Thalassaemia

INTRODUCTION
• Thalassaemia, is an inherited blood disorder caused by defect in a gene causing microcytic anemia. • The two types of thalassaemia is alpha and beta thalassaemia. • Sign and symptoms of thalassaemia include weakness, fatigue, and failure to grow and organomegaly.

noted particularly in the middle and anterior portions of the ribs • Extramedullary hematopoiesis • premature fusion of the epiphyses . • skull : classic “hair on end” appearance • facial bones: rodent facies • ribs : “ribs within a rib” appearance. and resorption of cancellous bone resulting in a generalized loss of bone density.Radiographic features • Skeletal • Marrow proliferation consists of expansion of the medulla. thinning of cortical bone.

The hair on end sign refers to a radiographic appearance on a skull which results from a periosteal reaction .

Also note dental malocclusion and underpneumatized paranasal sinuses with rodent facies .Lateral skull from a patient with thalassemia major showing diploic widening with relative sparing of the occipital bone.

“rib within a rib" appearance .

marrow expansion and cortical thinning .

.Bilateral paravertebral soft-tissue masses as well as marked medullary expansion of the bony structures are associated with compensatory extramedullary hematopoiesis.

a group of plasma cells (myeloma cells) becomes cancerous and multiplies. raising the number of plasma cells to a higher than normal level. .Multiple myeloma • In multiple myeloma.

. and thus is worse during the day • anaemia – typically normochromic / normocytic • renal failure / proteinuria • Hypercalcaemia • pathological fracture – vertebral compression fracture – long bone fracture (e. and includes : • bone pain – initially intermittent.g. but becomes constant – worse with activity / weight bearing. proximal femur) • recurrent infection : e.g.• Clinical presentation of patients with multiple myeloma is varied. pneumonia due to leucopoenia.

g. well circumscribed lytic bone lesions : more common – punched out lucencies e.• Radiographic features • Radiology has a number of roles in the diagnosis and management or multiple myeloma. • suggest the diagnosis / exclude other causes • assess possible mechanical complications (e. pepperpot skull or raindrop skull – endosteal scalloping • generalized osteopaenia : less common – often associated with vertebral compression fracture/ vertebral plana . pathological fracture) • assess disease progression • Disseminated multiple myeloma has two common radiological appearances: • numerous.g.

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well-defined lytic lesions (punched out lesions) of various size scattered throughout the skull constitutes the raindrop skull / pepperpot appearance of multiple myeloma.The appearance of multiple. .

due to slow growing medullary lesions. .• Endosteal scalloping refers to the focal resorption of the inner margin of cortical bones. typically seen in long bones.

LEUKEMIA .

.INTRODUCTION Leukemias are a group of heterogeneous neoplastic disorders of white blood cells.

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Radiological Features Radiographic osseous changes occur in 50-70% of children with leukemia The radiological features Flat and long bones Common •Diffuse osteoporosis •Radiolucent submetaphyseal bands •Bone destruction •Periosteal reaction Spine •Osteoporosis •Compression fractures •Radiolucent subendplate bands Joints •Juxta-articular osteoporosis •Effusion •Soft tissue swelling SKELETAL DISTRIBUTION OF LEUKEMIA .

Note that submetaphyseal bands (arrows) are often the first radiologic signs of childhood leukemia. A. B. AP Knee. . A submetaphyseal band appears as a linear radiolucent region in the metaphysis beneath and parallel to the opaque zone of provisional calcification at the growth plate LEUKEMIA: RADIOLUCENT SUBMETAPHYSEAL BANDS. AP Hips.Radiolucent Submetaphyseal Bands.

Note the multiple transverse linear opacities within the femur and tibia.LEUKEMIA: GROWTH ARREST LINES. AP Knee. . which reflect the cyclic repression of bone growth in this child with leukemia.

Observe the discrete metaphyseal and diaphyseal lesions (arrows). Note the more confluent. mimicking osteomyelitis.LEUKEMIA: BONE DESTRUCTION. A. . Later. B. moth-eaten destruction. with periostitis. Early.

.LEUKEMIA. Lateral Thoracic Spine. Generalized osteoporosis and accentuated trabecular changes in this patient with chronic lymphatic leukemia.

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