Articles

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336 results found
Article

Chédiak-Higashi syndrome

Chédiak-Higashi syndrome is a rare, potentially lethal, multisystemic disorder, characterized by congenital immunocompromise with susceptibility to bacterial infections, a bleeding diathesis, partial albinism affecting the skin and eyes, and gradual neurological decline 2,3. Epidemiology The e...
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Light chain amyloidosis

Amyloid light chain (AL) amyloidosis or immunoglobulin light chain amyloidosis is a systemic amyloidosis and a plasma cell proliferative disorder characterized by deposition of misfolded monoclonal kappa or lambda light chains produced by clonal plasma cells 1-6. Epidemiology Amyloid light cha...
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Leukemoid reaction

A leukemoid reaction refers to a marked increase in white blood cells (>50000 cells/mL), typically neutrophils, which manifests as an acute inflammatory reaction that can mimic leukemia. Pathology Leukemoid reactions often occur from accelerated release of cells from the bone marrow. Features...
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Gallbladder lymphoma

Gallbladder lymphoma is exceedingly rare and presents diagnostic challenges due to its imaging characteristics often resembling those of gallbladder adenocarcinoma. Epidemiology Primary lymphomas of the gallbladder are extremely rare, accounting for approximately 0.1-0.2% of all malignant tumo...
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Hemorrhagic shock and encephalopathy syndrome

Hemorrhagic shock and encephalopathy syndrome (HSES) is a rare pediatric encephalopathy syndrome with a high mortality rate. Epidemiology Hemorrhagic shock and encephalopathy syndrome is considered rare, although the exact global incidence and prevalence is not known. The condition occurs in i...
Article

Systemic lupus erythematosus (mnemonic)

A mnemonic to remember the clinical features of systemic lupus erythematosus is: MD SOAP BRAIN Mnemonic M: malar "butterfly" rash D: discoid rash S: serositis O: oral ulcers A: ANA positive P: photosensitivity, pleuritis/pericarditis B: blood (hematologic) abnormality R: renal disease ...
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Anemia of chronic disease

Anemia of chronic disease, also known as anemia of inflammation, is a type of anemia caused by chronic inflammation.  Terminology According to some sources, the term 'anemia of inflammation' should replace, or has already replaced, the terms 'anemia of chronic disease' and 'anemia of chronic d...
Article

Hereditary elliptocytosis

Hereditary elliptocytosis, also known as hereditary ovalocytosis, is a condition which results from varying genetic mutations that lead to the formation of abnormally shaped red blood cells that are ovoid. It may cause a hemolytic anemia. Epidemiology There are various geographic trends for t...
Article

Agammaglobulinemia

Agammaglobulinemia is a rare inherited immunodeficiency disorder, characterized by a complete absence of mature B cells, which can result in severe antibody deficiency and recurrent infections 1. This is in contrast to hypogammaglobulinaemia where there is a reduction in all types of gammaglobul...
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Apheresis

Apheresis is an extracorporeal procedure in which the main components of blood (red blood cells, plasma, and plasma proteins) are separated and removed from the body. It is used as therapeutic measure for certain conditions. Radiological implications It is one of the indications of placement o...
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Yttrium-90 ibritumomab tiuxetan

Yttrium-90 ibritumomab tiuxetan, also known by the trade name Zevalin (Acrotech Biopharma LLC, USA), is a theranostic radiopharmaceutical approved for the treatment of patients with relapsed or refractory low grade or follicular non-Hodgkin lymphoma (NHL). More specifically, it is a radioimmunot...
Article

Lederer-Brill disease

Lederer-Brill disease is a transient autoimmune hemolytic anemia with very variable clinical features 1. Epidemiology Lederer-Brill disease is slightly more prevalent in females 1. Clinical presentation It usually occurs in children with a recent history of infection. Symptoms and signs incl...
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Nezelof syndrome

Nezelof syndrome is a hereditary primary immunodeficiency state caused by thymic dysplasia, lack of T cell function and normal levels of immunoglobulins. Epidemiology Nezelof syndrome is extremely rare. Clinical presentation Common clinical features include 4,5 failure to thrive metaphysea...
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X-linked agammaglobulinemia

X-linked agammaglobulinemia also known as Bruton disease or is a hereditary condition due to a mutation in the Bruton tyrosine kinase (BTK) gene located on the long arm of the X-chromosome. BTK is critical in the maturation of pre-B cells to mature B cells. Clinical presentation Recurring bact...
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Prothrombin 20210 mutation

Prothrombin 20210 mutation is a prothrombotic hematological condition which predisposes individuals to thromboembolic events such as deep venous thrombosis cerebral venous thrombosis cerebrovascular events pulmonary embolism Epidemiology It is considered the second most commonly inherited ...
Article

Splenic ultrasound

Splenic ultrasound is a non-invasive diagnostic imaging method that uses high-frequency sound waves to visualize and assess the size, shape, structure, and potential abnormalities of the spleen. Indication trauma: splenic injuries resulting from accidents, sports injuries, or any other form of...
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Fat-containing splenic lesions

Fat-containing splenic lesions are rare, and the differential diagnosis is limited. Differential diagnosis Neoplastic splenic hamartoma 1 splenic myelolipoma 2 splenic lipoma splenic liposarcoma 3 splenic angiomyolipoma Non-neoplastic Non-mass and pseudo-lesions may also occasionally co...
Article

Anti Sjögren syndrome related antigen antibodies

Anti Sjögren’s syndrome related antigen antibodies are a group of antibodies against autoantigens which include Ro/SSA anti-Ro52 anti-Ro60 La/SSB They are currently thought to be associated a number of autoimmune conditions. They were originally identified in patients with Sjögren syndrom...
Article

Evans syndrome

Evans syndrome is an autoimmune condition with two or more cytopenias, usually caused by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), with or without immune neutropenia. Epidemiology Evans syndrome is considered rare, with defining bicytopenias present in less than 5% ...
Article

Disseminated histoplasmosis

Disseminated histoplasmosis, also known as progressive disseminated histoplasmosis, is a severe form of histoplasmosis infection typically seen in immunosuppressed patients, especially in the setting of HIV infection. It results from hematogenous dissemination of the infection, involving multipl...

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