Extramedullary haematopoiesis within hepatic sinusoids of a patient with primary myelofibrosis

2021 ◽  
Author(s):  
Kazuto Yamazaki ◽  
Shotaro Suzuki ◽  
Takeharu Kawaguchi
Keyword(s):  
Primary Myelofibrosis ◽  
Extramedullary Haematopoiesis

scholarly journals Non-hepatosplenic extramedullary haematopoiesis in primary myelofibrosis

2013 ◽  
Vol 165 (3) ◽  
pp. 271-271
Author(s):  
Federica Lessi ◽  
Gianni Binotto ◽  
Monica Castelli ◽  
Anna Colpo ◽  
Giuseppe Rolma ◽  
...  
Keyword(s):  
Primary Myelofibrosis ◽  
Extramedullary Haematopoiesis

scholarly journals Perinephric extramedullary haematopoiesis in primary myelofibrosis

2012 ◽  
Vol 157 (2) ◽  
pp. 157-157 ◽  
Author(s):  
Lisa Baumann Kreuziger ◽  
Michelle Carlson ◽  
Hector Mesa ◽  
Pankaj Gupta
Keyword(s):  
Primary Myelofibrosis ◽  
Extramedullary Haematopoiesis

scholarly journals Primary Myelofibrosis with Mesenteric Extramedullary Haematopoiesis

2018 ◽  
Author(s):  
Saurabh Kumar ◽  
Shaloo Shaloo ◽  
Juhi Chawla ◽  
Subha Ashok ◽  
Shalini Thapar Laroia
Keyword(s):  
Primary Myelofibrosis ◽  
Extramedullary Haematopoiesis

Primary myelofibrosis

2020 ◽  
pp. 5247-5253
Author(s):  
Evan M. Braunstein ◽  
Jerry L. Spivak
Keyword(s):  
Bone Marrow ◽  
Low Dose ◽  
Bone Marrow Failure ◽  
Allogeneic Bone Marrow Transplantation ◽  
Myeloproliferative Neoplasm ◽  
Primary Myelofibrosis ◽  
Blood Film ◽  
Extramedullary Haematopoiesis ◽  
Night Sweats ◽  
Marrow Fibrosis

Myelofibrosis is a reactive process common to many malignant and benign disorders. Primary myelofibrosis is a chronic myeloproliferative neoplasm arising in a pluripotent haematopoietic stem cell. It results in abnormalities in red cell, granulocyte, and platelet production in association with marrow fibrosis and extramedullary haematopoiesis. While the precise aetiology is unclear, primary myelofibrosis is known to be a clonal disorder caused by acquired genetic mutations in haematopoietic stem cells. Many patients are asymptomatic at the time of diagnosis, but common presenting manifestations include fatigue, weight loss, night sweats, fever, dyspnoea, and abdominal discomfort due to splenomegaly. The major complications are the consequences of bone marrow failure and extramedullary haematopoiesis. About 20% of patients develop acute myeloid leukaemia as a terminal event. Anaemia is the most consistent abnormality, with the blood film showing evidence of a leucoerythroblastic reaction due to extramedullary haematopoiesis. The presence of marrow fibrosis is essential for diagnosis and usually results in the inability to aspirate marrow from a properly placed needle (‘dry tap’). Treatment is aimed at improving symptoms. Splenomegaly is generally the most distressing complication, and the nonselective JAK2 inhibitor, ruxolitinib, is effective in reducing spleen size and alleviating constitutional symptoms in a majority of patients. Patients with good performance status as well as those with advanced stage disease who have a matched, related donor should be considered for allogeneic bone marrow transplantation. Other therapies found to be effective include low-dose interferon, low-dose thalidomide and prednisone, low-dose busulfan, hydroxycarbamide, splenectomy, and splenic irradiation. Folic acid supplementation is often given to prevent deficiency in the context of increased folate requirements, and hyperuricaemia should be treated with allopurinol.

Ineffective Thrombopoiesis

1990 ◽  
Vol 48 (3) ◽  
pp. 266-267
Author(s):  
JM Radley ◽  
SL Ellis
Keyword(s):  
Microscopic Examination ◽  
Phosphate Buffer ◽  
Primary Myelofibrosis ◽  
Transient Increase ◽  
Major Difficulty ◽  
Adult Mice ◽  
Dense Cytoplasm

In effective thrombopoies is has been inferred to occur in several disease sates from considerations of megakaryocyte mass and platelet kinetics. Microscopic examination has demonstrated increased numbers of megakaryocytes, with a typical forms particularly pronounced, in primary myelofibrosis. It has not been documented if megakaryocyte ever fail to reach maturity in non-pathological situations. A major difficulty of establishing this is that the number of megakaryocytes normally present in the marrow is extremely low. A large transient increase in megakaryocytopoiesis can how ever be induced in mice by an injection of 5-fluorouracil. We have utilised this treatment and report here evidence for in effective thrombopoies is in healthy mice.Adult mice were perfused (2% glutaraldehyde in 0.08M phosphate buffer, pH 7.4) 8 days following an injection of 5-fluorouracil (150mg/kg). Femurs were subsequently decalcified in 10% neutral E.D.T.A. and embedded in Spurrs resin. Transverse sections of marrow revealed many megakaryocytes at various stages of maturity. Occasional megakaryocytes (less than 1%) were found to be under going degeneration prior to achieving full maturation and releasing cytoplasm as platelets. These cells were characterized by a peripheral rim of dense cytoplasm which enveloped a mass of organelles and vacuoles (Fig. 1). Numerous microtubules were foundaround and with in the organelle-rich zone (Fig 2).

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