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Haemophilic arthropathy

Haemophilic arthropathy refers to permanent joint disease occurring in hemophilia sufferers as a long-term consequence of repeated hemarthrosis. Epidemiology Around 50% of patients with hemophilia will develop severe arthropathy. Clinical presentation Presents similarly to osteoarthritis, w...

Orbital lymphoma

Primary lymphoma of the orbit is one of the most common orbital tumors and accounts for as much as half of all orbital malignancies. It is a B-cell non-Hodgkin lymphoma, and in most cases arises from mucosa-associated lymphoid tissue (MALT). Epidemiology Orbital lymphomas account for only 2% o...

Hemophilia

Hemophilia is an inherited bleeding disorder that is mainly X-linked recessive and therefore occurs almost exclusively in males. There are two main subtypes: hemophilia A (80%) and hemophilia B (20%). Epidemiology The incidence of hemophilia A is around 1 in 5000 male births, and the incidenc...

Primary mediastinal large B-cell lymphoma

Primary mediastinal large B-cell lymphoma is a distinct entity, recognized in the WHO classification of lymphoma. Epidemiology Primary mediastinal large B-cell lymphoma accounts approximately 5% of large B-cell lymphoma, which is usually disseminated or found in the abdomen. There appears to b...

WHO classification of haematolymphoid tumors

The World Health Organization (WHO) classification of haematolymphoid tumors is the most widely used pathologic classification system for hematopoietic and lymphoid neoplasms. The current revision 1, known as the 5th edition, was published in 2022 and supersedes the 4th edition revised published...

Lymphoma

Lymphoma (historically lymphosarcoma was used for diffuse forms of the disease) 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 a...

Non-lymphomatous pulmonary lymphoid disorders

There are several non-lymphomatous lymphoid disorders that can affect the lung. This implies that they consist of lymphoid tissue but may not have progressed as far as an overt lymphoma. They include: Castleman disease plasma-cell granuloma lymphocytic interstitial pneumonia angioimmunoblast...

Tumors associated with increased erythropoetin

Tumors associated with increased erythropoetin are varied and numerous. Patients will be polycythemic due to erythropoetin secretion. Tumors in this group include: renal cell carcinoma (RCC) 2 hepatocellular carcinoma (HCC) hemangioblastoma

Lymphomatoid granulomatosis (pulmonary manifestations)

Pulmonary manifestations of lymphomatoid granulomatosis are important since the lung is one of the most frequent sites of involvement in lymphomatoid granulomatosis. It falls under the group of pulmonary angiitis and granulomatosis. Pathology Some consider the condition to be midway between ov...

Lymphomatoid granulomatosis

Lymphomatoid granulomatosis, also known as angiocentric lymphoma or angiocentric immunoproliferative lesion, is a rare type of non-Hodgkin's lymphoma associated with Epstein-Barr virus infection. Pathology There is a recognized association with antecedent infection with the Epstein Barr virus...

Hodgkin lymphoma (pulmonary manifestations)

Pulmonary manifestations of Hodgkin lymphoma are relatively rare, present in 5-12% of patients at the time of diagnosis. It is relatively more common with the nodular sclerosing subtype. Pulmonary involvement usually indicates stage IV disease. Radiographic features Bilateral involvement is ...

POEMS syndrome

POEMS syndrome is the acronymic name for a rare multisystem paraneoplastic disorder including the following features: P: polyneuropathy O: organomegaly E: endocrinopathy M: monoclonal gammopathy S: skin changes Clinical presentation The diagnostic criteria differ from the acronym. Accordi...

Autosplenectomy

Autosplenectomy denotes spontaneous infarction of the spleen with resulting hyposplenism. Epidemiology Autosplenectomy is most frequently encountered in patients with homozygous sickle cell disease, although it has also been reported in pneumococcal septicemia 1, and systemic lupus erythematos...

Primary hepatic lymphoma

Primary hepatic lymphoma (PHL) is very rare, with approximately 100 described cases. If it is being considered as a diagnosis, distant lymphadenopathy, splenomegaly, bone marrow disease, and leukemia should not be present for at least 6 months after the liver tumor has been detected (see: second...

Sickle cell disease (acute chest syndrome)

Acute chest syndrome in sickle cell disease is a leading thoracic complication - as well as leading cause of mortality - in those affected by sickle cell disease. The diagnosis is made on the combination of new pulmonary opacities on chest radiograph with at least one new clinical symptom or sig...

Small bowel lymphoma

Small bowel lymphoma is one of the most common small bowel malignancies, accounting for ~25% of all primary small bowel malignancies, and ~40% of all primary gastrointestinal lymphomas. Epidemiology Small bowel lymphoma is most commonly secondary extranodal involvement in widespread systemic l...

Splenunculus

Splenunculi, also known as supernumerary spleens, accessory spleens, or splenules, are small nodules of spleen that are separate from the rest of the organ. Epidemiology They are common, seen in up to 16% of CTs of the abdomen and up to 30% of autopsies 2. Pathology Accessory spleens are c...

Gaucher disease

Gaucher disease is the most common lysosomal storage disorder in humans. It is an autosomal recessive, multisystem disease arising from a deficiency of glucocerebrosidase or beta-glucosidase activity, resulting in the accumulation of a glycolipid (glucocerebroside) within the lysosomes of macrop...

Splenomegaly

Splenomegaly refers to enlargement of the spleen. The upper limit of the normal adult splenic length is traditionally cited at 12 cm, but lengths upwards of 14 cm can be seen in normal, taller males 7. Terminology Massive splenomegaly is variably defined, including when the spleen is 5 standar...

Lymph node enlargement

Lymph node enlargement (rarely lymphadenomegaly) is often used synonymously with lymphadenopathy, which is not strictly correct. Terminology Lymphadenopathy (or adenopathy) is, if anything, a broader term, referring to any pathology of lymph nodes, not necessarily resulting in increased size; ...

Typhlitis

Typhlitis, also known as neutropenic colitis, is a necrotizing inflammatory condition which typically originates in the cecum and, often extends into the ascending colon, appendix or terminal ileum, by the time of presentation. In general patients are immunocompromised, usually neutropenic. Ter...

Splenic cyst

Splenic epithelial cysts, also known as splenic epidermoid cysts or primary splenic cysts, are unilocular fluid lesions with thin and smooth walls and no enhancement. They represent ~20% of cysts found in the spleen, and are usually an innocuous incidental imaging finding. Note that most (~80%)...

Polyglandular autoimmune syndromes

Polyglandular autoimmune syndromes (PAS) are a rare set of diseases characterized by the presence of ≥2 autoimmune endocrine disease. Pathology Three types of PAS have been described. PAS type I a.k.a. APECED (autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy) or MEDAC (mu...

Pulmonary Pneumocystis jiroveci infection

Pulmonary Pneumocystis jiroveci infection, also known as Pneumocystis jiroveci pneumonia (PJP) or Pneumocystis pneumonia (PCP), is an atypical pulmonary infection and the most common opportunistic infection in patients with acquired immunodeficiency syndrome (AIDS). Terminology Classically, "P...

Lead poisoning

Lead poisoning or plumbism refers to the multi-organ toxicity exerted by exposure to lead. Manifestations differ based on a myriad of features including chronicity, exposure intensity, and age. Neurologic toxicity and hematologic toxicity are common features. Clinical manifestations vary, rangin...

Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare multisystem disease with a wide and heterogeneous clinical spectrum and variable extent of involvement. Terminology Langerhans cell histiocytosis was previously known as histiocytosis X. The newer term is preferred as it is more descriptive of its...

Lymphoma (staging)

Many lymphoma staging systems for both Hodgkin lymphoma and non-Hodgkin lymphoma have been developed. The most widely used currently is the Lugano staging classification, which also separately defines criteria for response to treatment assessed by PET-CT or by CT alone. Patients having undergon...

Hodgkin lymphoma

Hodgkin lymphoma or Hodgkin disease (HD) is a type of lymphoma and accounts for ~1% of all cancers. Hodgkin disease spreads contiguously and predictably along lymphatic pathways and is curable in ~90% of cases, depending on its stage and subtype. Epidemiology There is a bimodal distribution in...

Sinusoidal obstruction syndrome

Sinusoidal obstruction syndrome (SOS), previously known as hepatic veno-occlusive disease (VOD), is a condition arising from occlusion of hepatic venules. Clinical presentation right upper quadrant pain painful hepatomegaly ascites abnormal liver function tests Pathology Toxic injury to l...

Hemochromatosis

Hemochromatosis is an iron overload disorder characterized 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 of organ dysfunction. This article focuses on the general principles of ...

H-shaped vertebra

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 disease, and results from microvascular endplate infarction (Figure 1) 3. It may occasionall...

Breast lymphoma

Breast lymphoma refers to the involvement of the breast with lymphoma and may be primary or secondary. Epidemiology Both primary and secondary breast lymphomas are rare. Breast lymphoma accounts for <1% of all breast malignancies and <2% of all extranodal non-Hodgkin lymphoma 11. Secondary lym...

Bone marrow

Normal bone marrow is divided into red and yellow marrow, a distinction made on the grounds of how much fat it contains. Gross anatomy Red marrow is composed of: hematopoietic cells supporting stroma reticulum (phagocytes and undifferentiated progenitor cells) scattered fat cells rich va...

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