Acute myeloid dendritic cell leukemia
Encyclopedia
Acute myeloid dendritic cell leukemia is an exceedingly rare form of leukemia. This form of leukemia represents only about 0.8% of all cases of acute myeloid leukemia
Acute myeloid leukemia
Acute myeloid leukemia , also known as acute myelogenous leukemia, is a cancer of the myeloid line of blood cells, characterized by the rapid growth of abnormal white blood cells that accumulate in the bone marrow and interfere with the production of normal blood cells. AML is the most common acute...

. Dendritic cells function as antigen-presenting cells. They process antigen material and present it on the surface to other cells of the immune system. Dendritic cells develop from progenitors in the bone marrow and transform into two subtypes: the myeloid dendritic cell and the plasmacytoid dendritic cell
Plasmacytoid dendritic cell
Plasmacytoid dendritic cells are innate immune cells that circulate in the blood and are found in peripheral lymphoid organs. They constitute...

. Leukemic transformation can occur in any of these two cells, but transformation of myeloid dendritic cell is less common and it leads to a form of leukemia known as acute myeloid dendritic cell leukemia.

Molecular findings, cytochemistry, and molecular genetics

The WHO
Who
Who may refer to:* Who , an English-language pronoun* who , a Unix command* Who?, one of the Five Ws in journalism- Art and entertainment :* Who? , a 1958 novel by Algis Budrys...

 criterion for diagnosis of AML is that myeloid precursors or blasts must represent at least 20% of the cellularity of bone marrow. In the case of acute myeloid dendritic cell leukemia, the blast cells are positive for markers of dendritic cells or monocytes. The markers include CD11c
CD11c
CD11c, also known as Integrin, alpha X , is a human gene.CD11c is a type I transmembrane protein found at high levels on most human dendritic cells, but also on monocytes, macrophages, neutrophils, and some B cells that induces cellular activation and helps trigger neutrophil respiratory burst;...

, CD80
CD80
Cluster of Differentiation 80 is a protein found on activated B cells and monocytes that provides a costimulatory signal necessary for T cell activation and survival...

, CD83
CD83
CD83 is a human protein encoded by the gene....

, CD86
CD86
Cluster of Differentiation 86 is a protein expressed on antigen-presenting cells that provides costimulatory signals necessary for T cell activation and survival...

, and major histocompatibility complex
Major histocompatibility complex
Major histocompatibility complex is a cell surface molecule encoded by a large gene family in all vertebrates. MHC molecules mediate interactions of leukocytes, also called white blood cells , which are immune cells, with other leukocytes or body cells...

 class II antigens. The neoplastic dendritc cells are negeative for the enzymes myeloperoxidase
Myeloperoxidase
Myeloperoxidase is a peroxidase enzyme that in humans is encoded by the MPO gene. Myeloperoxidase is most abundantly expressed in neutrophil granulocytes . It is a lysosomal protein stored in azurophilic granules of the neutrophil...

 and esterase
Esterase
An esterase is a hydrolase enzyme that splits esters into an acid and an alcohol in a chemical reaction with water called hydrolysis.A wide range of different esterases exist that differ in their substrate specificity, their protein structure, and their biological function.- EC classification/list...

. The cells elaborate many cytokines including IL-6 and IL-12
IL-12
IL-12 or IL 12 can refer to:Medicine* Interleukin 12Airplanes* Ilyushin Il-12Politics* Illinois' 12th congressional districtRoads* Illinois Route 12...

.

Clinical findings

Anemia, thrombocytopenia, and blood, marrow, and skin involvement with dendritic-like blast and more mature appearing dendritic cells are characteristic findings. Lymph node and spleen enlargement from leukemic cell infiltration usually is present.
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