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...
Primary pulmonary lymphoma (PPL) refers to a clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is a much rarer type of pulmonary lymphoma and is most frequently represented by lymphoma of B cell lineage - often marginal zone B-cell lymphoma o...
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...
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...
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...
The Rai staging system is one of the two staging systems currently adopted in assessment of chronic lymphocytic leukaemia (CLL).
It comprises of stages 0 to IV and classifies chronic lymphocytic leukemia into low, intermediate and high-risk categories, which correspond with stages 0, I & II, an...
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...
Reed-Sternberg cells are a classical finding diagnostic of Hodgkin lymphoma. They are giant, multinucleated cells with abundant pale cytoplasm. Reed-Sternberg cells are rare, making up <1% of lymphoid tissue, with the background comprised of lymphocytes, plasma cells, eosinophils and macrophages.
Renal lymphoma is usually seen as a part of spectrum of multi-systemic lymphoma, however, rarely may be seen as a primary disease.
While renal lymphoma has autopsy incidence of 30-60% in lymphoma patients, actual CT diagnosis incidence is ~ 5%1.
The kidneys are the most common ab...
The reticuloendothelial system (RES) comprises of a number of tissues:
liver Kupffer cells
Retrorenal spleen refers to variant position of the spleen, when its inferior portion lies posterior to the upper pole of the left kidney.
This normal variant occurs in ~20% of patients, with the finding more common in patients who lie supine 1.
Recognition of this variant is important to avoi...
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...
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....
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.
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.
Secondary hepatic involvement with lymphoma (secondary hepatic lymphoma) is common, much more so than primary hepatic lymphoma.
Hepatomegaly with deranged liver function tests is the most common presentation. Jaundice is common. Rarely, patients may present with acute li...
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...
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.
Secondary pulmonary lymphoma (SPL) refers to pulmonary involvement with lymphoma when the condition is not limited to the lung and has mediastinal lymph node involvement or evidence of extrathoracic dissemination for at least three months after the initial diagnosis. This is a more common form o...
Sézary syndrome (SS) is a type of cutaneous T cell lymphoma. It is sometimes considered an advanced and leukaemic form of cutaneous T cell lymphoma (CTCL).
It is clinically characterised by an extensive erythematous rash covering most of the body as well as presence of ma...
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. ...
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 ...
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...
Small lymphocytic lymphoma (SLL) is a non-Hodgkin lymphoma affecting the B-lymphocytes of the immune system. It is considered the same disease process as chronic lymphocytic leukaemia (CLL) although unlike CLL, the tumour cells are mostly confined to the lymph nodes rather than the blood stream...
Splenic cysts, although not particularly common, are the most common focal lesion of the spleen. They may be congenital or secondary.
The incidence is ~0.75 per 100,000.
Usually asymptomatic and incidentally discovered at imaging. Left upper quadrant pain and t...
Splenic (artery) embolisation is an endovascular technique for treatment of splenic and splenic artery pathology as an alternative to splenic artery ligation or splenectomy. It often results in successfully treating the underlying pathology, while maintaining at least partial splenic function.
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...
Splenic hamartomas are very rare and usually solitary although may be present as multiple nodules present in tuberous sclerosis or Wiskott-Aldrich syndrome.
The only recently described entity sclerosing angiomatoid nodular transformation (SANT) of the spleen, a non-neoplastic vascular entity de...
Splenic metastases are considered a rare clinical diagnosis, although they are more commonly found on autopsy. Typically they are part of widespread metastatic disease.
The rate of splenic metastases vary between 1-10% of autopsy studies, depending on whether microscopic or macros...
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...
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...
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.
Sweet syndrome (acute febrile neutrophilic dermatosis) is characterised by a constellation of clinical symptoms, physical features, and pathologic findings which include:
tender erythematous skin lesions (papules, nodules, and plaques)
a diffuse infiltrate consisting predo...
T cell prolymphocytic leukaemia (T-PLL) is a rare and unusual haematological malignancy.
It represents around 2% of all mature lymphocytic leukaemias in adults over the age of 30 1. It usually affects older adults with an average age at presentation being around 65 years. There ma...
Testicular leukemia can be seen in patients during and after acute leukemia. The blood-testis barrier limits chemotherapy from reaching the testicle, and therefore the testicle can act as a harbor for leukaemic cells.
Typically presents with painless testicular enlargemen...
Testicular lymphoma is an uncommon cause of testicular malignancy. Lymphoma can involve the testes in three ways:
primary site of extranodal disease (primary testicular lymphoma)
secondary involvement of systemic disease
primary manifestation of subclinical systemic disease
This article is c...
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...
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...
Tower vertebrae a rare manifestation of sickle cell disease, in which short infarcted vertebrae are seen adjacent to other abnormally grown in height vertebrae.
codfish or h-shaped vertebrae
anterior vertebral vascular notches
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.
Tumours associated with increased erythropoetin are varied and numerous. Patients will be polycythaemic due to erythropoetin secretion. Tumours in this group include:
renal cell carcinoma (RCC) 2
hepatocellular carcinoma (HCC)
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 twin-to-twin transfusion syndrome cases 2.
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...
Uterine lymphoma refers to involvement of the uterus with lymphoma. Some authors also place lymphoma of the uterine cervix under this group.
It is rare condition with initial uterine involvement occurring in only 1% of patients with lymphoma 3. However, uterine involvement is more...
Vaginal lymphoma can refer to:
secondary involvement of the vagina (secondary vaginal lymphoma) from widespread generalised lymphoma
usually comprises of diffuse large cell B non-Hodgkin's lymphoma (DLBCL) 2
primary vaginal lymphoma
Vanishing vertebrae is a rare ischaemic manifestation of sickle cell disease, in which a completely infarcted vertebral body literally disappears or vanishes, as a result of infarction. In the few reported cases, the posterior elements remain intact.
codfish or h-shaped vertebrae
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.
Waldenström macroglobulinaemia, previously also known as lymphoplasmacytic lymphoma, is a type of B-cell lymphoma. It is a rare condition, accounting for only 1% of all lymphoproliferative disorders.
Recent publications classify Waldenström macroglobulinaemia as an lymphoplasmacytic lymphoma w...
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, ...
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