نتائج البحث - "Extramedullary Hematopoiesis"

1

Reactivation of tuberculosis following ruxolitinib therapy for primary myelofibrosis: Case series and literature review

المؤلفون: Moussab Damlaj, Farhan Khalid, Giamal Gmati, Mohsen Alzahrani, Khadega A. Abuelgasim

المصدر: Hematology/Oncology and Stem Cell Therapy, Vol 14, Iss 3, Pp 252-256 (2021)

مصطلحات موضوعية: Male, Ruxolitinib, Myelofibrosis, 03 medical and health sciences, Osteosclerosis, 0302 clinical medicine, Nitriles, Myeloproliferation, medicine, Humans, Tuberculosis, Diseases of the blood and blood-forming organs, Myeloproliferative neoplasm, RC254-282, Aged, business.industry, Hematopoietic Stem Cell Transplantation, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, Hematology, General Medicine, Middle Aged, Th1 Cells, Allografts, medicine.disease, Extramedullary hematopoiesis, Pyrimidines, medicine.anatomical_structure, Oncology, JAK2, Primary Myelofibrosis, 030220 oncology & carcinogenesis, Cancer research, Cytokines, Pyrazoles, Female, Bone marrow, RC633-647.5, Janus kinase, business, 030215 immunology, medicine.drug

الوصف: Primary myelofibrosis (PMF) is a subtype of BCR-ABL1 negative myeloproliferative neoplasm. Its characteristic features include clonal myeloproliferation, dysregulation of kinase signaling pathway, abnormal release of cytokines leading to fibrosis in the bone marrow, osteosclerosis, and extramedullary hematopoiesis. Approximately 20% of deaths occur because of disease progression, but death may also result occur because of cardiovascular complications or as a consequence of either infection or bleeding. The only and curative option for PMF is allogeneic hematopoietic stem cell transplant (allo-HSCT); however, the Janus kinase (JAK) 1/2 inhibitor ruxolitinib is highly effective in reducing constitutional symptoms and spleen volume, and has been found to improve survival. Ruxolitinib decreases the activity of type I T-helper cells, leading to decreased release of cytokines including tumor necrosis factor-α, interleukin-1 (IL-1), IL-6, interferon-γ, and production of IL-12, which can be a risk factor for opportunistic infections. In this report, we describe three cases of tuberculosis reactivation shortly after initiation of ruxolitinib therapy followed by a literature review.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a830d122ef9098f4aedfcda6d6bfa8c6Test
http://www.sciencedirect.com/science/article/pii/S1658387620300327Test

2

Spinal cord compression by extramedullary hematopoiesis in beta‐thalassemia major

المؤلفون: Hend Riahi, Hanene Ferjani, Kaouther Maatallah, Wafa Hamdi, Dhia Kaffel, Sonia Bouallegue, Lobna Ben Ammar

المصدر: Clinical Case Reports, Vol 8, Iss 8, Pp 1433-1436 (2020)
Clinical Case Reports

الوصف: Extramedullary hematopoiesis is a normal compensatory reaction that can affect the epidural space, leading to spinal cord compression syndrome. MRI is the imaging method of choice for diagnosis and monitoring. Treatment is still controversial.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::231ff2c0016ca9f7bb15b56936d5287eTest
https://doaj.org/article/04dec21b20ce4bd598921f383b2d6ed5Test

3

Renal post-mortem findings in myeloproliferative and myelodysplastic/myeloproliferative neoplasms

المؤلفون: Helmut Hopfer, Sara C. Meyer, Thomas Menter, Fermin Person

المصدر: Virchows Archiv

مصطلحات موضوعية: Adult, Male, medicine.medical_specialty, Polycythaemia, Arteriolosclerosis, Myeloproliferative neoplasm, Chronic myelomonocytic leukemia, 030204 cardiovascular system & hematology, Kidney, Gastroenterology, Pathology and Forensic Medicine, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Clinical significance, Myelofibrosis, Molecular Biology, Aged, Retrospective Studies, Aged, 80 and over, Diffuse glomerulosclerosis, Essential thrombocythemia, business.industry, Hematopoietic Stem Cell Transplantation, food and beverages, Cell Biology, General Medicine, Middle Aged, medicine.disease, Prognosis, Myelodysplastic-Myeloproliferative Diseases, Extramedullary hematopoiesis, Acute tubular injury, 030220 oncology & carcinogenesis, Original Article, Female, Kidney Diseases, Autopsy, business, Nephropathology

الوصف: Myeloproliferative neoplasms (MPN) are a heterogeneous group of hematological disorders presenting with an increased proliferation in one or several hematological cell lines. Renal manifestations of MPN have not been fully characterized so far. To morphologically assess the potential renal involvement in MPN patients, we analyzed histomorphological findings of a post-mortem cohort (n = 57) with a disease history of Philadelphia-negative MPN including polycythaemia vera, primary myelofibrosis, essential thrombocythemia, or chronic myelomonocytic leukemia (CMML). Seven (12.2%) patients presented with a pattern of diffuse glomerulosclerosis not attributable to diabetic or hypertensive nephropathy. Weak C4d staining suggestive for chronic thrombotic microangiopathy (TMA) was observed in 4/7 cases. Glomerulonephritis was excluded by light microscopy and immunohistochemistry. Patients with a pattern of diffuse glomerulosclerosis did not differ from the rest of the cohort regarding MPN subtype, disease duration, age, or sex. No significant proteinuria had been observed before death. Further findings attributed to MPNs were extramedullary hematopoiesis (n = 5; 8.8%) and tumor involvement in advanced disease (n = 4; 7.0%). Other common findings included arteriolosclerosis (n = 18; 31.6%) and signs of shock (n = 8; 14.0%). To our knowledge, this study is so far the largest investigating renal findings in MPN patients. There may be a causal relationship between idiopathic diffuse glomerular sclerosis and MPN, although its clinical significance and pathophysiology remain uncertain with TMA probably being relevant in a subgroup of cases. Our findings demonstrate the spectrum of renal findings in MPN from early to terminal disease of which hematologists should be aware of in daily clinical practice.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::07e8858d924d812e28c3326670cefd2bTest
http://europepmc.org/articles/PMC8572822Test

4

Extramedullary hematopoiesis causing spinal cord compression with excellent durable response after radiation therapy: Case report and review of the literature

المؤلفون: Harold Margolis, Giovanni R Fontanesi, James Fontanesi

المصدر: Journal of Family Medicine and Primary Care, Vol 9, Iss 7, Pp 3741-3744 (2020)
Journal of Family Medicine and Primary Care

الوصف: Extramedullary hematopoiesis (EMH) is a rare occurrence in the setting of spinal cord compression. We report on a 72-year-old who was initially diagnosed with polycythemia vera (PV) which after approximately 15 years converted to myelofibrosis which confirmed on bone marrow biopsy. In 2016, he presented to our ED with clinical symptoms suggested of spinal cord compression at the T3-8 region. This was confirmed by MRI imaging. After a review of existing literature, it was elected to treat the affected area with radiation consisting of 15 fractions of 200 cGy. Within 10 days, the patient had begun to regain strength in the affected regions both motor and sensory. At the 2 month follow-up, he was symptom-free and imaging also showed a complete response. In January 2019, the patient again presented with clinical symptoms of spinal cord compression in the T10-12 area. Again, this was confirmed by MRI imaging. The same fractionation scheme was used and again the patient had a complete resolution of all symptoms both motor and sensory at the 1-month follow-up. Of interest is that during both the courses of treatment there was not a significant in any blood indices from baseline presentation. In the setting of EMH-causing cord compression, the use of radiation is warranted with excellent early response that appears durable. In addition, we present a review of the literature on this topic.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f2bdc0e3b4685f0dd38b6cb87a9782d0Test
http://www.jfmpc.com/article.asp?issn=2249-4863;year=2020;volume=9;issue=7;spage=3741;epage=3744;aulast=FontanesiTest

5

Histidine decarboxylase deficiency inhibits NBP-induced extramedullary hematopoiesis by modifying bone marrow and spleen microenvironments

المؤلفون: Yasuo Endo, Hirotada Otsuka, Hiroshi Ohtsu, Masanori Nakamura, Satoshi Soeta, Satoshi Inoue, Syunya Noguchi

المصدر: International Journal of Hematology. 113:348-361

مصطلحات موضوعية: Spleen, Bone Morphogenetic Protein 4, Histidine Decarboxylase, Granulocyte, Mice, 03 medical and health sciences, 0302 clinical medicine, Erythroid Cells, Bone Marrow, Granulocyte Colony-Stimulating Factor, medicine, Animals, RNA, Messenger, Mice, Knockout, Alendronate, Chemistry, Macrophages, Anemia, Hematology, Flow Cytometry, Hypoxia-Inducible Factor 1, alpha Subunit, medicine.disease, Histidine decarboxylase, Chemokine CXCL12, Extramedullary hematopoiesis, Mice, Inbred C57BL, Haematopoiesis, medicine.anatomical_structure, Cellular Microenvironment, Bone morphogenetic protein 4, Enzyme Induction, Hematopoiesis, Extramedullary, 030220 oncology & carcinogenesis, Cancer research, Erythropoiesis, Bone marrow, Histamine, 030215 immunology

الوصف: Histidine decarboxylase (HDC), a histamine synthase, is expressed in various hematopoietic cells and is induced by hematopoietic cytokines such as granulocyte colony-stimulating factor (G-CSF). We previously showed that nitrogen-containing bisphosphonate (NBP)-treatment induces extramedullary hematopoiesis via G-CSF stimulation. However, the function of HDC in NBP-induced medullary and extramedullary hematopoiesis remains unclear. Here, we investigated changes in hematopoiesis in wild-type and HDC-deficient (HDC-KO) mice. NBP treatment did not induce anemia in wild-type or HDC-KO mice, but did produce a gradual increase in serum G-CSF levels in wild-type mice. NBP treatment also enhanced Hdc mRNA expression and erythropoiesis in the spleen and reduced erythropoiesis in bone marrow and the number of vascular adhesion molecule 1 (VCAM-1)-positive macrophages in wild-type mice, as well as increased the levels of hematopoietic progenitor cells and proliferating cells in the spleen and enhanced expression of bone morphogenetic protein 4 (Bmp4), CXC chemokine ligand 12 (Cxcl12), and hypoxia inducible factor 1 (Hif1) in the spleen. However, such changes were not observed in HDC-KO mice. These results suggest that histamine may affect hematopoietic microenvironments of the bone marrow and spleen by changing hematopoiesis-related factors in NBP-induced extramedullary hematopoiesis.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0e5865facd516bdfdfbd4877164fe79bTest
https://doi.org/10.1007/s12185-020-03051-0Test

6

A focal extramedullary hematopoiesis of the spleen in a patient with essential thrombocythemia presenting with a complicated postoperative course: a case report

المؤلفون: Hikaru Hayashi, Akira Shimizu, Ryoichiro Kobayashi, Koji Kubota, Shinsuke Sugenoya, Koya Yasukawa, Kiyotaka Hosoda, Yuji Soejima, Tsuyoshi Notake

المصدر: Surgical Case Reports, Vol 7, Iss 1, Pp 1-6 (2021)
Surgical Case Reports

الوصف: Background Extramedullary hematopoiesis is a compensatory response occurring secondary to inadequate bone marrow function and is occasionally observed in essential thrombocythemia (ET). This disease usually presents as multifocal masses in the paravertebral or intra-abdominal region; however, formation of a focal mass in the liver or spleen is rare. In addition, ET is characterized by increased platelet count and shows a tendency toward thrombosis and, occasionally, bleeding. Serious bleeding is common in ET patients, caused by the decrease in or abnormalities of von Willebrand factor (vWF) as a consequence of the precipitous rise in platelets. Therefore, strict management of platelet count using medication is crucial in patients with ET who require invasive procedures, especially splenectomy. Case presentation A 68-year-old man with ET was found to have an enlargement of a focal splenic tumor. Imaging findings revealed that the tumor was likely a hemangioma or hamartoma; however, the possibility of malignant disease could not be completely ruled out because of short-term tumor enlargement, and we conducted a splenectomy. The surgery was uneventful, but the patient presented with severe polycythemia and vWF abnormalities postoperatively, which resulted in bleeding from the drain insertion site and wound, epistaxis, and hemorrhoidal bleeding. Three months after discharge, polycythemia still persisted and the level of vWF gradually decreased. With a decrease in vWF, the patient suffered from an increased bleeding tendency. Therefore, the patient has been referred for bone marrow transplantation and is currently awaiting a suitable donor. Conclusions Extramedullary hematopoiesis should be listed as a differential diagnosis of focal enlarged splenic tumors, especially in patients with myeloproliferative disorders. Additionally, in splenectomy for ET patients, careful perioperative management taking into consideration the conflicting features of a tendency toward thrombus formation and bleeding is necessary.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::82087f1f04b559559e70e60c667fee0aTest
https://doaj.org/article/ea33d961ee0c46b2b0ad3a1876db8651Test

7

Case Report: An Undefined Liver Lesion in a Young Man With Severe Aplastic Anemia: A Teachable Moment

المؤلفون: Liming Wang, Jidong Sui, Zhenming Gao, Jinsong Yan, Jin Liu, Jie Bian, Deguang Sun, Kun Guo

المصدر: Frontiers in Surgery
Frontiers in Surgery, Vol 8 (2021)

الوصف: In this work, we reported a young man complaining of asthenia and intermittent fever for 10 days, and an ultrasound showed an undefined lesion on his liver. Facing the patient's situation with severe agranulocytosis, anemia, and thrombocytopenia, we passed through a tough diagnostic process for choosing an appropriate treatment for him with an ambiguous result of pathological biopsy. The undefined liver lesion was successfully solved by withdrawing the androgen for observation, without lobectomy. The lesion gradually diminished over 2 years of follow-up.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::21d446660ff72138dc4add0c21d91650Test

8

A rare cause of lumbar nerve root compression: Extramedullary hematopoiesis in a patient with thalassemia major

المؤلفون: H. Boussaa, Wafa Hamdi, Kaouther Maatallah, Hanene Ferjani, Dhia Kaffel

المصدر: Clinical Case Reports, Vol 9, Iss 5, Pp n/a-n/a (2021)
Clinical Case Reports

الوصف: Extramedullary hematopoiesis (EMH) can rarely involve the epidural space, leading to spinal cord compression syndrome. No treatment guidelines have been established yet.
Magnetic resonance imaging showing spectacular improvement of epidural and sacral extramedullary hematopoiesis after radiotherapy in a 23‐year‐old Tunisian man with thalassemia major.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::632acf36f2b4371a9c649f950f0467e9Test
https://doaj.org/article/f5609c67c88649be88b91a91346d80d6Test

9

Extramedullary Hematopoiesis in Adrenal Incidentaloma: Case Report of an Atypical Histopathological Finding

المؤلفون: Rafael H. Pérez-Soto, José Alfredo Álvarez-López, Jorge Montalvo-Hernández, Rafael Silva

المصدر: Indian Journal of Surgery. 83:1286-1289

الوصف: Adrenal gland extramedullary hematopoiesis is extremely rare, and when present, imaging studies may mimic an incidentaloma suspicious of malignancy. We present a 55-year-old woman without a history of hematologic disorder or neoplasms. There was an incidental finding of a 3.2 × 2.4-cm heterogenous adrenal mass and enhanced by contrast abdominal CT scan. Suspicious of malignancy was established, and laparoscopic left adrenalectomy was performed. Histopathological examination and immunohistochemical staining exhibited myeloid and erythroid precursors, as well as megakaryocyte confirming the diagnosis of extramedullary hematopoiesis in the adrenal gland.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_________::a8f6eeaf42a93b25b3ef50041ab9a0c3Test
https://doi.org/10.1007/s12262-020-02661-6Test

10

Management of pyruvate kinase deficiency in children and adults

المؤلفون: Rachael F. Grace, Wilma Barcellini

المصدر: Blood. 136:1241-1249

مصطلحات موضوعية: Male, 0301 basic medicine, Hemolytic anemia, medicine.medical_treatment, Disease, Pyruvate Metabolism, Inborn Errors, Biochemistry, 0302 clinical medicine, Cholelithiasis, Pregnancy, Prevalence, Child, Clinical Trials as Topic, Hematopoietic Stem Cell Transplantation, Disease Management, Anemia, Hemolytic, Congenital Nonspherocytic, Hematology, Gallstones, Hemolysis, Jaundice, Neonatal, Extramedullary hematopoiesis, Fetal Diseases, Child, Preschool, Splenectomy, Female, Pyruvate kinase deficiency, Adult, medicine.medical_specialty, Iron Overload, Adolescent, Genotype, Anemia, Pyruvate Kinase, Immunology, Iron Chelating Agents, 03 medical and health sciences, medicine, Humans, Blood Transfusion, Intensive care medicine, business.industry, Infant, Newborn, Infant, Genetic Therapy, Cell Biology, medicine.disease, Chelation Therapy, 030104 developmental biology, Mutation, Splenomegaly, business, 030215 immunology

الوصف: Pyruvate kinase deficiency (PKD) is an autosomal-recessive enzyme defect of the glycolytic pathway that causes congenital nonspherocytic hemolytic anemia. The diagnosis and management of patients with PKD can be challenging due to difficulties in the diagnostic evaluation and the heterogeneity of clinical manifestations, ranging from fetal hydrops and symptomatic anemia requiring lifelong transfusions to fully compensated hemolysis. Current treatment approaches are supportive and include transfusions, splenectomy, and chelation. Complications, including iron overload, bilirubin gallstones, extramedullary hematopoiesis, pulmonary hypertension, and thrombosis, are related to the chronic hemolytic anemia and its current management and can occur at any age. Disease-modifying therapies in clinical development may decrease symptoms and findings associated with chronic hemolysis and avoid the complications associated with current treatment approaches. As these disease-directed therapies are approved for clinical use, clinicians will need to define the types of symptoms and findings that determine the optimal patients and timing for initiating these therapies. In this article, we highlight disease manifestations, monitoring approaches, strategies for managing complications, and novel therapies in development.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9f1b9a063cfbb43e43bbcac7b823a7d7Test
https://doi.org/10.1182/blood.2019000945Test

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