5. Sickle cell disease
Clinical symptoms:
Bony, chest, abdominal or neurological.
Which could be secondary to infarction or infection.
Radiology work up:
Plain x-ray.
CT.
MRI.
Autosomal recessive hereditary condition caused by the
formation of abnormal hemoglobin.
Manifests as multisystem ischemia and infarction, as well
as hemolytic anemia.
6. Musculoskeletal Findings
marrow hyperplasia
long bones: widening of
medullary spaces and thinning
of cortical bone
skull: widening of diploic space,
thinning of outer table, “hair-on-
end” appearance
osteopaenia and
pathological fractures
extramedullary
haematopoiesis; paraspinal
masses
Normal
8. osteonecrosis
bone infarcts typically
involve medullary cavities
and epiphyses
the proximal humerus,
proximal femora, and
vertebral bodies are often
affected
vertebral infarcts may
result in
H-shaped vertebrae
11. Osteomyelitis
• Diffuse intramedullary altered signal with periosteal reaction in the tibia.
• Interosseous and circumferential soft tissue abscess.
• Diffuse circumferential myositis.
12. Fever, cough and chest pain.
Radiological workup
Plain x-ray.
CT chest and high resolution CT
Chest Findings
13. Acute chest syndrome (ACS)
In sickle cell disease is a leading thoracic
complication - as well as leading cause of
mortality.
Patients may present with acute fever, cough,
wheezing, tachypnea and/or chest pain on a
background of established sickle cell disease.
In those affected by sickle cell disease. The
diagnosis is made on the combination of
new pulmonary opacity on chest x-ray with at
least one new clinical symptom or sign.
19. Moyamoya syndrome
(Puff of smoke)
Also termed the Moya-moya pattern or phenomenon.
It is a cerebrovascular condition that predisposes
affected patients to stroke in association with
progressive stenosis of the intracranial internal
carotid arteries and their proximal branches.
It is due to arterial occlusion of the circle of Willis,
with resultant collaterals, and appearances
reminiscent of Moyamoya disease.
22. Thalassemia
• Autosomal recessive microcytic anaemia .
• The genetic defect causes a reduction in the rate of globin
chain synthesis which causes the formation of
abnormal haemoglobin molecules. That leads to anemia.
• Normal adult hemoglobin is composed of HbA (98%)
and HbA2 (2%).
HbA contains two α globin chains and two β globin
chains,Thalassemia patients produce a deficiency of
either α or β globin, unlike sickle cell disease, which
produces a specific mutant form of β globin.
• Thalassemia is a quantitative problem of globin synthesis,
whereas sickle cell disease is a qualitative problem of
synthesis of an incorrectly functioning globin.
23. Thalassemia
Radiological features:
There is morrow proliferation resulting of expansion as well
as thinning of cortical bone. Giving hair on end
appearance on the skull, rib within a rib as well as
premature fusion of epiphysis.
Rodent face due to no pneumatisation of the paranasal
sinuses and overgrowth of the facial bones.
25. extramedullary hematopoiesis may appear as lobulated masses covering the anterior
or posterior segments of the ribs on chest x-ray. Extramedullary hematopoiesis is also
more frequently seen in the posterior mediastinum compared to the anterior
mediastinum or the pelvis.
26. • Diffusely low signal throughout the bony skeleton on both T1 and T2 sequences.
related to active red marrow and iron deposition from multiple blood transfusions.
• Note the remarkable difference when compared to normal T1 and T2 marrow signal.
29. Lymphoma
• malignancy arising from lymphocytes or lymphoblasts.
• Lymphoma can be restricted to the lymphatic system or
can arise as extranodal disease.
• Hodgkins lymphoma and NHL – based on pathology
• Lymphoma can often present with B symptoms (fever,
night sweats and weight loss).
30. Lymphoma
• Role of radiologists is to suggest the diagnosis and
monitoring of the disease.
• Imaging is used for Staging:
• CT with contrast
• PET CT
Ann Arbor
classification
32. Extranodal disease
GIT system
Stomach, small
intestine, colon,
esophagus
Aneurysmal parietal
thickening with lack of
obstruction
can be polypoidal
Main Ddx is
adenocarcinoma
34. Extranodal disease
Renal
Usually extension from
neighboring
retroperitoneum or
hematological
dissemination
Intraparenchymal
nodules
Perirenal infiltration
35. Extranodal disease
Chest
Mimics tumoral or
inflammatory
conditions
nodules and
masses with or
without cavitation,
consolidationa,
ground glass
opacities
37. Leukemia
• group of cancers that usually begin in the bone marrow and
result in high numbers of abnormal white blood cells.
• These white blood cells are not fully developed and are
called blasts or leukemia cells.[
• Symptoms may include bleeding
and bruising problems, feeling tired, fever, and an
increased risk of infections.
• Diagnosis is typically made by blood tests or bone marrow
biopsy.[
Types of leukemia :
1. Acute lymphoblastic leukemia (ALL).
2. Acute myeloid leukemia (AML).
3. Chronic lymphocytic leukemia (CLL).
4. Chronic myeloid leukemia (CML
38. Chronic lymphocytic leukaemia
• CLL is considered the most common type of leukemia
• Up to half of patients can be asymptomatic
• Imaging may identify various features of the disease such
as splenomegaly, hepatomegaly +/- lymphadenopathy,
39. Acute leukemia
• is the most common malignancy in childhood
under 15 years age.
• The growing skeleton is the main site of
involvement in children
• Bony lesions are more prevalent in children than
adults.
• A metaphyseal radiolucent band is one of the
most important radiological findings.
40. Bony lesions in acute leukemia
Multiple collapsed vertebra
Metaphyseal lucent bands in radius and
ulna metaphysis
Lucent bone lesions in first and third
metacarpal bones
41. CNS manifestations
CNS manifestations directly
attributed to leukaemia
leukaemic meningitis
intradural spinal involvement
Granulocytic sarcoma (chloroma)
haematologic and cerebrovascular
complications
Cerebral haemorrhage
most common in acute
leukaemia
disseminated intravascular
coagulation
Dural venous sinus
thrombosis
42. Granulocytic sarcoma
Granulocytic sarcoma (also called myeloid sarcoma and chloroma) is a rare
neoplasm comprised of myeloid precursor cells. It is typically seen is in children.
Being extramedullary is a key feature. Almost any tissue can be affected.
marrow hyperplasia
long bones: widening of medullary spaces and thinning of cortical bone
skull: widening of diploic space, thinning of outer table, “hair-on-end” appearance
osteopaenia and pathological fractures
extramedullary haematopoiesis
red marrow reconversion
osteonecrosis
bone infarcts typically involve medullary cavities and epiphyses
the proximal humerii, proximal femora, and vertebral bodies are often affected
in the humerii, serpiginous sclerosis is characteristic of infarction
vertebral infarcts may result in
H-shaped vertebrae
metaphyseal lucent band (arrow head) in radial and ulnar metaphyses. Note the lucent bony lesions of the first and third metacarpal bone on the right side (arrows).
Lateral thoracolumbar X-ray reveals multiple collapsed vertebrae.
Granulocytic sarcoma (also called myeloid sarcoma and chloroma) is a rare neoplasm comprised of myeloid precursor cells.
It is typically seen is in children.
Being extramedullary is a key feature. Almost any tissue can be affected