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
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
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,...
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....
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....
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...
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...
Autosplenectomy denotes spontaneous infarction of the spleen with resulting hyposplenism.
Autosplenectomy is most frequently encountered in patients with homozygous sickle cell disease, although it has also been reported in pneumococcal septicaemia 1, and SLE 2. The demographics t...
BALT lymphoma is an abbreviated term for bronchus-associated lymphoid tissue lymphoma. These neoplasms fall under the broader umbrella of mucosa associated lymphoid tissue (MALT) lymphomas. It is sometimes considered a type of primary pulmonary lymphoma.
Up to half of pat...
The Binet staging system is one of the two staging systems currently adopted in assessment of chronic lymphocytic leukaemia (CLL).
It classifies CLL according to the number of lymphoid tissues that are involved (i.e. the spleen and the lymph nodes of the neck, groin, and underarms), as well as ...
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...
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...
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 ...
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...
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...
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...
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.
Chronic myelomonocytic leukemia (CMML) is a relatively rare clonal haematologic disorder. It is sometimes classified as a type of myelodysplastic syndrome (MDS) but also has features of myeloproliferative disease (MPD).
Can be variable but many patients tend to present wi...
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...
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...
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).
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...
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. ...
Ebola virus disease (EVD) (also known as Ebola haemorrhagic fever (EHF) or simply Ebola) is a viral haemorrhagic disease caused by the Ebola Filovirus. Ebola is an extremely virulent virus with case fatality rates of approximately 70% 1.
First recognized in 1967 after polio vaccin...
Essential thrombocythaemia (ET) refers to an acquired myeloproliferative neoplastic state characterised by an expansion of the megakaryocytic lineage, leading to an isolated elevation of platelets. It falls under the group of myeloproliferative disorders. It increases the risk of both thrombosis...
Extramedullary haematopoiesis is a response to failure of erythropoiesis in the bone marrow.
chronic myelogenous leukaemia
myelofibrosis with myeloid metaplasia
sickle cell disease
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...
Fanconi anaemia (FA) is a rare disorder characterised by progressive bone marrow failure, various congenital abnormalities, and predisposition to malignancies (often acute myeloid leukaemia). It is considered the commonest type of inherited marrow failure syndrome 7.
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
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...
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...
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...
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...
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...
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...
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
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)
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...
Haemolytic uraemic syndrome (HUS) is a multisystem thrombotic microangiopathic disease characterised by the triad of renal failure, haemolytic anaemia and thrombocytopenia. It is the most common cause of renal failure in infancy and childhood requiring dialysis.
There are two forms of this syn...
Haemophilia is an inherited bleeding disorder which is X-linked recessive and therefore occurs almost exclusively in males. There are two subtypes - haemophilia A (80%) and haemophilia B (20%).
The incidence of haemophilia A is around 1 in 5000 male births, and the incidence of h...
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...
Haemosiderosis is a general term referring to accumulation of haemosiderin, which particularly occurs in the reticuloendothelial system (RES) and does not cause organ damage.
Some causes include:
mainly depositional siderosis in RES
if >40 units transfused: t...
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...
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.
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.
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
Hepatic myeloid sarcoma is a collection of immature myeloid cells than can present as a mass (or as masses) in the liver. It is a rare complication of haematologic disease.
Hepatic myeloid sarcoma is also referred to as "granulocytic sarcoma" or "chloroma".
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...
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...
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 ...
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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...
Hypersplenism is a cytopaenia resulting from blood pooling in the spleen, and is almost always associated with splenomegaly.
There is an almost overwhelming list, some more common causes are given below 1,3,4:
congestive splenomegaly: cirrhosis, Budd-Chiari syndrome, por...
Idiopathic hypereosinophilic syndrome (IHES) is a leukoproliferative disorder and refers to a situation when there is an unexplained prolonged eosinophilia with associated organ system dysfunction. The condition can affect several organ systems which includes:
heart: cardiac involvement in idio...
Idiopathic portal hypertension (noncirrhotic portal hypertension or Banti syndrome) is a term that has been given to portal hypertension occurring without hepatic cirrhosis, parasitic infection, or portal venous thrombosis.
Rare condition. More common in India and Japan.
The human body regularly encounters harmful microorganisms, and because of this it has developed a system of defences to help identify and eliminate infective pathogens in the body, known as the Immune system.
Humans have two types of immunity: innate immunity and acquired immunity.
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...
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:
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
The Lugano classification recommends two methods of visualization for standing lymphoma:
Computer tomography (CT)
Positron emission tomography–computed tomography (PET/CT)
Response Assessment on CT
CT uses for the standing of all types of lymphoma (if CT is performed for tumor size measure...
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...
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...
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 ...
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...
Mucosa associated lymphoid tissue (MALT) lymphoma, also called extranodal marginal zone B-cell lymphoma, is a type of low-grade extranodal lymphoma.
MALT lymphoma represents ~7.5% of non-Hodgkin lymphomas. Average age of presentation is 60 years with a slight female predominance ...
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...
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...
Mononucleosis is the term for infection with Epstein Barr Virus (EBV). The infection classically occurs in teenagers and young adults. It usually is diagnosed clinically and with serum lab work, but also may have suggestive imaging findings.
Exposure to EBV, but symptoms are often...
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...
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...
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...
Muscle lymphoma is a rare manifestation of lymphoma.
Muscle lymphoma is rare, representing <2% of all lymphomas. Average age of presentation is 70 years 1.
Focal swelling and/or pain along with B-type symptoms 2. Any muscle can be ...
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...
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)
Myeloproliferative disorders (MPDs) are a diverse group of conditions that are characterised by the overproduction of red cells, white cells and/or platelets in bone marrow. There are numerous conditions considered in this group but the most common are:
Neurolymphomatosis (NL) is a rare condition characterised by the direct invasion of the cranial and peripheral nerves and roots by lymphoma, in the great majority B-cell non-Hodgkin lymphoma.
It should be differentiated from other non-tumour conditions associated with lymphoma that also affect ...
Nodular lymphoid hyperplasia (NLH) is a type of rare, benign, lymphoproliferative disease. It is most commonly reported affecting the gastrointestinal and respiratory systems.
The presence of gut/mucosa associated lymphoid tissue (GALT/MALT) can be seen in children and young adults as a normal ...
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.
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
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
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...
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.
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...
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-...
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...
Pleural involvement with lymphoma can occur in two situations:
primary pleural lymphoma
secondary involvement of the pleura with lymphoma
POEMS syndrome is an acronym for a rare multisystem disorder comprising of a minimum of three of the following features in the setting of a plasma cell dyscrasia:
M: monoclonal gammopathy
S: skin changes (including hyperpigmentation and sk...
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.
Polyglandular autoimmune syndromes (PAS) are a rare set of diseases characterised by the presence of ≥2 autoimmune endocrine disease.
Three types of PAS have been described.
PAS type I
a.k.a. APECED (autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy) or MEDAC (mu...
Primary bone (skeletal/osseous) lymphoma (PBL) is a less common manifestation of lymphoma than secondary involvement from disseminated lymphoma. It is rare, accounting for <5% of bone tumours and <1% of non-Hodgkin lymphoma.
PBL is defined as the presence of lymphoma isolated to on...
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...
Primary pleural lymphoma is extremely rare, especially in immunocompetent patients.
Primary pleural lymphoma accounts for <0.5% of all non-Hodgkin lymphoma 2 and ~2.5% of primary chest wall tumours 4.
Primary pleural lymphoma may be Hodgkin or non-Hodgkin lymphoma wit...