Osteomyelofibrose
Myelodysplastisches
Syndrom mit Myelosklerose: links T1, rechts T2. Beim MDS würde man eigentlich ein niedriges T1-Signal und ein hohes T2-Signal erwarten. Das hier auch niedrige T2-Signal legt den Verdacht einer begleitenden Myelosklerose nahe.
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. 99mTc Tc-99 m isotope HDP nuclear bone scan in a different patient demonstrates an appearance approaching a ‘superscan’ appearance due to intense skeletal tracer distribution. This case also provides a clue to the cause of skeletal uptake: note that the left kidney has been displaced inferiorly by an enlarged spleen from marked splenomegaly (black arrow). 99mTc-99 m nuclear bone scans may be helpful in identifying marrow presence in extramedullary sites of haematopoiesis
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Presacral extramedullary haematopoiesis. a Sagittal. b T2. c Pre-contrast T1. d Post-contrast T1FS. e Axial post-contrast T1FS. f Fusion PET/CT with colour map. FDG-18 PET/CT MIP reconstruction demonstrating a presacral lesion with increased FDG uptake, consistent with presacral extramedullary haematopoiesis. Note the ‘superscan’ appearance from diffuse FDG uptake in the axial and appendicular skeleton (f)
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT of the abdomen demonstrates symmetrical bulky and homogenous low attenuating masses in the adrenal glands (white arrows), consistent with extramedullary haematopoiesis in the adrenal glands. Note also similar attenuation bulky perihilar soft tissue consistent with periportal extramedullary haematopoiesis and splenomegaly (asterisk)
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Chest radiograph demonstrating diffuse osteosclerosis in a patient with myelofibrosis. The symmetrical and diffuse appearances of osteosclerosis on plain radiographs are often subtle and difficult to identify
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Lateral radiograph of a 62-year-old male with known primary myelofibrosis demonstrating endosteal sclerosis, the osteosclerotic pattern of myelofibrosis in the long bones (white arrows)
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. AP radiograph in the same patient demonstrates mild periostitis at the proximal tibial metaphysis, a rare feature of myelofibrosis (white arrows). Periostitis in myelofibrosis occurs at the metaphyseal regions of the distal femur or proximal tibia
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. 18F-FDG PET/CT MIP image of a patient with myelofibrosis demonstrates diffuse FDG uptake in the bones. Note the presence of hepatosplenomegaly in the patient
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. 18F-FLT PET in a patient with myelofibrosis demonstrating diffuse increased tracer uptake in the bones and enlarged spleen. 18F-FLT PET is an excellent alternative to FDG due to the ability to directly assess myeloproliferative activity without the superimposed inflammatory component of disease. 18F-FLT PET can also be used to determine disease status or treatment outcome when performed at baseline
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT demonstrating bilateral lobular perirenal soft tissue masses, contained by Gerota’s fascia and often not causing contour deformity against the kidneys (white arrows), consistent with perirenal extramedullary haematopoiesis, a common site of extramedullary haematopoiesis in the abdomen
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Sagittal T1 (a) and T2 (b) MRI of the lumbar spine in a normal patient demonstrating normal marrow appearances. The vertebrae are homogenous, and the intervertebral disks are hypointense relative to the marrow. In contrast, the marrow signal in myelofibrosis appears markedly hypointense on both T1 (c) and T2 (d). The intervertebral disks also appear hyperintense relative to the marrow signal
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Portovenous phase axial CT demonstrating Budd-Chiari syndrome in a patient with myelofibrosis. The middle hepatic vein (black arrow) and left hepatic vein (white arrow) are occluded and non-enhancing, and the inferior vena cava (not shown) is non-opacifying and slit-like. Note the early ‘nutmeg liver’ appearance in segment 7 which is a typical feature of Budd-Chiari syndrome
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial (a) and coronal (b) CT demonstrating a filling defect within the portal vein (white arrows). Note the presence of splenomegaly in the patient (asterisk) and multiple splenic varices
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT in the same patient demonstrates a filling defect within a branch of the superior mesenteric vein. Myeloproliferative neoplasms are now recognised as the leading systemic cause of splanchnic vein thrombosis [19] and affecting a younger age group in general
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Coronal T2 MRI (a) demonstrating a filling defect in the right transverse sinus (white arrow). Maximum intensity projection (MIP) reconstruction of an MRI intracranial venogram (b) demonstrates complete non-opacification of the right transverse sinus. This was due to a chronic right transverse sinus thrombosis in a patient with myelofibrosis
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT intracranial angiogram demonstrates a filling defect within the basilar artery (black arrow) in a patient with myelofibrosis. Other multiple filling defects were seen in both vertebral arteries (not shown). Arterial embolisms are common in myelofibrosis and can result in transient ischaemic events, cerebrovascular accidents, angina or myocardial infarctions, and peripheral vascular disease
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial portal venous phase CT of the abdomen demonstrates a markedly enlarged spleen with multiple wedge-shaped and linear areas of low attenuation consistent with splenic infarctions (white arrows). Marked enlargement of the spleen and liver may result in infarction, portal hypertension, hypersplenism, plasma volume expansion, and splanchnic vein thrombosis
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. T2-weighted MRI demonstrating bilateral posterior mediastinal masses (white arrows) consistent with extramedullary haematopoiesis. The soft tissue has intruded the right neural exit foramen and into the vertebral canal, causing effacement and mild displacement of the thoracic cord (black arrow)
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Coronal CT demonstrating bilateral lobular perirenal soft tissue masses, contained by Gerota’s fascia and not causing contour deformity against the kidneys (white arrows), consistent with perirenal extramedullary haematopoiesis, a common site of extramedullary haematopoiesis in the abdomen. Note also the presence of periportal extramedullary haematopoiesis (black arrow) and splenomegaly (asterisk) in addition to osteosclerosis
Primary
myelofibrosis • Myelofibrosis - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis • Myelofibrosis - Ganzer Fall bei Radiopaedia
Bone tumors
• Myelofibrosis - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis • Myelofibrosis - Ganzer Fall bei Radiopaedia
Splenomegaly
• Splenic infarction - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis • Myelofibrosis (massive splenomegaly with infarct) - Ganzer Fall bei Radiopaedia
Subdural
hemorrhage • Acute subdural hematoma with myelofibrosis - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis • Myelofibrosis - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis • Myelofibrosis - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis • Myelofibrosis - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT demonstrating potential sites of extramedullary haematopoiesis (EMH) in the body. The commonest sites of involvement are the liver and spleen which manifest as hepatosplenomegaly. Less commonly, EMH may present in the periportal region (white arrows) and may be indistinguishable from periportal oedema. Clues to differentiating periportal EMH from periportal oedema include a more lobular appearance to EMH and soft tissue attenuation, whereas periportal oedema is generally less lobular and may have fluid attenuation
Primary
myelofibrosis • Myelofibrosis - Ganzer Fall bei Radiopaedia
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Plain abdominal radiograph demonstrating diffuse increased osteosclerosis and a large left upper quadrant shadow consistent with massive splenomegaly (asterisk) in a patient with known primary myelofibrosis. There is marked displacement of the large and small bowel to the right by the spleen
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Coronal CT of the abdomen demonstrating a markedly enlarged spleen (asterisk). The combination of osteosclerosis and massive splenomegaly has a narrow differential diagnosis and is most suggestive of myelofibrosis
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Coronal CT of the chest demonstrating extramedullary haematopoiesis in the posterior mediastinum, which typically appear as bilateral and symmetrical posterior mediastinal masses (white arrows)
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT of the chest demonstrating extramedullary haematopoiesis in the posterior mediastinum, which typically appear as bilateral and symmetrical posterior mediastinal masses (white arrows). In the paravertebral regions, the soft tissue may compress the exiting nerves in the neural exit foramina or enter the vertebral canal, causing cord displacement or compression
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT of the chest in another patient with myelofibrosis demonstrating soft tissue masses in the left and right intercostal spaces (asterisk), proven extramedullary haematopoiesis on histology. Note the bilateral bulky posterior mediastinal masses, also extramedullary haematopoiesis (white arrows)
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Portovenous phase axial (a) and coronal (b) CT of the abdomen in two different patients with myelofibrosis demonstrating osteosclerosis and massive splenomegaly. Note the presence of splenic varices and cavernous transformation of the portal vein on the axial image (a). Periportal low attenuation is also seen, consistent with periportal oedema and in keeping with portal hypertension. On the coronal image, linear low attenuation at the inferior aspect of the spleen is consistent with splenic infarction, another complication of massive splenomegaly
Primary
myelofibrosis: spectrum of imaging features and disease-related complications. Axial CT of the abdomen demonstrates a chronic subcapsular splenic haematoma in a markedly enlarged spleen (white arrow). Severe organomegaly increases the risk of organ rupture with minor trauma or even spontaneously
Primary myelofibrosis is a myeloproliferative neoplasm in which there is the replacement of bone marrow with collagenous connective tissue and progressive fibrosis. It is characterized by:
- extramedullary hematopoiesis
- progressive splenomegaly
- anemia
- variable change in the number of granulocytes and platelets including thrombocytopenia
Epidemiology
It usually affects the middle-aged to elderly, with a mean age of 60 years. The estimated prevalence is ~1:100,000.
Pathology
It is considered a chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)-negative myeloproliferative disorder .
Non-neoplastic fibroblasts produce collagen, which replaces normal bone marrow elements. This bone marrow fibrosis is a result of an inappropriate release of PDGF and TGF-ß from neoplastic megakaryocytes .
Radiographic features
Most radiological features are a result of extramedullary hematopoiesis and seen in many systems.
General
Musculoskeletal
Abdominal
- hepatomegaly
- splenomegaly: can be massive
- some patients may also experience splenic infarcts
- evidence of portal hypertension
- from increased splenic blood flow
- from portal flow obstruction from the sinusoidal hematopoietic proliferation
Cardiovascular
- may show evidence of congestive cardiac failure due to anemia
Treatment and prognosis
Prognosis is poor, with slow progression and death usually within 2-3 years. It can also transform into acute myeloid leukemia in a small number of patients .
Complications
- gout: from hyperuricemia due to increased hematopoietic turnover
- splenic rupture (rare)
- bleeding from thrombocytopenia (see case 8)
Differential diagnosis
General differential considerations include:
- for musculoskeletal manifestations: consider the differential diagnosis of diffuse bony sclerosis
- for splenic manifestations: consider differential diagnosis for splenomegaly
See also
Siehe auch:
- Splenomegalie
- Sichelzellenanämie
- Tumoren des hinteren Mediastinums
- Thalassämie
- Bürstenschädel
- paraspinal mass
- Hereditäre Sphärozytose
- thoracic paraspinal mass
- diffuse skelettale Sklerosierung
und weiter:
- diffuse bony sclerosis (mnemonic)
- diffus hypointenses Knochenmarksignal in T1
- extramedulläre Hämatopoese
- Knochenmarksveränderungen im MRT
- Superscan Szintigraphie
- Knochenmark
- Knochentumoren
- Knochenmarksveränderungen nach Bestrahlung
- secondary gout
- secondary gout in primary myelofibrosis
- Myeloproliferative Neoplasien (MPN)
Assoziationen und Differentialdiagnosen zu Osteomyelofibrose:
