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...
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...
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.
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...
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...
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 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...
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...
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...
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 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...
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 ...
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 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.
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 ...
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 ...
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 ...
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 ...
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.
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...
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...
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.
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...
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...
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...
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...
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....
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 an 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. The highest ...
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...
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...
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...
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...
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
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...