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Secondary Myelofibrosis


Presentation

  • The authors present the case of an infant with severe rickets and secondary myelofibrosis and review the differential diagnosis of hepatosplenomegaly from the viewpoint of the pediatric hematologist/oncologist.[ncbi.nlm.nih.gov]
  • We report a 15-year-old boy presented with generalized weakness, vomiting, and pallor. A parathyroid adenoma was detected on the left distal parathyroid gland. PHP was diagnosed together with hepatosplenomegaly and pancytopenia.[ncbi.nlm.nih.gov]
  • Recently, a rare case of pancytopenia caused by myelofibrosis in a 41-year-old woman who complained of general weakness and arthralgia presented to our clinical service. The patient was diagnosed with primary hyperparathyroidism with pancytopenia.[ncbi.nlm.nih.gov]
  • Loss of the BRCA2 locus was present in a single case only. Our results indicate that cryptic deletions of the 13q14 in myelofibrosis are rare.[ncbi.nlm.nih.gov]
  • METHODS: Seventeen patients with chronic myelogenous leukemia and 3 with idiopathic polycythaemia presented with splenomegaly, splenic pain and anemia.[ncbi.nlm.nih.gov]
Gaucher Disease
  • […] various pathological processes that involve the bone marrow Causes of secondary myelofibrosis: Neoplastic – infiltration by cancer cells (primary is usually located in breast, lung or prostate) Infective – tuberculosis, fungal infections, HIV Metabolic – Gaucher’s[pgblazer.com]
Large Pericardial Effusion
  • A bedside echocardiogram in the emergency room revealed a large pericardial effusion with features concerning for pericardial tamponade.[hsrc.himmelfarb.gwu.edu]
Cesarean Section
Clumsiness
  • […] to 2002 ECP criteria in Table 5 , hypercellular ET associated with prefibrotic CIMF (“false” ET) or PMGM is dominated by an increase of clustered atypical dysmorphic megakaryocytes due to increases of cellular and nuclear size and bulky nuclei with clumsy[omicsonline.org]
Babinski Sign
Limb Weakness
  • Case presentation The patient was a 36-year-old G2P1 patient at 28 weeks’ gestation whose chief complaint was fatigue for more than 20 days accompanied by vomiting, diarrhea, and cough with sputum for 10 days and right limb weakness for more than 3 days[bmccancer.biomedcentral.com]

Workup

  • Hyperuricemia is due to enhanced turnover of hematopoietic tissue. 26 Mutation testing Testing for the JAK2 V617F mutation should be performed early during the diagnostic workup of suspected MPNs.[mpncanada.com]
Decreased Platelet Count
  • platelet count, increased bone marrow fibrosis, older age, and longer duration of disease. [1] , [10] , [11] , [12] We found no significant correlation of leukocytosis, higher blast percentage, spleen size, presence of constitutional symptoms, and grade[ijpmonline.org]
Pleural Adhesions
  • He was found to have dense pleural adhesions, with 700ml of sanguinous fluid drained from the pericardial space.[hsrc.himmelfarb.gwu.edu]
Dry Tap Bone Marrow

Treatment

  • RESULTS: The patients showed excellent response to treatment one month post-radiotherapy, while treatment was well tolerated without severe toxicity. The dimensions of the spleen decreased significantly.[ncbi.nlm.nih.gov]
  • Thus, RIST for an older patient with secondary myelofibrosis was successful without severe treatment-related morbidity. This case suggests that RIST could be an effective treatment modality for secondary myelofibrosis.[ncbi.nlm.nih.gov]
  • Treatment Treatment of secondary myelofibrosis treatment Department of Internal Medicine: Department of Hematology Treatment: drug treatment support treatment of surgical treatment Treatment cycle: 1-3 months Cure rate: 80% (A) treatment The disease can[healthfrom.com]
  • The skin lesion almost disappeared after treatment with hydroxyurea. We report here this rare manifestation in secondary myelofibrosis including a review of literature.[ncbi.nlm.nih.gov]
  • Ruxolitinib is a JAK-1/JAK-2 inhibitor indicated for the treatment of polycythemia vera and primary or secondary myelofibrosis.[ncbi.nlm.nih.gov]

Prognosis

  • HLA-mismatched donors, lower platelet count ( 4 /μl), and previous blastic transformation were associated with a significantly worse prognosis.[jstage.jst.go.jp]
  • Also, these cases may represent a clonal disorder with genetic instability allowing evolution to a more aggressive malignancy. [13] The prognosis in our patient could not be judged following ATT since the child was lost to follow up.[ijcasereportsandimages.com]
  • […] contrary, such as bone marrow fibrosis Metastatic cancer and other malignant diseases, myelinated lesions progress faster, poor prognosis.[healthfrom.com]
  • Mutations and prognosis in primary myelofibrosis . Leukemia 2013; 27 : 1861–1869. 11. Rumi E , Pietra D , Pascutto C , Guglielmelli P , Martinez-Trillos A , Casetti I et al .[nature.com]
  • What is the Prognosis for MF? There is no single prognosis for people who suffer from myelofibrosis– the prognosis of MF is different for every patient.[mpnresearchfoundation.org]

Etiology

  • MVD and MSA were significantly increased in fibrotic marrows regardless of etiology.[ncbi.nlm.nih.gov]
  • This case highlights an unusual etiology for pericardial effusion and tamponade, and further research should be considered with regards to JAK2 mutations in cases of myelofibrosis.[hsrc.himmelfarb.gwu.edu]
  • Introduction Etiology Prevention Complication Symptom Examine Diagnosis Treatment Basic Nursing Introduction Introduction to secondary myelofibrosis Secondary myelofibrosis refers to a variety of different causes of bone marrow hematopoietic tissue fibrosis[healthfrom.com]
  • Discussion The phenotypes of ET in various MPDs (MPNs) according to bone marrow features based on cellularity and megakaryocyte morphology and molecular etiology can be diagnosed as JAK2 V617F -postive normocellular ET and ET with features of PV in the[omicsonline.org]
  • Even though the discovery of the Janus kinase 2 (JAK2), thrombopoietin receptor (MPL) and calreticulin (CALR) mutations have brought new insights into the complex pathogenesis of MPNs, the etiology of fibrosis is not well understood.[einstein.pure.elsevier.com]

Epidemiology

  • […] epigenetic, immuno- well as for practicing hematologists or oncologists. logical and biological causes of myeloproliferative dis- Each chapter follows a similar architecture and leads orders has substantially improved in the last few years. through epidemiology[books.google.com]
  • […] permission PEIR, University of Alabama at Birmingham, Department of Pathology Myelofibrosis Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Myelofibrosis from other Diseases Epidemiology[wikidoc.org]
  • Epidemiology and clinical relevance of mutations in post-polycythemia vera and post-essential thrombocythemia myelofibrosis. A study on 359 patients of the AGIMM group . Am J Hematol 2016; 91 : 681–686. 7.[nature.com]
  • […] granulocytes in bone marrow, marrow fibrosis, extramedullary hematopoiesis (EMH) Terminology Also called agnogenic (idiopathic) myeloid metaplasia, chronic idiopathic myelofibrosis, myelofibrosis / sclerosis with myeloid metaplasia, idiopathic myelofibrosis Epidemiology[pathologyoutlines.com]
  • Epidemiology of myeloproliferative neoplasms in the United States. Leukemia & Lymphoma. 2013;Early online: 1-6.[mpninfo.org]
Sex distribution
Age distribution

Pathophysiology

  • […] prostate) Infective – tuberculosis, fungal infections, HIV Metabolic – Gaucher’s disease Hematological malignancies – chronic myeloid leukemia, multiple myeloma, lymphomas Radiation, radiomimetic drugs Others – Sarcoidosis, congenital osteopetrosis Pathophysiology[pgblazer.com]
  • […] higher in frequency in patients of tuberculosis than in general population. [10] The pathogenetic mechanism that links myelofibrosis and tuberculosis remains unclear but the possible role of transforming growth factor-β (TGF-β) has been postulated in the pathophysiologic[ijcasereportsandimages.com]
  • Image courtesy of Professor Peter Anderson DVM PhD and published with permission PEIR, University of Alabama at Birmingham, Department of Pathology Myelofibrosis Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology[wikidoc.org]
  • Metastatic carcinoma or infections with mycobacteria can also cause myelofibrosis. return to top Pathophysiology Fibrosis progressively displaces hematopoietic elements, inculding stem cells, from the marrow.[sharinginhealth.ca]
  • Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres. Br J Haematol 2007;139:351–62. Web of Science Google Scholar 5. Farolino DL, Rustagi PK, Currie MS, Doeblin TD, Logue GL.[degruyter.com]

Prevention

  • Prevention Prevention of secondary myelofibrosis The disease no effective preventive measures, early detection of early diagnosis is the key to prevention and treatment of this disease.[healthfrom.com]
  • Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case # 1 Related chapters Myelophthisic anemia[wikidoc.org]
  • CTFPHC REFERENCE BOOK Screening for Colorectal Cancer CTFPHC REFERENCE BOOK Screening for Cognitive Impairment CTFPHC REFERENCE BOOK Screening for Depression CTFPHC REFERENCE BOOK Screening for Developmental Delay CTFPHC Review Canadian Task Force on Preventive[qxmd.com]
  • Over time this destroys the normal bone marrow environment, preventing the production of adequate numbers of red cells, white cells and platelets.[leukaemia.org.au]
  • Neurologists Type 2 Diabetes and Cardiovascular Disease: Risk Factors Managing Asthma With Biologic Therapy Selecting and Using a GLP-1 Agonist to Treat T2D Potential of Cannabidiol in Epileptic Encephalopathies Schizophrenia: Quality of Care & Relapse Prevention[raredr.com]

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