Musculoskeletal

Bone Marrow Disease

Mohamed Zaitoun

Assistant Lecturer-Diagnostic Radiology Department , Zagazig University Hospitals

EgyptFINR (Fellowship of Interventional

Neuroradiology)-Switzerlandzaitoun82@gmail.com

Knowing as much as possible about your enemy precedes successful battle

and learning about the disease process precedes successful management

Mohamed Zaitoun

Radiology Assistant LecturerZagazig University-Egypt

zaitoun82@gmail.com

Bone Marrow Diseasea) Malignant Infiltrationb) Secondary Marrow Hyperplasia c) Lysosomal Storage Diseases

a) Malignant Infiltration : See (Bone Tumors)

1-Myeloma2-Leukemia / Lymphoma3-Metastases (small cell tumors)4-Myelofibrosis

b) Secondary Marrow Hyperplasia :1-Hemoglobinopathies (Sickle Cell Anemia)2-Hemolytic Anemias (Thalassemia)

1-Hemoglobinopathies (Sickle Cell Anemia) :

a) Incidenceb) Radiographic Features

a) Incidence :-Hereditary condition caused by the formation of

abnormal hemoglobin (Hemoglobin S) which manifests as multisystem ischemia and infarction as well as hemolytic anaemia

-The highest incidence occurs in individuals of African descent followed by eastern Mediterranean and Middle Eastern populations

b) Radiographic Features :1-Skeletal Manifestations :a) Bone marrow hyperplasiab) Vascular occlusionc) Osteomyelitis2-Other Manifestations

1-Skeletal Manifestations :a) Bone marrow hyperplasia :1-Hair on end appearance of skull (the diploic

space is markedly widened due to marrow hyperplasia, trabeculae are oriented perpendicular to the inner table)

2-Pathologic fractures3-Biconcave H-shaped vertebra (central endplate

depression)4-Osteopenia

b) Vascular occlusion :1-AVN2-Bone sclerosis from infarctions3-Dactylitis (hand-foot syndrome) : bone

infarcts of hands and feet4-Involvement of growing epiphyses leads to

growth disturbances5-H shaped vertebrae

AVN of the hip

Flattening of the femoral heads with a mixture of sclerosis and lucency characteristic of osteonecrosis

Bone infarct , patchy sclerosis of the humeral head and shaft representing multiple prior bone infarcts

Patchy sclerosis of the proximal tibia due to old infarctions

Bone infarct

Hand-foot syndrome , soft tissue swelling with periosteal new bone formation and a moth-eaten lytic process at the proximal aspect of the fourth phalanx

Advanced dactylitis , lytic processes are present at the first and fifth metacarpals along with periostitis which is most prominent in the third metacarpal

c) Osteomyelitis :-High incidence : most caused by

Staphylococcus-Salmonella infection more common than in

general population-Most commonly at diaphysis of long bones-Osteomyelitis and infarction may be difficult

to distinguish

2-Other Manifestations :-Small calcified fibrotic spleen due to autoinfarction-Cholelithiasis-Progressive renal failure-Papillary necrosis-Cardiomegaly : high output congestive heart

failure (CHF)-Pulmonary infarcts

2-Thalassemia : (Cooley’s Anemia)a) Incidenceb) Radiographic Featuresc) Differential Diagnosis

a) Incidence :-Autosomal recessive microcytic anemia that

originated in the Mediterranean region-Quantitative problem of globin synthesis

b) Radiographic Features :1-Skeletal Manifestations :a) Bone marrow hyperplasiab) Vascular occlusion2-Other Manifestations

1-Skeletal Manifestations :a) Bone marrow hyperplasia :-Marrow proliferation consists of expansion of the medulla,

thinning of cortical bone and resorption of cancellous bone resulting in a generalized loss of bone density

-Expands the marrow space : hair-on-end skull, boxlike digits

-Osteopenia-Premature closure of growth plates-Paravertebral masses due to extramedullary

hematopoiesis-Ribs : rib within a rib appearance noted particularly in the

middle and anterior portions of the ribs

A generalized loss of bone density is observed , the cortex is thinned and the trabeculae are coarsened and outline localized lucency , widening of the medullary cavity has resulted in squaring of the metacarpals

Marrow expansion and cortical thinning

Left : There are large paraspinal masses (white arrows) with smoothly marginated lobulated contours , all of the ribs (red arrow) are expanded and the overall bone density is increased , Right : large paraspinal masses are seen (white arrows)

Extramedullary hematopoiesis , lobulated soft tissue opacities are noted overlying the ribs anteriorly and posteriorly

Extramedullary hematopoiesis

Rib within rib sign

b) Vascular occlusion :-Scattered bone sclerosis-H-shaped vertebral bodies-AVN less common than in sickle cell

disease

2-Other Manifestations :-Cardiomegaly and CHF-Secondary hemochromatosis-Cholelithiasis

c) Differential Diagnosis : OsteosclerosisRegular Sex Makes Occasional Perversions Much More

Pleasurable And Fantastic :1-Renal osteodystrophy2-Sickle cell anemia / Thalassemia3-Myelofibrosis4-Osteopetrosis5-Pyknodystosis6-Metastases7-Mastocytosis8-Paget’s disease9-Athletes10-Fluorosis

c) Lysosomal Storage Diseases :1-Gaucher's Disease2-Niemann-Pick Disease

1-Gaucher's Disease :a) incidenceb) Radiographic Featuresc) Differential Diagnosis

a) Incidence :-Is the most common lysosomal storage disease-The most common genetic disease among

Ashkenazi Jews - It is a genetic multisystem disease arising from a

deficiency of glucocerebrosidase activity resulting in accumulation of a glycolipid (glucocerebroside) within the lysosomes of macrophages particularity in the bone marrow , spleen and liver 

b) Radiographic Features :1-Skeletal manifestations2-Other manifestations

1-Skeletal manifestations :-Osteopenia-Osteonecrosis-Pathological / crush fractures-Endosteal scalloping (erosion of the inner cortex)-Erlenmeyer flask deformities (typically on a

femoral radiograph where there is relative constriction of the diaphysis and flaring of the metaphysis)

-H shaped vertebra

Erlenmeyer flask deformity

2-Other manifestations :-Spleen :-Massive splenomegaly-Splenic nodules-Splenic infarcts-Liver :-Hepatomegaly

c) Differential Diagnosis :-Erlenmeyer flask deformity, can be seen in :L CHONG1-Lead poisoning2-Craniometaphyseal dysplasia3-Hemoglobinopathies : thalassemia , sickle cell

anemia4-Osteopetrosis5-Niemann pick syndrome6-Gaucher’s disease

2-Niemann-Pick Disease :-Deficiency of sphingomyelinase-Radiographically similar to Gaucher's

disease except that AVN and cystic bone lesions do not occur