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Extramedullary Hematopoiesis
Extramedullary Haematopoiesis

Images

WIKIDATA, CC BY-SA 3.0

Presentation

Its clinical course and detailed pathological findings are presented along with a review of the related literature. [ncbi.nlm.nih.gov]

Entire Body System

  • Anemia

    It is commonly seen in patients with hematopoietic disorders or chronic anemia. Here, we report the case of a 55-year-old patient who had extramedullary hematopoiesis that manifested as an asymptomatic posterior mediastinal mass. [ncbi.nlm.nih.gov]

  • Splenectomy

    An increased risk was seen in patients with splenectomy and lower hemoglobin. The imaging scoring system described is helpful in diagnosing and describing this entity, hence precluding unnecessary biopsies. [ncbi.nlm.nih.gov]

    The presence of a radiologic opacity at this level in a patient with a history of chronic anemia with or whithout splenomegaly, or with previous splenectomy should rise the suspection of intrathoracic extramedullar hemopoiesis. [archbronconeumol.org]

  • Lymphadenopathy

    Chest CT showed pretracheal and subcarinal lymphadenopathy up to 2 cm in short axis. [oapublishinglondon.com]

    The close association between the tumor cells and extramedullary hematopoietic tissue in the absence of lymphadenopathies or other tissue involvement suggests a relationship of this tumor with the recently described primary FL of the bone marrow. [ncbi.nlm.nih.gov]

    Clinical Image A 37–year–old male presented with bilateral neck masses and lymphadenopathy. The patient was evaluated with ultrasound and CT of the neck. [austinpublishinggroup.com]

    Repeat imaging in the form of CECT was performed at 5 months which showed complete resolution of the ascites, associated omental thickening, peritoneal enhancement as well as lymphadenopathy. [tropicalgastro.com]

  • Hodgkin Lymphoma

    الصفحة 173 - The Non-Hodgkin's Lymphoma Pathologic Classification Project. National Cancer Institute sponsored study of classifications of Non-Hodgkin's lymphomas. Summary and description of a Working Formulation for clinical usage. ‏ [books.google.com]

    The Authors report a case where bone marrow cells were identified in centrifuge cerebrospinal fluid of a patient suffering from non-Hodgkin lymphoma. [ncbi.nlm.nih.gov]

    (ML)悪性リンパ腫 megaloblastic anemia巨赤芽球性白血病 minimal residual disease (MRD)微小残存病変 monoclonal gammopathy単クローン性免疫グロブリン異常症 multiple myeloma (MM)多発性骨髄腫 myelodysplastic syndrome (MDS)骨髄異型成症候群 myelofibrosis (MF)骨髄繊維症 n non-Hodgkin lymphoma (NHL)非ホジキンリンパ腫 non-myeloablative [tokyo-med.ac.jp]

  • Swelling

    This case report describes a 4-year-old African American male with sickle cell disease (HbSS) who presented with bilateral orbitofacial swelling. Diagnosis was made with imaging and confirmed with tissue sampling. [ncbi.nlm.nih.gov]

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]

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]

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

  • Mediastinal Mass

    Here, we report the case of a 55-year-old patient who had extramedullary hematopoiesis that manifested as an asymptomatic posterior mediastinal mass. Chest computed tomography showed a 30 × 35-mm mass located on the left side of the D11 vertebra. [ncbi.nlm.nih.gov]

    Usually it is diagnosed incidentally and very rarely it presents as a posterior mediastinal mass clinically mimicking with many other benign or malignant mediastinal tumors. [lungindia.com]

    We present the case of a 57 years old male patient in whom a routine chest radiograph showed a posterior mediastinal mass. After thoracotomy the diagnosis of intrathoracic extramedullar hemopoiesis was made. [archbronconeumol.org]

  • Posterior Mediastinal Mass

    Here, we report the case of a 55-year-old patient who had extramedullary hematopoiesis that manifested as an asymptomatic posterior mediastinal mass. Chest computed tomography showed a 30 × 35-mm mass located on the left side of the D11 vertebra. [ncbi.nlm.nih.gov]

    Usually it is diagnosed incidentally and very rarely it presents as a posterior mediastinal mass clinically mimicking with many other benign or malignant mediastinal tumors. [lungindia.com]

    However, in the less common situation where extramedullary hematopoiesis involves other tissues within the thorax, they can be seen as a posterior mediastinal mass. [radiopaedia.org]

    We present the case of a 57 years old male patient in whom a routine chest radiograph showed a posterior mediastinal mass. After thoracotomy the diagnosis of intrathoracic extramedullar hemopoiesis was made. [archbronconeumol.org]

Serum

  • Erythroblast

    Megakaryocytic and erythroblastic cells were also seen in the pleural effusion. [ncbi.nlm.nih.gov]

    It is characterized by the production of erythroid progenitors or nucleated erythrocytes, also known as erythroblasts or megaloblasts. [en.wikipedia.org]

    This period of development, the mesoblastic period, produces primitive erythroblasts and embryonic hemoglobins (Hgbs) such as Hgb Gower I and Gower II and Hgb Portland. [guwsmedical.info]

    Gata1 is a master regulator of erythrocyte development ( Cantor and Orkin, 2002 ); Gata1 −/− mice die during gestation owing to failed differentiation of pro-erythroblasts into mature erythrocytes. [doi.org]

  • Thrombocytosis

    Distinct from the JAK2V617F retroviral transplant model, the MPLW515L model recapitulates many features of ET and MF, including severe fibrosis and thrombocytosis. [ncbi.nlm.nih.gov]

    […] involved organ, please refer to the specific articles on: extramedullary hematopoiesis in the spleen extramedullary hematopoiesis in the adrenal gland Pathology Etiology myeloproliferative disorders chronic myelogenous leukemia polycythemia vera essential thrombocytosis [radiopaedia.org]

    Due to the extramedullary hematopoiesis, patients with myelofibrosis can develop pulmonary hypertension secondary to hematopoietic infiltration, portal hypertension, thrombocytosis, hypercoagulability, and left ventricular failure. 2, 3 Radiographic findings [haematologica.org]

    […] agnogenic myeloid metaplasia with myelofibrosis and chronic idiopathic myelofibrosis. [5] Primary myelofibrosis is categorized as a chronic myeloproliferative disorder, along with chronic myelogenous leukemia (CML), polycythemia vera, and essential thrombocytosis [emedicine.medscape.com]

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]

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]

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