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...
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...
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...
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...
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...
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 ...
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...
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...
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...