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]

School of Medicine and Health Sciences Poster Presentations Title Secondary Myelofibrosis with Pericardial Extramedullary Hematopoiesis: A Unique Case of Polycythemia Vera Progression Keywords extramedullary hematopoiesis; polycythemia vera; myelofibrosis [hsrc.himmelfarb.gwu.edu]

• 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 [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]

• High Fever

  fever, spleen degree of light, lymph node enlargement and other characteristics, the patient's tuberculosis serious and broadcast Scattered, shorter course of disease. [healthfrom.com]

• Hoarseness

  The patient reported increasing weakness in his upper and lower extremities without pain over the course of 4 days, in addition to difficulty moving, swallowing, mild hoarseness, urinary retention, and constipation. [oncologynurseadvisor.com]

• Myopathy

  Dermatomyositis (DM) is an idiopathic autoimmune myopathy characterized by muscle weakness and purple-pigmented rashes. [oncologynurseadvisor.com]

• Urinary Retention

  The patient reported increasing weakness in his upper and lower extremities without pain over the course of 4 days, in addition to difficulty moving, swallowing, mild hoarseness, urinary retention, and constipation. [oncologynurseadvisor.com]

(See Presentation, Workup, and Treatment .) Splenic infarction may occur and results in an acute or subacute onset of severe pain in the left upper quadrant that may be associated with nausea, fever, and referred left shoulder discomfort. [emedicine.medscape.com]

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]

Of patients with Budd-Chiari syndrome (hepatic-inferior vena cava obstruction), 10% to 13% have coexisting PV; therefore, testing for the presence of JAK2 V617F mutation is part of the routine workup for unexplained liver thrombosis. [clevelandclinicmeded.com]

• Decreased Platelet Count

  The study by Tefferi et al. [14] also showed some similarities with our study; they found older age group, normal to high platelet count and blast percentage et al. [15] was also in line with our observations. [ijpmonline.org]

  Decreasing platelet counts in ET to below 600 x 10 9 /L can decrease thrombotic complications. 46 High platelet counts (>1,000 x 10 9 /L) are associated with acquired von Willebrand disease resulting from the adsorption of von Willebrand multimers onto [clevelandclinicmeded.com]

• Mycobacterium Avium Complex

  We observed two (3%) mycobacterial infections (one due to Mycobacterium tuberculosis and one due to Mycobacterium avium complex) in our cohort of 65 patients receiving ruxolitinib. [ncbi.nlm.nih.gov]

• 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

  She was subjected to bone marrow examination. Bone marrow aspiration revealed a dry tap. [ijcasereportsandimages.com]

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]

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]

Determine DIPSS risk category and prognosis DIPSS score DIPSS risk category Median OS (y) 0 Low Not reached 1 - 2 Intermediate-1 14.2 3 - 4 Intermediate-2 4 5 - 6 High 1.5 IWG-MRT prognostic score for myelofibrosis Applies at the time of diagnosis. 1. [bloodref.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]

Prognosis is poor, with slow progression and death usually within 2-3 years. [radiopaedia.org]

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]

HLA-mismatched donors, lower platelet count ( 4 /μl), and previous blastic transformation were associated with a significantly worse prognosis. [jstage.jst.go.jp]

MVD and MSA were significantly increased in fibrotic marrows regardless of etiology. [ncbi.nlm.nih.gov]

[…] the underlying etiology. [icd10data.com]

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]

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]

Etiology The etiology of chronic IMF is unknown. Nonrandom chromosome abnormalities such as 9p, 20q-, 13q-, trisomy 8 or 9, or partial trisomy 1q are common, but no cytogenetic abnormality specific to the disease has been identified. [theoncologyinstitute.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]

[…] 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]

Epidemiology The possible association between PH and CMPDs has been suggested by several case reports and small case series. [err.ersjournals.com]

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]

[…] 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]

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]

[…] with myeloid metaplasia, idiopathic myelofibrosis Epidemiology Often occurs in sixth and seventh decades No gender preference Sites Blood and bone marrow Spleen, in cases with EMH Other less common sites include liver, kidney, gastrointestinal tract Pathophysiology [pathologyoutlines.com]

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]

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]

[…] way to prevent myelofibrosis, except in cases caused by exposure to radiation or industrial chemicals. [healthcommunities.com]