematopoiesis. Sep 16, 2010 extramedullary hematopoiesis emh is defined as the presence of hematopoietic stem cells such as erythroid and myeloid lineage plus megakaryocytes in extramedullary sites like liver, spleen and lymph nodes and is usually associated with either bone marrow or hematological disorders. Extramedullary paraspinal hematopoiesis in hereditary. Pet ct imaging in extramedullary hematopoiesis and lung. The preferred sites of extramedullary hematopoietic involvement are the spleen, liver and lymph nodes. Extramedullary hematopoiesis jama dermatology jama network. Extramedullary hematopoiesis emh or sometimes eh refers to hematopoiesis occurring outside of the medulla of the bone bone marrow. Extramedullary hematopoiesis niche in the spleen escholarship. Extramedullary hematopoiesis presented as posterior mediastinal masses in two brothers with thalassemia intermedia. Extramedullary hematopoiesis emh is the formation and development of blood cells outside the medullary spaces of the bone marrow. Extramedullary hematopoiesis, or myeloid metaplasia, is a frequently encountered condition, occurring usually in association with certain types of anemia. Mammary emh is a rare condition either in human and veterinary medicine and can be associated with benign mixed.
Spleen, red pulp extramedullary hematopoiesis, increased in a treated male b6c3f1n mouse from a chronic study. Pulmonary extramedullary hematopoiesis mimicking plasmacytoma. Cutaneous extramedullary hematopoiesis emh is a rare manifestation of intrauterine viral infections and hematologic diseases hereditary spherocytosis, twin transfusion syndrome in children. Extramedullary hematopoiesis hematopoiesis that occurs outside of the medulla of the bone is associated with hematological disorders, such as thalassemias, myelofibrosis and other states of ineffective hematopoiesis, and is accompanied by chronic anemia, splenomegaly and marked hyperplasia on bone marrow aspirate. Extramedullary hematopoiesis emh is a rare compensatory process associated with many hematologic disorders and bone marrow dysfunction with anemia.
Imaging and clinical implications department of radiology, tulane university school of medicine. Extramedullary hematopoiesis presented as cytopenia and massive paraspinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin tasleem katchi, krishna kolandaivel, pallavi khattar, taliya farooq, humayun islam, delong liu. Extramedullary hematopoiesis is usually asymptomatic and is often located in the lower paravertebral sulci and rarely in the pleura. Extramedullary hematopoiesis extramedullary hematopoiesis is a response to the failure of erythropoiesis in the bone marrow. Regulation of extramedullary hematopoiesis during immune responses. How i treat extramedullary acute myeloid leukemia blood.
Massive splenomegaly and extramedullary hematopoiesis in chronic idiopathic myelofibrosis, blood, volume 116, issue 14, 7 october 2010, pages 2410. We report a case of spinal cord compression due to recurrent spinal epidural emh, which was treated with a combination of surgery and radiotherapy. It has been reported to occur in approximately 15% of cases of thalassemia, and it also occurs in myelofibrosis and in other anemic conditions. Thoracic cord compression by extramedullary hematopoiesis. When considering the differential diagnosis, a primary concern was an extramedullary proliferation of neoplastic. Extramedullary hematopoiesis on f18 fdg petct in a case with metastatic colon carcinoma. The mechanisms of pathological extramedullary hematopoiesis in.
Hematopoiesis is a process in which the hematopoietic stem cells hscs differentiate to give rise to all lineages of blood cells. When the primary sites of hematopoiesis in the adult fail, as in myelofibrosis and hemoglobinopathies especially thalassemia and sickle cell disease, various extramedullary sites take on the role of blood formation. The yolk sac haematopoiesis, known as primitive haematopoiesis, is transient haematopoiesis that serves the purpose of rapidly providing blood to the growing embryo. Extramedullary hematopoiesis eh is a rare but specific cutaneous manifestation of myelofibrosis with myeloid metaplasia mmm. In normal adults, hscs reside in the bone marrow bm and their cell niche microenvironment in the bm is wellstudied in the literature. Niches for extramedullary hematopoiesis in the spleen core. With the exception of hemorrhagic purpuric eruptions in neonates, eh has only been described in patients with myelofibrosis. Hepatocellular carcinoma hcc, hypervascular metastasis and hepatic adenoma. Mar 29, 2019 extramedullary hematopoiesis emh can occur in the liver, especially when there is longstanding anemia. Pdf imaging features of extramedullary hematopoiesis. Laminectomy resulted in complete return of function.
Extramedullary hematopoiesis emh can occur in the liver, especially when there is longstanding anemia. Extramedullary hematopoiesis an overview sciencedirect topics. Physiologic emh occurs during embryonic and fetal development mainly. Often these cases occur in patients with an underlying hematological disorder such as acute myelogenic. Extramedullary hematopoiesis emh is commonly observed in rodents as a. Most of the reported cases are extradural lesions or, on rare occasions, foci within another neoplasm such as hemangioblastoma, meningioma or pilocytic astrocytoma. Extramedullary hematopoiesis in an adult sciencedirect. Extramedullary haematopoiesis emh refers to the development of haematopoietic tissue outside the bone marrow and normally occurs in the reticulendothelial system liver, spleen and lymph nodes.
Thereafter, the bone marrow is the primary site of hematopoiesis. Oct 07, 2010 massive splenomegaly and extramedullary hematopoiesis in chronic idiopathic myelofibrosis, blood, volume 116, issue 14, 7 october 2010, pages 2410. Extramedullary hematopoiesis is a compensatory response to insufficient bone marrow blood cell production. The red pulp is markedly expanded by numerous hematopoietic cells arrow. Magnetic resonance imaging mri offers a precise diagnosis. Sites of hematopoiesis depend on the presence of disease and on the developmental state of the individual.
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