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Sickle cell disease
Thalassemia
Lymphoma
Leukemia
Radiology workup
 Plain x-ray.
 Ultrasound.
 CT.
 MRI.
 Bone scan.
Sickle cell Disease
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.
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
Extra medullary hematopoiesis
 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
MRI Later X-ray
H-shaped Vertebra
Normal
Bone infarct
MRI
Osteomyelitis
• Diffuse intramedullary altered signal with periosteal reaction in the tibia.
• Interosseous and circumferential soft tissue abscess.
• Diffuse circumferential myositis.
Fever, cough and chest pain.
Radiological workup
Plain x-ray.
CT chest and high resolution CT
Chest Findings
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.
ACS
ACS
Abdominal Findings
Sickle cell nephropathy – multiple renal infarcts
Abdominal findings
Auto-splenectomy
i.e spontaneous infarction of the
spleen with resulting hyposplenism.
Normal
 Tortuosity of arteries.
 Moyamoya syndrome .
 Cerebral atrophy.
 Cortical infarction
Cerebral manifestations
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.
Normal MRA Brain
Moya Moya
Normal Brain
CT
Brain Atrophy and infarction on left
Thalassemia
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.
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.
Skeleton manifestation
diffusely osteopenic, with
expansion of the medullary
spaces and a lace-like
trabecular pattern.
Normal
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.
• 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.
Gastrointestinal
manifestations
Lymphoma
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).
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
Nodal disease
 Lymph node
form
 >1 cm is
considered
pathological
Extranodal disease
GIT system
 Stomach, small
intestine, colon,
esophagus
 Aneurysmal parietal
thickening with lack of
obstruction
 can be polypoidal
 Main Ddx is
adenocarcinoma
Extranodal disease
Liver
 Solitary form (most
common) >10 cm
 Multinodular form
 Ddx is HCC
Extranodal disease
Renal
 Usually extension from
neighboring
retroperitoneum or
hematological
dissemination
 Intraparenchymal
nodules
 Perirenal infiltration
Extranodal disease
Chest
 Mimics tumoral or
inflammatory
conditions
 nodules and
masses with or
without cavitation,
consolidationa,
ground glass
opacities
Extranodal disease
CNS
 Usually
leptomeningeal
lesion
 Most common
locations are deep
white matter, corpus
callosum,
periventricular zone
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
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,
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.
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
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
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.
Thank you!

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MED401-Rad-Lect- radiological finding of common heamatology disease.ppt

  • 1.
  • 3. Radiology workup  Plain x-ray.  Ultrasound.  CT.  MRI.  Bone scan.
  • 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
  • 9. MRI Later X-ray H-shaped Vertebra Normal
  • 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.
  • 14. ACS
  • 15. ACS
  • 16. Abdominal Findings Sickle cell nephropathy – multiple renal infarcts
  • 17. Abdominal findings Auto-splenectomy i.e spontaneous infarction of the spleen with resulting hyposplenism. Normal
  • 18.  Tortuosity of arteries.  Moyamoya syndrome .  Cerebral atrophy.  Cortical infarction Cerebral manifestations
  • 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.
  • 20. Normal MRA Brain Moya Moya Normal Brain CT Brain Atrophy and infarction on left
  • 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.
  • 24. Skeleton manifestation diffusely osteopenic, with expansion of the medullary spaces and a lace-like trabecular pattern. Normal
  • 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
  • 31. Nodal disease  Lymph node form  >1 cm is considered pathological
  • 32. Extranodal disease GIT system  Stomach, small intestine, colon, esophagus  Aneurysmal parietal thickening with lack of obstruction  can be polypoidal  Main Ddx is adenocarcinoma
  • 33. Extranodal disease Liver  Solitary form (most common) >10 cm  Multinodular form  Ddx is HCC
  • 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
  • 36. Extranodal disease CNS  Usually leptomeningeal lesion  Most common locations are deep white matter, corpus callosum, periventricular zone
  • 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.

Editor's Notes

  1. 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
  2. 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.
  3. 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