393 results match your criteria Agnogenic Myeloid Metaplasia With Myelofibrosis
J Cytol 2018 Jul-Sep;35(3):189-192
Department of Pathology, NKP Salve Institute of Medical Sciences and Research Centre, Nagpur, Maharashtra, India.
Extramedullary hematopoiesis (EMH) is a compensatory mechanism that occurs when the marrow is unable to maintain sufficient red cell mass. EMH generally occurs in the patients with deficient bone marrow hematopoiesis secondary to either peripheral red cell destruction or marrow replacement. Although EMH is known to occur in agnogenic myeloid metaplasia with myelofibrosis, chronic myelogenous leukemia, thalassemia, and infiltrative disorders, such as lymphomas, it is rare in acute leukemias. Read More
Biochem Cell Biol 2018 Dec 25;96(6):742-751. Epub 2018 Jun 25.
a Department of Respiratory Medicine, The Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong 250011, People's Republic of China.
Idiopathic pulmonary fibrosis is an agnogenic chronic disorder with high morbidity and low survival rate. Quercetin is a flavonoid found in a variety of herbs with anti-fibrosis function. In this study, bleomycin was employed to induce a pulmonary fibrosis mouse model. Read More
Vet Clin Pathol 2017 Mar 22;46(1):46-53. Epub 2016 Nov 22.
Department of Paraclinical Sciences, Faculty of Veterinary Science, University of Pretoria, Pretoria, South Africa.
A 2.5-year-old spayed female American Pit Bull Terrier dog presented with a primary complaint of chronic refractory ascites. The dog's CBC displayed a moderate to severe macrocytic, hypochromic, nonregenerative anemia, and a moderate leukopenia as result of a moderate neutropenia and monocytopenia. Read More
Zhonghua Xue Ye Xue Za Zhi 2013 Nov;34(11):977-9
Department of Hematopathy, the Affiliated Cancer Hospital of Zhengzhou University, Zhengzhou 450008, China.
Exp Ther Med 2013 May 7;5(5):1516-1518. Epub 2013 Mar 7.
Department of Respiratory Medicine, Taizhou People's Hospital, Taizhou, Jiangsu 225300, P.R. China.
Pulmonary embolism may escape prompt diagnosis since clinical symptoms and signs are nonspecific. The occurrence of syncope as the sole initial symptom in a previously healthy patient with no predisposing factors to embolism and no hemodynamic instability is extremely rare, which may have been a factor in the delayed diagnosis. We describe a case of agnogenic massive pulmonary embolism with syncope as the initial symptom. Read More
Chin J Integr Med 2012 Dec 13;18(12):897-902. Epub 2012 Dec 13.
Institution of Hematology Research, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, China.
Pancytopenia (hemocytopenia) such as pr imary immune primary thrombocytopenia (ITP), aplastic anemia and chronic neutropenia (agnogenic leukocytopenia) were of ten t reated by glucocor t icoids, androgen and often treated glucocorticoids, immunosuppressive agents at present, but the response to these treatments has not been always satisfactory, and may cause serious adverse events. Our research has identified a biological active component in ginseng extract and the active component, panaxadiol saponins component (PDS-C), was isolated from total saponins of ginsenosides, and formulated into capsules named as Painengda. We successfully obtained approval from State Food and Drug Administration (SFDA) of China in 2010 to conduct clinical trials of PDS-C as class-five new Chinese patent medicine. Read More
Eur J Med Res 2011 Jul;16(7):324-7
Department of Ophthalmology, Chinese PLA General Hospital, Beijing 100853, China.
Background: To recognize ocular presentations in cranial venous sinus thrombosis (CVST) which were easy to be misdiagnosis.
Design: Retrospective study.
Methods: Review clinical informations including general informations, general performances, and ocular presentations of 118 inpatients with CVST in the general hospital of chinese people's liberation army during 2005-2009. Read More
Acta Chir Iugosl 2010 ;57(2):79-83
Urolotka sluba KBC Zemun.
In healthy population, uric acid comprises the major component of 10-20% of renal stones. Extreme hiperuricaemia is seen in cancer patients with tumour lysis syndrome (TLS) which is classically associated with haematological malignancies with rapid tumour growth rates such as acute lymphoid leukaemia and high grade lymphomas. Primary melofibrosis (Agnogenic myeloid metaplasia-AMM) is a chronic myeloproliferative disease characterized by splenomegaly, a leukoerythroblastic blood picture, teardrop poikilocytosis and varying degrees of marrow fibrosis. Read More
Z Gastroenterol 2010 Oct 30;48(10):1215-8. Epub 2010 Sep 30.
Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany.
Background: Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disease, which is characterized by myelofibrosis, osteosclerosis and pancytopenia. Affected patients frequently develop portal hypertension secondary to extramedullary hematopoiesis, which rarely becomes clinically relevant.
Case Report: We here report on a 63-year-old patient with a first presentation of variceal bleeding. Read More
South Med J 2010 Oct;103(10):1075-6
Rev Med Chir Soc Med Nat Iasi 2010 Apr-Jun;114(2):465-9
Universitatea de Medicină şi Farmacie Gr.T. Popa Iaşi Facultatea de Medicină, Disciplina de Obstetrică-Ginecologie.
Unlabelled: Idiopathic myelofibrosis is a rare myeloproliferative disorder characterized by excessive accumulation of connective tissue in the bone marrow in association with anemia, splenomegaly and extramedullary hematopoiesis. A rare case of pregnancy in a patient with agnogenic myeloid metaplasia who carried a term pregnancy is described. In spite of the increased perinatal risks, a favorable outcome was possible with close antepartum surveillance. Read More
Curr Stem Cell Res Ther 2009 Dec;4(4):281-6
Genzyme Genetics, 521 West 57th Street, New York, NY 10019, USA.
Primary Myelofibrosis previously also known as Agnogenic Myeloid Metaplasia or Idiopathic Myelofibrosis is a complex myeloproliferative disease. Although the initial genetic insult that causes uncontrolled proliferation of the defective Hematopoietic Stem Cell/Hematopoietic Progenitor Cell is still elusive, literature is being enriched with reports on the molecular mechanisms that provide growth advantage to the mutant clone and the secondary events that lead to stem cell mobilization, bone marrow fibrosis, osteosclerosis and angiogenesis. Identification of these mechanisms reveals dys-regulation of genes. Read More
Clin Cancer Res 2008 May;14(9):2756-62
Duke University Medical Center, Durham, North Carolina 27710, USA.
Purpose: Deforolimus (AP23573), a novel non-prodrug rapamycin analogue, inhibits the mammalian target of rapamycin, a downstream effector of the phosphatidylinositol 3-kinase/Akt and nutrient-sensing pathways. A phase 2 trial was conducted to determine the efficacy and safety of single-agent deforolimus in patients with relapsed or refractory hematologic malignancies.
Experimental Design: Eligible patients were assigned to one of five disease-specific, parallel cohorts and given 12. Read More
Rev Med Interne 2008 Jul 19;29(7):573-6. Epub 2008 Feb 19.
Service 2BNord, département d'oncologie médicale, centre Léon-Bérard, 28, rue Laennec, 69008 Lyon, France. mrabti
Introduction: Agnogenic myeloid metaplasia, associated with myelofibrosis, is a myeloproliferative disorder. Extramedullary hematopoiesis in the pleura is rare and its prognosis is often severe.
Exegesis: Herein we report a 64-year-old woman, who presented with pleural extramedullary hematopoiesis, treated by hydroxyurea-based chemotherapy with disease control. Read More
Rinsho Ketsueki 2007 Jun;48(6):518-23
Fourth Department of Internal Medicine, Sapporo Medical University School of Medicine.
A 67-years old woman was referred to our hospital in October 1992 with thrombocytopenia and splenomegaly. A bone marrow biopsy revealed decreased cellularity, with moderately increased reticulin fibrosis and discrete dysmorphic megakaryocytes but no signs of dysplasia in the erythroid or the myeloid lineages. The karyotype of the bone marrow cells was t(12;17) (q24;q11). Read More
Cancer Genet Cytogenet 2007 Feb;173(1):57-62
Institute of Hematology, Clinical Center of Serbia, Koste Todorovića 2, 11000 Belgrade, Serbia.
Agnogenic myeloid metaplasia (AMM) or idiopathic myelofibrosis is a chronic myeloproliferative disorder characterized by fibrotic bone marrow, extramedullar haematopoiesis, and a leukoerythroblastic picture in circulating blood. The cytogenetic data on AMM are scanty and no recurring chromosome abnormality has been associated with the natural course of this disease. Trisomy 1q, del(13q), del(20q), and trisomy 8, appear in about two thirds of patients with demonstrable chromosome aberrations. Read More
Leuk Res 2007 Jun 8;31(6):737-40. Epub 2007 Jan 8.
Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
The International Working Group for Myelofibrosis Research and Treatment (IWG-MRT) is comprised of hematologists, hematopathologists, and laboratory scientists and its main goal is to provide a forum for scientific exchange and collaboration. During its first general meeting in April 2006, the IWG-MRT established uniform treatment response criteria for chronic idiopathic myelofibrosis (CIMF); also known as agnogenic myeloid metaplasia (AMM), myelofibrosis with myeloid metaplasia (MMM), and many other names in the hematologic literature. This document summarizes the proceedings from the second meeting of the IWG-MRT, in November 2006, where the group discussed and agreed to standardize the nomenclature referring to CIMF: (i) the term primary myelofibrosis (PMF) was chosen over several other designations including CIMF, AMM, and MMM, (ii) myelofibrosis that develops in the setting of either polycythemia vera (PV) or essential thrombocythemia (ET) will be referred to as post-PV MF and post-ET MF, respectively, and (iii) "leukemic" transformation will be recognized as blast phase disease (PMF-BP, post-PV/ET MF in blast phase). Read More
Exp Hematol 2006 Dec;34(12):1617-23
Division of Hematology/Oncology, Department of Medicine, Maimonides Medical Center, Brooklyn, NY 11219, USA.
Objective: The current study quantified the growth factors in megakaryocytes and monocytes and correlated them to the degree of fibrosis, as there is no quantitative analysis of growth factors from megakaryocytes or monocytes reported in patients with agnogenic myeloid metaplasia (AMM).
Materials And Methods: Megakaryocytes were obtained from cultured blood CD34+ cells. CD14+ cells were sorted by magnetic cell sorting. Read More
Semin Thromb Hemost 2006 Jun;32(4 Pt 2):381-98
AP-HP, Service d'Hematologie Biologique, Hôpital Lariboisière, Denis Diderot, Faculté de Médecine, Paris, France.
Exaggerated erythropoiesis and megakaryocytopoiesis are present at a variable extent in polycythemia vera (PV) and essential thrombocythemia (ET). With the recent discovery of the V617F mutation in the Janus kinase 2 (JAK2) tyrosine kinase in almost all cases of PV and in a subset of patients with ET, studies are now pending to assess the role of this mutation in the hematopoietic cell activation process and/or in the occurrence of thromboses in ET and PV. The JAK2 V617F point mutation makes the normal hematopoietic progenitor cells hypersensitive to thrombopoietin, erythropoietin, and myeloid progenitor cells, leading to trilinear hematopoietic myeloproliferation. Read More
Clin Lab Haematol 2006 Apr;28(2):134-7
Department of Hematology, Sultan Qaboos University, Muscat, Oman.
Allogeneic hematopoietic transplantation is the only currently available therapy that has the potential to cure agnogenic myeloid metaplasia (AMM) or primary myelofibrosis (PMF). Amelioration of fibrosis and eradication of the abnormal clone is thought to occur through the repopulation of marrow by donor-derived hematopoiesis and graft-vs.-host reaction leading to graft vs. Read More
Cancer 2006 May;106(9):1985-9
Division of Hematology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Background: Postpolycythemic (PV) and postthrombocythemic (ET) myeloid metaplasia are consensually referred to as secondary myelofibrosis (sMF). Prognostic variables in sMF are not as well defined as they are for de novo myelofibrosis with myeloid metaplasia (MMM), which is also known as agnogenic myeloid metaplasia (AMM). Such information is particularly crucial for management decisions in transplant-eligible patients. Read More
Cancer 2006 Feb;106(3):623-30
Division of Hematology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Background: Allogeneic hematopoietic stem cell transplantation is potentially curative in agnogenic myeloid metaplasia (AMM) but is associated with substantial mortality and morbidity that necessitates accurate identification of patients in whom benefit outweighs risk. The current study describes the natural history of AMM in transplant-eligible patients and proposes a new prognostic scoring system that favorably compares with other established models.
Methods: Patients diagnosed with AMM before the age of 60 years and seen at Mayo Clinic were identified and the diagnosis confirmed. Read More
Hematology 2005 Dec;10(6):501-3
University Hospital of Heraklion, Department of Hematology, Heraklion, Crete, Greece.
Agnogenic myeloid metaplasia (AMM) is characterized by bone marrow fibrosis, splenomegaly and leukoerythroblastic anemia and is frequently accompanied by extramedullary hematopoiesis (EMH). Pulmonary interstitial EMH associated with myelofibrosis has rarely been described in the medical literature and is usually fatal. We report the case of a 77-year-old man with agnogenic myeloid metaplasia (AMM) treated with hydroxyurea, who seven years after diagnosis presented with dyspnea and severe hypoxemia. Read More
Leuk Res 2006 Jun 8;30(6):677-80. Epub 2005 Nov 8.
Division of Hematology, 200 First Street SW, Mayo Clinic, Rochester, MN 55905, USA.
Microcytosis is a characteristic laboratory feature for both iron deficiency anemia and thalassemia. It is also infrequently seen in "anemia of chronic disease" that accompanies a spectrum of chronic conditions including rheumatoid arthritis, polymyalgia rheumatica, diabetes mellitus, connective tissue disease, and protracted infection. In addition, there is a well established but pathogenetically obscure association of microcytosis with Hodgkin's lymphoma, Castleman's disease, and renal cell carcinoma. Read More
Br J Haematol 2005 Nov;131(3):320-8
Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
An association between an activating JAK2 mutation (JAK2(V617F)) and BCR/ABL-negative myeloproliferative disorders was recently reported in multiple simultaneous publications. In the current study, mutation analysis for JAK2(V617F) was performed in peripheral blood mononuclear cells (PBMC) from 157 patients with myelofibrosis with myeloid metaplasia (MMM) including 117 with agnogenic (AMM), 22 with postpolycythaemic (PPMM), and 18 with post-thrombocythaemic (PTMM) myeloid metaplasia. The detection rate for JAK2(V617F) was significantly higher in PPMM (91%; homozygous in 18%) compared with either AMM (45. Read More
Semin Oncol 2005 Aug;32(4):359-64
Academic Unit of Haematology, Henry Wellcome Laboratories for Medical Research, Sheffield, UK.
Agnogenic myeloid metaplasia (AMM), a clonal hematopoietic stem cell disorder also known as chronic idiopathic myelofibrosis, is characterized by a polyclonal, or reactive, stromal proliferation, resulting from the inappropriate release of megakaryocyte/monocyte-derived growth factors. Cytogenetic studies indicate that 30% of cases possess a clonal cytogenetic abnormality at diagnosis, a figure that increases to approximately 90% following leukemic transformation. Patients with specific abnormal karyotypes have a poor prognosis and may fail to respond to androgen therapy. Read More
Br J Haematol 2005 Oct;131(2):166-71
Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Polycythaemia vera (PV) is closely associated with both an acquired activating mutation of the JAK2 tyrosine kinase (JAK2(V617F)) in granulocyte-derived DNA and increased granulocyte polycythaemia rubra vera-1 (PRV-1) expression. In order to explore the correlation between these two biological markers and compare their diagnostic utility, mutation analysis for JAK2(V617F) and quantitative measurement of granulocyte PRV-1 expression were performed on the same study sample from 100 participants: 38 with PV, 22 with essential thrombocythaemia (ET), 10 with agnogenic myeloid metaplasia (AMM), 19 with secondary polycythaemia (SP) and 11 healthy volunteers. The respective overall (homozygous) JAK2(V617F) mutational frequencies were 95% (26%), 55% (0%), 30% (0%), 0% and 0%. Read More
Hematology 2005 ;10 Suppl 1:215-23
Department of Hematology, University Hospital Antwerp, Belgium.
The combined use of bone marrow histopathology, biomarkers and clinical features has the potential to diagnose, stage and distinguish early and overt stages of ET, PV and idiopathic myelofibrosis, that has an important impact on prognosis and treatment of MPD patients. As the extension of the PVSG and WHO for ET, PV and agnogenic myeloid metaplasia (AMM), a new set of European clinical and pathological (ECP) criteria clearly distinct true ET from early or latent PV mimicking true ET, overt and advanced polycythemia vera (PV), and from thrombocythemia associated with prefibotic, early fibrotic stages of chronic megakaryocytic granulocytic metaplasia (CMGM) or chronic idiopathic myelofibrosis (CIMF). Cases of atypical MPD and masked PV are usually overlooked by clinicians and pathologists. Read More
Leuk Lymphoma 2005 Sep;46(9):1261-8
Division of Hematology/Oncology, Maimonides Hospital Medical Center, Brooklyn, NY-11219, USA.
Tissue inhibitors of metalloproteinase (TIMP) and matrix metalloproteinases (MMP) are key elements in the formation, remodeling and degradation of matrix protein. Bone marrow fibrosis in AMM, with deposition, not only of interstitial and basement membrane collagen but also of fibronectin, vitronectin, laminin and proteoglycans, results from a disturbed balance between synthesis and proteolytic degradation of matrix protein. Although TIMP and MMP play important roles in the development of fibrosing diseases of skin, liver and lung, only a few studies of TIMP and MMP in the formation of bone marrow fibrosis in AMM have been published. Read More
Leuk Res 2006 Jan 28;30(1):47-53. Epub 2005 Jul 28.
Division of Hematology/Oncology, Department of Medicine, Maimonides Medical Center, 953, 49th Street, Brooklyn, NY 11219, USA.
Agnogenic myeloid metaplasia (AMM) is characterized by bone marrow fibrosis and enhanced proliferation of megakaryocytes and CD34+ cells. We have analyzed the factors that could lead to reduced expression of TGF beta1RII in CD34+ cells of AMM patients. Our results demonstrate absence of mutations in the coding region and the promoter of this gene and absence of CpG methylation of its promoter in AMM patients. Read More
Ann Vasc Surg 2005 May;19(3):421-4
Unit of Vascular Surgery, Fundación Hospital Alcorcón, Alcorcón, Madrid 28922, Spain.
We present a case of a lumbar pseudoaneurysm in a 62-year-old patient with myelofibrosis (agnogenic myeloid metaplasia). The patient was submitted to two anesthetic infiltrations for lumbar pain and one biopsy of a suspected hematopoietic site. Subsequently, a 4. Read More
Stem Cells 2005 May;23(5):631-7
Division of Laboratory Medicine, University of California, 513 Parnassus Ave. S864, San Francisco, California 94143, USA.
Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disorder that leads to ineffective hematopoiesis, bone marrow fibrosis, and extramedullary hematopoiesis. The molecular mechanisms underlying the development of this syndrome are currently unknown. Therefore, the aim of this study was to characterize aberrant gene expression in CD34+ hematopoietic stem cells from patients with AMM. Read More
Cell Commun Signal 2005 Feb 3;3(1). Epub 2005 Feb 3.
Division of Hematology/Oncology, Department of Medicine, Maimonides Medical Center, Brooklyn, New York, USA.
BACKGROUND: Agnogenic myeloid metaplasia (AMM) is one of the Philadelphia chromosome negative myeloproliferative disorder and is diagnosed by hyperplasia of atypical megakaryocytes, hepatosplenomegaly, extramedullary hematopoiesis and bone marrow fibrosis. Fibrosis is considered to be a secondary consequence of enhanced levels of fibrogenic growth factors such as TGF beta1, bFGF and PDGF produced by enhanced numbers of megakaryocytes, while the primary cause is considered to be the enhanced proliferation of a defective stem cell. We have previously reported that thrombopoietin (TPO) is elevated in patients with AMM. Read More
Haematologica 2004 Dec;89(12):1454-8
Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA.
Background And Objectives: Previous retrospective studies have indicated markedly increased bone marrow angiogenesis (BMA) in myelofibrosis with myeloid metaplasia (MMM). This issue is further examined in the current prospective study and clinico-pathological correlates sought.
Design And Methods: This was a prospective single institutional study of 66 patients with bone marrow biopsy-proven MMM who were consecutively accrued. Read More
J Reprod Med 2004 Sep;49(9):765-8
Department of Pathology, Chang Gung Memorial Hospital Linkou Medical Center, Taoyuan, Taiwan.
Background: Extramedullary hematopoiesis in patients without hematologic disorders is very rare and has apparently never been described previously in association with an ovarian steroid cell tumor.
Case: An 18-year-old woman was admitted to our clinic due to primary amenorrhea. the On examination she had significant facial hirsutism, clitoromegaly and and Adam's apple. Read More
J Eur Acad Dermatol Venereol 2004 Nov;18(6):700-1
Department of Dermatology, Hospital General de Alicante, Spain.
Polycythemia vera is classified with myelogenous leukaemia, agnogenic myeloid metaplasia and primary thrombocythemia as a myeloproliferative syndrome. Cutaneous symptoms have been reported with polycythemia vera, including facial plethora, aquagenic pruritus, urticaria, purpura, Sweet's syndrome and pyoderma gangrenosum. However, polycythemia vera associated with systemic sarcoidosis has been rarely reported. Read More
J Support Oncol 2003 Sep-Oct;1(3):194-205
Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Thalidomide has re-emerged as a novel antineoplastic agent with immunomodulatory and antiangiogenic activities. In the early sixties, it was withdrawn from the market after its infamous association with congenital abnormalities that left about 10,000 children affected world-wide. With strict regulations and precautions, thalidomide is now approved by the FDA for the treatment of erythema nodosum leprosum. Read More
Clin Lab Haematol 2004 Aug;26(4):265-8
Department of Hematology, Hacettepe University Medical School, Ankara, Turkey.
Basic fibroblast growth factor (bFGF) is an important growth factor involved in clonal hematopoietic expansion, neoangiogenesis, and bone marrow fibrosis, all of which are important pathobiologic features of clonal chronic myeloproliferative disorders (CMPD) and myelodysplastic syndromes (MDS). The aim of this study was to assess circulating bFGF concentrations in patients with CMPD and MDS with respect to the presence of bone marrow fibrosis in histopathologic examination. The study group comprised 18 patients with CMPD (six female, 12 male; median age 50 years), seven patients with MDS (one female, six male; median age 66 years) and 10 healthy adults as controls (four female, six male; median age 29 years). Read More
Biochim Biophys Acta 2004 Aug;1689(3):179-81
Department of Pediatrics, Faculty of Medicine, University Magna Graecia of Catanzaro, Ospedale Civile A. Pugliese, Viale Pio X, 88100, Italy.
Carbonic anhydrase family (CAs) plays an important role in the extracellular acidification and several studies suggest a possible involvement of such enzymes in the increased tumor progression due to the acidic extracellular pH. We measured the activities of carbonic anhydrase I and II isoforms in a group of patients affected by four specific chronic haematological diseases, sharing a common origin but characterized by a different neoplastic evolution: agnogenic myeloid metaplasia (AMM), essential thrombocythemia (ET), chronic myeloid leukemia (CML) and polycythemia vera (PV) in order to understand the correlation between CAs activities and neoplastic outcome. In comparison to controls, our data demonstrate an increase of CAI and CAII activities in all our patients with a specific increase of the CAI activity in the group of the diseases with major malignancy (CML and AMM). Read More
Leuk Lymphoma 2004 Apr;45(4):815-8
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Agnogenic myeloid metaplasia (AMM) is one of the myeloproliferative disorders, and is usually accompanied by extramedullary hematopoiesis (EMH) in various organs, mainly in the liver, spleen and lymph nodes. Extramedullary hematopoiesis and/or leukemic transformation of EMH in the pleura is a rare occurrence and is usually asymptomatic. Pleural involvement is usually diagnosed on postmortem examination. Read More
Cancer 2004 May;100(10):2064-78
Department of Leukemia, The University of Texas M.D. Anderson Cancer Center, Houston, Texas 77030-4009, USA.
The myeloproliferative disorders (MPDs) are chronic malignant conditions originating from the clonal expansion of a multipotential hematopoietic stem cell. These diseases include polycythemia vera (PV), essential thrombocythenia, atypical chronic myeloid leukemia, idiopathic hypereosinophilic syndrome (HES), agnogenic myeloid metaplasia with myelofibrosis, and others. Receptor tyrosine kinases-the platelet-derived growth factor receptors (PDGFRs) and c-Kit-and their respective ligands have been implicated in the pathogenesis of MPDs. Read More
Arch Pathol Lab Med 2004 May;128(5):568-70
Department of Pathology, Centre Hospitalo-Universitaire Amiens, Amiens, France.
We report a case of gastric myeloid metaplasia in an 89- year-old woman with agnogenic myeloid metaplasia. The lesions were fortuitously discovered on upper endoscopy. The antral mucosa was thickened and polypoid, and on histologic examination contained immature granulocytes, megakaryocytes, and a few erythroblasts without desmoplastic stromal reaction. Read More
Hematol J 2004 ;5(2):93-102
Goodheart Institute, MPD Center Europe, Rotterdam, The Netherlands.
Clinical, hematological and morphological peripheral blood and bone marrow characteristics, in particular, megakaryopoiesis and bone marrow cellularity, reveal diagnostic clues and pathognomonic features, which enable a clear-cut distinction between essential thrombocythemia (ET), polycythemia vera (PV) and prefibrotic and fibrotic agnogenic myeloid metaplasia (AMM). The characteristic increase of enlarged mature megakaryocytes with mature cytoplasm and multilobulated nuclei and their tendency to cluster in a normal or slightly increased cellular bone marrow represent the hallmark of ET. The characteristic increase and clustering of enlarged mature and pleiomorphic megakaryocytes with multilobulated nuclei and proliferation of erythropoiesis in a moderate to marked hypercellular bone marrow with hyperplasia of dilated sinuses are the specific diagnostic features of untreated PV. Read More
J La State Med Soc 2003 Nov-Dec;155(6):306-13
Department of Internal Medicine, Louisiana State University, School of Medicine, New Orleans, USA.
Essential thrombocytosis is one of the chronic myeloproliferative disorders that includes polycythemia vera, chronic myelogenous leukemia, and agnogenic myeloid metaplasia. Despite established diagnostic criteria and greater than a quarter century of study, there are still several diagnostic and management dilemmas that plague researchers and clinicians alike. We present a case of essential thrombocytosis in a patient with multiple sequelae. Read More
Cancer Genet Cytogenet 2004 Jan;148(1):77-9
Institute of Hematology, Clinical Center of Serbia, Koste Todorovica 2 Street, Belgrade, Serbia, Yugoslavia.
A transformation of essential thrombocythemia to acute myelocytic leukemia (AML), myelodysplastic syndrome, or agnogenic myelocytic metaplasia is a relatively rare event. It occurs in 1%-4.5% of all patients with either treated or untreated essential thrombocythemia. Read More
Asian Pac J Allergy Immunol 2003 Jun;21(2):121-6
Chulabhorn Bone Marrow Transplantation Center, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Agnogenic myeloid metaplasia (AMM) is a clonal hematopoietic stem cell disorder characterized by bone marrow fibrosis, extramedullary hemopoiesis, splenomegaly and a leukoerythroblastic blood picture. Current standard therapies using hydroxyurea, interferon, androgens or corticosteroids have not shown to prolong survival of patients with AMM. In this study, we performed a curative approach using an HLA-matched sibling as a donor for allogeneic peripheral blood stem cell transplantation (PBSCT) for a 45-year-old woman with AMM. Read More
Bone Marrow Transplant 2003 Sep;32(6):581-5
University of California, San Diego School of Medicine, La Jolla, CA, USA.
A 38-year-old woman with agnogenic myeloid metaplasia complicated by the poor prognostic factors of severe osteosclerosis, prominent hepatosplenomegaly, and profound anemia was treated with FLAG chemotherapy to decrease her organomegaly before undergoing a nonmyeloablative allogeneic stem cell transplant from a matched-sibling donor. The patient's pre- and post transplant course were complicated by an autoimmune disorder and her post transplant course was complicated by severe hepatic and gastrointestinal GVHD. A technetium-99m sulfur colloid scan 4 months post transplant and bone marrow studies 8 months post transplant demonstrated intramedullary hematopoiesis, complete resolution of marrow fibrosis, and partial resolution of osteosclerosis. Read More
Cancer 2003 Jul;98(1):100-9
Department of Internal Medicine, Division of Hematology-Oncology, Cornell University Medical School, New York, New York, USA.
Background: In vitro and clinical data suggest a therapeutic role for alpha2 interferon (IFN) in the treatment of the chronic myeloproliferative disorders. Accordingly, a multiinstitutional, Phase II trial of IFN in patients with agnogenic myeloid metaplasia (AMM), essential thrombocythemia (ET), and polycythemia rubra vera (PRV) in the spent phase was initiated. The objectives of this study were 1) to investigate the response rates that may be achieved with IFN in the treatment of patients with these disorders, 2) to estimate the durability of the responses, and 3) to assess the toxicities of IFN in these populations. Read More
Bone Marrow Transplant 2003 Jul;32(1):35-40
Allogeneic Bone Marrow Transplant Program, Princess Margaret Hospital, University Health Network, Toronto, Ont., Canada.
We describe the course of 25 patients with myelofibrosis (MF) due to agnogenic myeloid metaplasia (n=19) or essential thrombocytosis (n=6) who underwent allogeneic stem cell transplantation (SCT) at one of two Canadian centers. The median age at transplantation was 48.7 (IQR 45. Read More
Haematologia (Budap) 2002 ;32(4):495-9
Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman.
We report an unusual case of a 40-year-old female patient with a severe case of direct Coombs positive haemolytic anaemia, moderate hepatomegaly and marked splenomegaly. Her initial response to steroids was transient and was rapidly followed by a relapse. Therefore, she underwent splenectomy both as a therapeutic measure and to rule out an underlying lymphoproliferative disorder. Read More