TY  - CHAP
M1  - Book, Section
TI  - Classification and Clinical Manifestations of Disorders of Monocytes and Macrophages
A1  - Lichtman, Marshall A.
A2  - Kaushansky, Kenneth
A2  - Lichtman, Marshall A.
A2  - Prchal, Josef T.
A2  - Levi, Marcel M.
A2  - Press, Oliver W.
A2  - Burns, Linda J.
A2  - Caligiuri, Michael
Y1  - 2015
N1  - 
T2  - Williams Hematology, 9e
AB  - SUMMARYDisorders  that  exclusively  result  in  abnormalities  of  monocytes,  macrophages,  or  dendritic  cells  are  uncommon  and  usually  are  referred  to,  pathologically,  as  histiocytosis.  These  disorders  can  be  inherited,  such  as  familial  hemophagocytic  lymphohistiocytosis;  inflammatory,  such  as  infectious  hemophagocytic  lymphohistiocytic  syndrome;  or  clonal  (neoplastic),  such  as  Langerhans  cell  histiocytosis.  They  can  result  from  an  inherited  enzyme  insufficiency  in  macrophages  that  lead  to  exaggerated  storage  of  macromolecules,  such  as  in  Gaucher  disease.  Monocytes  are  critical  sources  for  proinflammatory  and  inflammatory  cytokines  and,  when  inappropriately  activated,  can  result  in  the  lymphohistiocytic  hemophagocytic  syndrome  with  fever,  intravascular  coagulation,  and  organ  pathology.  A  variety  of  hematopoietic  neoplasms  may  have  a  phenotype  characterized  by  a  large  proportion  of  monocytes.  Idiopathic  (clonal)  monocytosis  is  a  rare  manifestation  of  a  myelodysplastic  syndrome.  Some  cases  of  myelogenous  leukemia  have  progenitor  cells  that  mature  preferentially  into  leukemic  monocytes,  including  acute  monoblastic  or  monocytic  leukemia,  chronic  myelomonocytic  leukemia,  and  juvenile  myelomonocytic  leukemia.  Two  acquired  diseases,  hairy  cell  leukemia  and  aplastic  anemia,  result  in  a  severe  depression  of  blood  monocytes  (along  with  other  blood  cell  types).  Mutations  in  GATA2  are  associated  with  severe  monocytopenia  and  mycobacterial  infections  (the  MonoMAC  syndrome).  Inherited  disorders  affecting  white  cells,  such  as  chronic  granulomatous  disease  and  Chédiak-Higashi  syndrome,  result  in  impaired  monocyte  function.  Monocyte  dysfunction  may  accompany  a  variety  of  severe  illnesses,  such  as  sepsis,  trauma,  and  cancer.  Monocytes  also  contribute  to  a  variety  of  diseases,  such  as  Crohn  disease  and  rheumatoid  arthritis,  by  virtue  of  their  being  a  principal  source  of  tumor  necrosis  factor.  Monocytes  play  a  pathogenetic  role  in  other  complex,  acquired  disorders,  such  as  thrombosis  and  atherogenesis.  Table  69–1  catalogues  the  qualitative  and  quantitative  abnormalities  of  monocytes,  macrophages,  and  dendritic  cells.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - hemonc.mhmedical.com/content.aspx?aid=1121096418
ER  -