Thalassaemia is an autosomal recessive haemoglobinopathy that originated in the Mediterranean region. The genetic defect causes a reduction in the rate of globin chain synthesis which causes the formation of abnormal haemoglobin molecules. The resultant microcytic anaemia is the characteristic p...
Myelofibrosis is a haematological disorder where there is the replacement of bone marrow with collagenous connective tissue and progressive fibrosis. It is also classified as a myeloproliferative disorder. It is characterised by:
extramedullary hematopoiesis (EMH)
Response Assessment on CT
CT uses for the standing of all types of lymphoma (if CT is performed for tumor size measurement) and it is important, that CT is primary assessment modality for non–FDG-avid lymphoma.
Response Assessment on CT:
Partial Remission/Partial Response...
Abdominal manifestations of sickle cell disease (SCD) are wide and can involve many organs.
For a general discussion, please refer to sickle cell disease.
may occur transiently with the sequestration syndrome, where rapid pooling of blood occurs in the spleen, resulting ...
There are a number of lymphoma staging systems for both Hodgkin lymphoma and non-Hodgkin lymphoma including the Ann Arbor classification, Cotswolds-modified Ann Arbor classification, and the most current, Lugano classification.
Evolution of lymphoma staging and treatment response evaluation cr...
Secondary involvement of the bone with lymphoma, also referred as secondary bone lymphoma, is much more common than primary bone lymphoma, occurring in ~15% of disseminated lymphomas.
Secondary bone lymphoma is defined as lymphoma involving the bone with nodal disease occurring wit...
Multiple myeloma is the most common primary malignant bone neoplasm in adults. It arises from red marrow due to monoclonal proliferation of plasma cells and manifests in a wide range of radiographic abnormalities. Multiple myeloma remains incurable.
Four main patterns are recognise...
PET-CT is a combination of cross-sectional anatomic information provided by CT and the metabolic information provided by positron emission tomography (PET).
PET is most commonly performed with 2-[F-18]fluoro-2-deoxy-D-glucose (FDG). Fluorine-18 (F-18) is an unstable radioisotope and has a half-...
Gaucher disease (GD) is the most common lysosomal storage disease in humans. It is an autosomal recessive, multisystem disease arising from a deficiency of glucocerebrosidase or beta-glucosidase activity, resulting in accumulation of a glycolipid (glucocerebroside) within the lysosomes of macrop...
Small bowel lymphoma is the most common small bowel malignancy, accounting accounts for ~25% of all primary small bowel malignancies and ~40% of all primary gastrointestinal lymphomas.
Small bowel lymphoma is most commonly secondary extranodal involvement in widespread systemic ly...
All-trans retinoic acid (ATRA) syndrome (more recently known as differentiation syndrome (DS)8) is a condition that can occur with patients with acute promyelocytic leukaemia who are on therapeutic all-trans-retinoic acid (ATRA).
All-trans-retinoic acid (ATRA) is a normal constituent of plasma....
example 1: abdominal film
example 2: erect and supine
example 3, example 4: paediatric
example 5: young adult male
example 1, example 2, example 3: barium swallow
example 1: upper GI series
example 1: barium follow through
example 1: barium enema
Low attenuation lymphadenopathy suggests underlying necrosis and can be seen in:
metastatic carcinoma (or lymphoma)
infections (tuberculous or fungal)
low attenuation lymphadenopathy
high attenuation lymphadenopathy
Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a recently recognised, rare, non-neoplastic vascular splenic neoplasm of uncertain etiology. The term SANT first appeared in the literature in a 2004 paper by Martel et al. 3 which examined a series of 25 cases.
Histiocytic sarcoma is a rare malignant haematopoietic neoplasm that has been reported in association with other hematological malignancies (particularly B and T cell lymphomas).
It comprises of tumour cells derived from the monocyte/macrophage lineage.
It usually occurs i...
Sickle cell disease (SCD) is a hereditary (autosomal recessive) condition resulting in the formation of abnormal haemoglobin (a haemoglobinopathy), which manifests as multisystem ischaemia and infarction, as well as haemolytic anaemia.
There is no recognised gender predilection. ...
H-shaped vertebrae, also known as Lincoln log vertebrae, are a characteristic finding of sharply delimited central endplate depression, classically seen in approximately 10% of patients with sickle-cell anaemia, and results from microvascular endplate infarction (figure 1) 3.
It may occasionall...
Non-Hodgkin lymphoma (NHL) is a catch-all term for all lymphomas that are not of the Hodgkin's subtype. It is a heterogeneous group of malignancies in terms of histology, clinical presentation and prognosis.
See the 2008 WHO classification for further information on subtypes.
Protein S deficiency is a hyperocoagulable state associated with increased risk of venous thrombosis (up to 5% of patients with deep venous thrombosis may carry this deficiency).
Protein S deficiency may be expected in ≈1 of every 500 people 3.
Protein S, a vitamin K-d...
Lymphoma is a malignancy arising from lymphocytes or lymphoblasts. Lymphoma can be restricted to the lymphatic system or can arise as extranodal disease. This, along with variable aggressiveness results in a diverse imaging appearance.
Lymphoma accounts for ~4% of all cancers 4. T...
Pulmonary graft versus host disease (GvHD) is one of the thoracic manifestations that can complicate haematopoetic stem cell transplantation. Pulmonary GvHD can be broadly divided into acute and chronic disease 1-4:
acute pulmonary GvHD
pulmonary involvement is rare
the median time of onset o...
Pulmonary leukostasis is a medical emergency that is most commonly seen as a complication of chronic myeloid leukaemia (CML) in blast crisis, and acute myeloid leukemia when white blood cell (WBC) counts are over 100 x 109/L (100,000/microL). It needs to be considered in any patient with myeloge...
Splenic trauma can occur after blunt or penetrating trauma or secondary to medical intervention (i.e. iatrogenic). The spleen is the most frequently injured organ after blunt trauma.
Patients may present with left upper quadrant/left chest pain, left shoulder tip pain (re...
Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma (NHL) and accounts for ~5% of all NHL. It is a malignant neoplasm of virgin B cells that closely resemble normal mantle zone B cells surrounding germinal centres.
They occur in older adults (mean age ~60 years). There is...
Pulmonary lymphoma refers to lung parenchymal involvement with lymphoma.
It can be broadly divided as primary or secondary.
primary pulmonary lymphoma: (rare) usually non-Hodgkin lymphoma which is limited to the lung with or without mediastinal lymph node involvement and with no evidence of ex...
Diffuse T1 vertebral bone marrow signal loss has a number of causes. T1-weighted imaging without fat suppression is one of the most important sequences for distinguishing between normal and abnormal bone marrow. Given the homogeneity, this appearance can often be difficult to spot as abnormal. ...
Hand-Schüller-Christian disease is a clinically intermediate form of a spectrum of histiocytic disorders, which ranges from acute fulminant to chronic indolent disease. It primarily affects children, less often young adults, and rarely older adults.
Hand-Schüller-Christian disease has been desc...
Splenomegaly is a term which refers to enlargement of the spleen. The normal adult splenic length upper limit is usually around 12-15 cm. Also one should know how to calculate splenic index, volume and mass by CT and MR techniques. Massive splenomegaly is a term used when the spleen weighs >1000...
CNS lymphoma refers to the involvement of the central nervous system with lymphoma. It can be broadly divided into primary and secondary, with a number of special types of also recognised.
primary CNS lymphoma (PCNSL)
MALT lymphoma of the dura 5
secondary CNS lymphoma...
Haemophilic arthropathy refers to permanent joint disease occurring in haemophilia sufferers as a long-term consequence of repeated haemarthrosis. Around 50% of patients with haemophilia will develop a severe arthropathy.
Haemophilia is an x-linked recessive disease affecting mal...
Lymph node enlargement is often used synonymously with lymphadenopathy, which is not strictly correct.
Lymphadenopathy (or adenopathy) is, if anything, a broader term, referring to any pathology of lymph nodes, not necessarily resulting in increased size; this includes abnormal num...
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Splenunculi (also known as supernumerary, accessory spleens, or splenules) are small nodules of spleen that are separate from the rest of the organ. They are benign and asymptomatic, their importance is mainly related to the need to distinguish them from more sinister pathology.
Antithrombin (AT) III deficiency (now simply called antithrombin deficiency) refers to a congenital lack of the endogenous anticoagulant antithrombin.
Antithrombin deficiency is considered the least common of the three main anticoagulant deficiencies (the other two being protein C...
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy. It was previously termed as blastic natural-killer lymphoma or agranular CD4+ natural killer cell leukemia.
It only represent a wel small propotion (around 0.44%) of all haematological malign...
Burkitt lymphoma (BL) is an aggressive B-cell lymphoma that predominantly affects children.
Burkitt lymphoma is the most common (40%) type of non-Hodgkin lymphoma in childhood. The median age of Burkitt lymphoma is eight years, and it has a male predominance (M:F = 4:1) 1. It is l...
Plasmacytomas are a discrete, solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue (extramedullary). It is a rare tumour that is associated with latent systemic disease in the majority of affected patients. It can be considered as a singular counterpart of multiple m...
Langerhans cell histiocytosis (LCH) is a rare multi-system disease with a wide and heterogeneous clinical spectrum and variable extent of involvement.
The disease is more common in the paediatric population, with a peak incidence between one and three years of age 5. Incidence is...
Haemochromatosis is an iron overload disorder characterised by a progressive increase in total body iron stores and deposition of iron in some non-reticuloendothelial system (RES) body organs which results in some instances in organ dysfunction.
This article focus on the general principles of h...
Aplastic anaemia is a rare haematopoietic stem-cell disorder. The condition results in pancytopaenia and hypocellular bone marrow. Most cases are acquired, however there are unusual inherited forms.
Aplastic anaemia manifests as a marked reduction in the number of pluripotent h...
Typhlitis, also called caecitis or neutropaenic colitis, is a necrotising inflammatory condition which typically involves the caecum and, sometimes, can extend into the ascending colon or terminal ileum.
Typhlitis was first described in children with leukaemia and severe neutropae...
Haemolytic anaemia is a form of anaemia where red blood cells (RBCs) are destroyed either intravascularly or extravascularly.
The patient presents with anaemia and jaundice. Diagnosis is based on several laboratory parameters 1:
increased unconjugated bi...
The anatomy curriculum is one of our curriculum articles and aims to be a collection of articles that represent the core anatomy knowledge for radiologists and imaging specialists.
Head and neck anatomy
Abdominal and pelvic anatomy
Angioimmunoblastic T-cell lymphoma (AITL) is a rare, aggressive (fast-growing) form of peripheral T-cell lymphoma. It only accounts for around 1-2% of all non-Hodgkin lymphoma. It is one of the more common subtypes of mature T-cell lymphomas.
It can be more common in the elderly....
The Wells criteria for pulmonary embolism is a risk stratification score and clinical decision rule to estimate the probability for acute pulmonary embolism (PE) in patients in which history and examination suggests acute PE is a diagnostic possibility. It provides a pre-test probability which, ...
Catheter-directed thrombolysis is an endovascular approach to the treatment of acute iliofemoral deep vein thrombosis. It involves the administration of a lytic agent directly into a thrombus.
precise diagnosis of iliofemoral deep vein thrombosis
first episode of acute iliofemor...
In periods of bodily stress the thymus may acutely shrink to 40% of its original volume (depending on the severity and duration of the stress). During the recovery phase it can grow back to its original size or even larger (up to 50% larger). This "rebound effect" is known as thymic rebound hype...
Polycythaemia vera is a myeloproliferative disorder that results in an excess of red blood cells in the bloodstream.
The estimated prevalence is around 2-3 per 10000 people. It typically presents in older individuals. There may be a slightly greater male predilection.
AIDS-related pulmonary lymphoma (ARPL) is classified as a distinct form of pulmonary lymphoma. Pulmonary involvement is a common extranodal site in AIDS-related NHL.
ARPL is typically a high-grade B-cell non-Hodgkin's lymphoma and the majority of patients have advanced HIV infection,...
Carbon monoxide (CO) poisoning can cause an anoxic-ischaemic encephalopathy. The neurotoxicity could lead to acute as well as delayed effects.
CO poisoning is related mostly to preventable causes such as malfunctioning heating systems, improperly ventilated motor vehicles, and res...
Haematopoietic stem cell transplantation (HSCT) is a relative common procedure used to treat a wide spectrum of conditions 1,2:
lymphoproliferative disorders, e.g. lymphoma, multiple myeloma (most common indication)
solid tumours, e.g. neuroblastoma, Ewing sarcoma, extragonadal germ...
Diamond-Blackfan anaemia, also known as pure red cell aplasia, is a rare congenital anaemia that typically presents in the first few years of life as a normocytic or macrocytic anaemia usually only affecting cells of the erythroid lineage 2. However, neutropenia and thrombocytopenia may occasion...
The 2008 WHO classification of tumours of haematopoietic and lymphoid tissues is at the time of writing (mid 2016) the most widely used classification system.
nodular lymphocyte predominance
classical Hodgkin lymphoma
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal haematological stem cell disorders. It has sometimes been referred to as a preleukaemia or a preleukaemic condition.
Its overall incidence is thought to be around 3.3 per 100,000. The incidence in patients older t...
Richter transformation is defined as development of high-grade non-Hodgkin lymphoma (NHL) in patients with chronic lymphocytic leukaemia (CLL) or small lymphocytic lymphoma.
It has been expanded to include other lymphoid malignancies that develop in CLL patients, including Hodgkin disease, prol...
Chronic lymphocytic leukaemia (CLL) is a haematological malignancy.
CLL is considered the most common type of leukemia in the Western hemisphere; its prevalence in Europe and North America ranges from 29-38% of all leukaemias 1. It primarily affects adults ~65-70 years of age 3.
Extramedullary haematopoiesis is a response to failure of erythropoiesis in the bone marrow.
chronic myelogenous leukaemia
myelofibrosis with myeloid metaplasia
sickle cell disease
Hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome (SOS), is a condition arising from occlusion of hepatic venules.
right upper quadrant pain
abnormal liver function tests
Toxic injury to liver s...
The twin anaemia polycythaemia sequence (TAPS) is considered a variant of the twin to twin transfusion syndrome (TTTS).
TAPS may occur spontaneously in up to 5% of monochorionic twins and may also develop after incomplete laser treatment in TTTS cases 2.
As with TTTS t...
Normal bone marrow is divided into red and yellow marrow, a distinction made on the grounds of how much fat it contains.
Red marrow is composed of:
reticulum (phagocytes and undifferentiated progenitor cells)
scattered fat cells
a rich ...
Fetal anaemia can result from many causes.
haemolytic disease of the newborn
fetomaternal ABO incompatibility
fetomaternal rhesus (Rh) incompatibility
fetal parvovirus B19 infection
homozygous alpha thalassaemia 7
Cryoglobulinaemic vasculitis (CV) is a form of immune mediated primary vasculitis involving small to medium sized vessels. It may involve multiple organs and can have a range of clinical presentations.
There are three main types of cryoglobulinaemia which are grouped, as per the Br...
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign idiopathic proliferative disease that involves phagocytic histiocytes.
The disease predominantly occurs in young adults with a mean age at presentation of 21 years....
Paraneoplastic syndromes occur secondary to the indirect effects of a malignancy and occur remotely to the primary malignancy. Symptoms are mediated by cytokines, hormones or immune cross-reactivity. These syndromes can cause a diverse range of symptoms and can affect multiple systems.
Bing-Neel syndrome is an extremely rare neurological complication of Waldenström macroglobulinemia where there is malignant lymphocyte infiltration into the central nervous system (CNS).
Treatment and prognosis
It is considered potentially treatable with cranial radiation therapy alone or in c...
Waldenström's macroglobulinaemia (WM), (previously also known as lymphoplasmacytic lymphoma (LPL)), is a type of B-cell lymphoma. It is a rare condition, accounting for only 1% of all lymphoproliferative disorders.
Recent publications classify Waldenström's macroglobulinaemia as an lymphoplasm...
Perisplenitis is acute inflammation of the splenic capsule and its peritoneal covering.
It is seen uncommonly but there is no data on its actual incidence.
It is seen usually in young and middle-aged patients, with acute left hypochondrial or lower chest pa...
Secondary involvement of the pleura with lymphoma (secondary pleural lymphoma) is very common, occurring in ~20% of lymphomas. It may be a result of an extension of lymphoma into the visceral or parietal pleura or be a complicating pleural effusion and is a poor prognostic factor.
This article lists examples of normal imaging of the liver and biliary tree and surrounding structures, divided by region and modality.
liver silhouette: example
example 1 with shear wave elastography
liver Doppler ultrasound: example ne...
There are many thoracic complications that can occur following haematopoietic stem cell transplantation. These can precipitate during various stages following transplantation and can be either infectious or noninfectious.
The Deauville five-point scale (Deauville 5ps) is an internationally recommended scale for clinical routine and clinical trials using FDG-PET/CT in the initial staging and assessment of treatment response in Hodgkin lymphoma (HL) and certain types of non-Hodgkin lymphomas (NHL).
Mannose-binding lectin (MBL) deficiency is a condition that affects the immune system.
It may be prevalent in 10-30% of the general population.
Affected individuals have low levels of an immune protein named mannose-binding lectin in their blood. They are prone to recu...
Primary hepatic lymphoma (PHL) is rare accounting for roughly 100 described cases. If it is being considered as a diagnosis, distal lymphadenopathy, splenomegaly, bone marrow disease, and leukaemia should not be present for at least 6 months after the liver tumour is detected (see: secondary hep...
Extraosseous myeloma refers to any manifestation of multiple myeloma where there is plasma cell proliferation outside the skeletal system. This can potentially affect any organ system and the reported disease spectrum includes:
lymph nodes (considered to be most com...
A number of sonographic features are helpful in distinguishing reactive versus malignant lymph nodes.
Grey scale features
Features that favour reactive/infective nodes over malignancy include:
surrounding soft tissue oedema
Doppler examination is particularly...
Rubinstein-Taybi syndrome (RTS) is a very rare genetic multi-system disorder primarily characterised by mental retardation, broad and often angulated thumbs and halluces, and distinctive facial features.
The estimated incidence is 1 in 100,000-125,000 live births 5.
Lymphoma of the uterine cervix is generally uncommon and when it does occur tends to present as cervical involvement with added background multi-organ disease rather than isolated primary cervical lymphoma 1. It is often considered part of the spectrum of uterine lymphoma.
In the ...
A hemoglobinopathy is a genetic disorder which alters the structure of hemoglobin 1. The result is reduced oxygen carrying capacity of the blood to the tissues.
Types of hemoglobinopathies include the following:
Sickle cell disease (Hb S)
Sickle cell trait (HB AS)
Von Willebrand disease (vWD) is the most common inherited bleeding diathesis (easy bleeding).
Bruising and mucosal bleeding are typical presentations, but there is a spectrum of severity. The more residual vWF a patient has, the less severe the bleeding.
Extramedullary plasmacytoma (EMP) is an uncommon plasma cell tumour that is composed of monoclonal plasma cells arranged in clusters or sheets. The rate of progression to multiple myeloma (MM) varies from 10% to 30%.
EMP occurs most commonly during the fourth through to seventh de...
Abdominal complications of haematopoietic stem cell transplantation can occur early (0-100 days) or late (>100 days) post transplant.
bacterial infections, e.g. pseudomembranous colitis
fungal infections, often affecting the oesophagus or as hepatic/splenic microabscesse...
Leukaemic infiltration of the liver can occur with several forms of leukaemia inclusive of acute myeloid leukaemia (AML), acute lymphoid leukaemia (ALL) and chronic myeloid leukaemia (CML).
Described features are non-specific but include:
Heparin-induced thrombocytopaenia (HIT) is a paradoxical thrombotic state resulting from an immune response to heparin.
Occurs in 1:5000 patients who have received heparin, most commonly unfractionated heparin.
HIT is induced by IgG antibodies.
Haemochromatosis is a systemic disease which affects many organs systems (see the main hemochromatosis article), including the joints, characterised by haemosiderin and calcium pyrophosphate deposition resulting in:
chondrocalcinosis: particularly knees and triangular fibrocartilage
Wiskott-Aldrich syndrome (WAS) is a rare immunodeficiency disease with a characteristic clinical phenotype that includes:
X-linked recessive and only occurs in boys
petechiae, bloody diarrhoea, epistaxis due to thrombocytopaenia with small platelets
eczema starts in first month of life
AIDS defining illnesses are conditions that in the setting of a HIV infection confirm the diagnosis of AIDS, and do not commonly occur in immunocompetent individuals 2. According to the CDC surveillance case definition 1, they are:
bacterial infections: multiple or recurrent
Hepatic lymphoma is a rather broad term given to any form of hepatic involvement with lymphoma. This can be broadly divided into:
secondary hepatic involvement with lymphoma: most common by far, many tend to be non-Hodgkin lymphoma (NHL) 1
primary hepatic lymphoma: extremely rare
MR liver iron quantification is a non-invasive means of measuring liver iron concentration, a key indicator in the management of patients with haemochromatosis (primary or secondary).
Apart from being non-invasive, sampling occurs in a large cross-section of the liver, as opposed to...
Follicular lymphoma is a subtype of non-Hodgkin lymphoma (NHL) and is, in fact, the most common type.
Estimated to account for ~45% of all NHL cases 1. Higher rates in North America and Europe 4.
Nodal effacement by closely packed follicles containing small cleaved ce...
Splenic epidermoid cysts are a type of non-parasitic primary splenic cyst.
They are thought to account for 10-25% of benign non-parasitic cysts of the spleen 3. There may be an increased female predilection.
The clinical presentation can vary ranging from b...
Hodgkin lymphoma or Hodgkin disease (HD) is a type of lymphoma and accounts for ~1% of all cancers. HD spreads contiguously and predictably along lymphatic pathways and is curable in ~90% of cases, depending on its stage and sub-type.
There is a bimodal distribution in the age of ...
Trousseau syndrome is referred to by a number of names, such as migratory thrombophlebitis and cancer-associated thromboembolism, but is essentially the association of venous thrombosis provoked by a yet to be diagnosed malignancy.
There are several non lymphomatous lymphoid disorders that can affect the lung. This implies that they comprise of lymphoid tissue but may not have progressed as far as an overt lymphoma. They include:
lymphocytic interstitial pneumonia