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Journal of Medical Case Reports Nov 2021Primary myelofibrosis is a rare myeloproliferative disorder in middle-aged and old adults and should be distinguished from secondary and reactive causes of bone marrow...
BACKGROUND
Primary myelofibrosis is a rare myeloproliferative disorder in middle-aged and old adults and should be distinguished from secondary and reactive causes of bone marrow fibrosis because, in reactive fibrosis, treatment approaches depend on the underlying etiology.
CASE PRESENTATION
Here we report the case of a middle-aged Iranian man who was diagnosed and treated as primary myelofibrosis at presentation, and whose final diagnosis was disseminated tuberculosis with reactive bone marrow fibrosis.
CONCLUSIONS
It is prudent to evaluate the potential causes of myelofibrosis in any patient with the diagnosis primary myelofibrosis. Tuberculosis can be an important etiology of bone marrow fibrosis, especially in endemic areas.
Topics: Adult; Humans; Iran; Male; Middle Aged; Primary Myelofibrosis; Tuberculosis
PubMed: 34749829
DOI: 10.1186/s13256-021-03038-3 -
Acta Haematologica 2016
Topics: Age Factors; Aged; Aged, 80 and over; Biomarkers; Erythrocyte Indices; Hemoglobins; Humans; Leukocyte Count; Middle Aged; Primary Myelofibrosis; Prognosis; Proportional Hazards Models; Risk Factors
PubMed: 27189016
DOI: 10.1159/000445247 -
Clinical Orthopaedics and Related... 1967
Topics: Aged; Anemia; Blood Cells; Blood Chemical Analysis; Bone Marrow Diseases; Diagnosis, Differential; Female; Humans; Male; Middle Aged; Primary Myelofibrosis; Radiography
PubMed: 6048921
DOI: 10.1097/00003086-196700520-00009 -
Medicina Clinica Sep 1989
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European Journal of Haematology Jun 2016
Topics: Erythrocyte Transfusion; Erythropoiesis; Humans; Iron Chelating Agents; Iron Overload; Primary Myelofibrosis; Treatment Outcome
PubMed: 26572646
DOI: 10.1111/ejh.12702 -
Blood Cancer Journal Dec 2017
Topics: Adult; Aged; Female; Humans; L-Lactate Dehydrogenase; Male; Middle Aged; Primary Myelofibrosis; Prognosis; Survival Analysis
PubMed: 29249804
DOI: 10.1038/s41408-017-0024-9 -
Leukemia & Lymphoma Mar 2002A patient with primary biliary cirrhosis (PBC) who developed idiopathic myelofibrosis (IM) is reported. The initial diagnosis of PBC was established by liver biopsy,...
A patient with primary biliary cirrhosis (PBC) who developed idiopathic myelofibrosis (IM) is reported. The initial diagnosis of PBC was established by liver biopsy, performed after a 2-month history of constitutional symptoms associated with abnormalities of the serum liver enzymes, with typical serum immunological markers being found. Although a favorable response of PBC to prednisone was observed, one and a half year later the patient developed anemia with anisocytosis and poikilocytosis, tear-drop cells, and leukoerythroblastic picture, and IM was diagnosed by bone marrow biopsy. A few months later, a rapid worsening of the patient's clinical condition was noted, with an increase in the constitutional symptoms and need for frequent packed RBC transfusions, and she finally died from an infectious complication. This case represents a new association of IM with an autoimmune disease, supporting the hypothesis of a possible immune basis of IM in some cases.
Topics: Aged; Autoimmune Diseases; Fatal Outcome; Female; Humans; Liver Cirrhosis, Biliary; Primary Myelofibrosis
PubMed: 12002780
DOI: 10.1080/10428190290012272 -
Modern Rheumatology Case Reports Jan 2023Pachydermaperiostosis (PDP) is a rare condition of connective tissue presenting with abnormal skin and skeletal findings that usually occur as a complication of an...
Pachydermaperiostosis (PDP) is a rare condition of connective tissue presenting with abnormal skin and skeletal findings that usually occur as a complication of an underlying disease, especially malignancies. We described a case of a patient with severe transfusion-dependent anemia and both skin and joint findings, diagnosed as PDP. The haematological assessment revealed myelofibrosis as the underlying disease, and treatment with ruxolitinib as the first-line choice was given by skipping hydroxyurea due to pancytopenia. The patient got benefits in arthralgia and amelioration of pancytopenia and a reduced spleen volume was observed, despite the negative result for JAK2 gene mutation. This is the first case of ruxolitinib being used in PDP with myelofibrosis, and it highlights the importance of evaluating PDP as not just a skin and joint problem but a result of systemic disease and individual-based treatment.
Topics: Humans; Primary Myelofibrosis; Pancytopenia; Nitriles; Pyrimidines
PubMed: 36208298
DOI: 10.1093/mrcr/rxac076 -
International Journal of Hematology Aug 2002Myelofibrosis with myeloid metaplasia (MMM) encompasses the diagnoses of agnogenic myeloid metaplasia (idiopathic myelofibrosis), as well as the advanced phases of... (Review)
Review
Myelofibrosis with myeloid metaplasia (MMM) encompasses the diagnoses of agnogenic myeloid metaplasia (idiopathic myelofibrosis), as well as the advanced phases of polycythemia vera and essential thrombocythemia (post polycythemic and post thrombocythemia myeloid metaplasia, respectively). MMM is a clonal, hematopoietic stem cell disorder in which neither the pathogenesis, nor a broadly applicable effective therapy have been described. Clinically, these patients experience progressive marrow replacement by fibrotic tissue, ineffective hematopoiesis, problematic cytopenia's, significant hepato-splenomegaly, extramedullary hematopoiesis, profound constitutional symptoms, and a risk of blastic transformation. Historically, therapies have been targeted at palliating symptoms (i.e. splenectomy, transfusions, hydroxyurea, erythropoietin, androgens, localized radiotherapy). Stem cell transplantation appears promising, but is often toxic and not broadly applicable due to co-morbidities and age of MMM patients. Non-myeloablative approaches to conditioning may broaden the applicability of stem cell transplantation in MMM, yet results to date are preliminary. Although a definitive molecular abnormality responsible for the pathogenesis of MMM has not been described, much has been learned about the aberrant expression of pro-fibrotic cytokines and the presence of increased angiogenesis in MMM. These pathogenetic insights have led to a series of pilot clinical trials with therapeutic agents targeting aberrantly expressed cytokines (and possibly angiogenesis) including Thalidomide (alone or in combination), Etanercept, and STI-571. Amongst these later agents Thalidomide has demonstrated the most promise (palliating disease associated cytopenia's), whereas the TNF-alpha inhibitor Etanercept has aided with MMM associated constitutional symptoms. Although these later trials have been helpful in a subset of patients, no agent to date has led to solid complete responses in MMM across the spectrum of disease manifestations. Further insights into the pathogenetic mechanisms responsible for myeloproliferation (aberrant cell signaling pathways, apoptotic resistance, other) are necessary to guide selection and testing of the expanding number of novel anti-neoplastic agents in chronic myeloid disorders and MMM.
Topics: Combined Modality Therapy; Humans; Primary Myelofibrosis; Treatment Outcome
PubMed: 12430941
DOI: 10.1007/BF03165138 -
Diagnosis and evaluation of prognosis of myelofibrosis: A British Society for Haematology Guideline.British Journal of Haematology Jan 2024
Topics: Humans; Primary Myelofibrosis; Prognosis; Hematology
PubMed: 37932932
DOI: 10.1111/bjh.19164