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]

• 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]

• 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]

• Macroglossia

  Clinical examination of the patient revealed features of Down's syndrome like short stature, macroglossia, low set ears, simian crease and delayed milestones. Abdominal examination revealed palpable splenomegaly two cm below the costal margin. [ijcasereportsandimages.com]

• Low Set Ears

  Clinical examination of the patient revealed features of Down's syndrome like short stature, macroglossia, low set ears, simian crease and delayed milestones. Abdominal examination revealed palpable splenomegaly two cm below the costal margin. [ijcasereportsandimages.com]

• Myopathy

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

• Purpura

  All nine of those patients had thrombotic thrombocytopenic purpura (TTP). Among those patients, two patients received chemotherapy prior to the development of TTP. [ncbi.nlm.nih.gov]

• Cesarean Section

  She had a history of cesarean section in 2004 and pelvic fracture in 2006. [bmccancer.biomedcentral.com]

• Clumsiness

  According 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

  Right Babinski sign was positive. [bmccancer.biomedcentral.com]

• 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]

(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

  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]

  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

  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]

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]

[…] 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]

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]

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]

[…] the underlying etiology. [icd10data.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]

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

[…] 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]

[…] 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]

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]