loading...| Preferred Name |
Lymphohistiocytosis, Hemophagocytic |
|
| Synonyms |
|
|
| Definitions |
A lymphatic system disease that is characterized by an expansion of the monocyte-macrophage population and intense hemophagocytosis. It can occur de novo, but more often occurs in the setting of another disorder, usually an infection or a malignancy. A clinical picture of fever, hepatosplenomegaly, lymphadenopathy and peripheral pancytopenia. The morphologic hallmark of this syndrome is the phagocytosis of hematopoietic elements by morphologically normal macrophages. |
|
| ID |
http://www.phoc.org.cn/pmo/class/PMO_00037399 |
|
| Database_Cross_Reference |
OMIM:MTHU038295 NDFRT:N0000171663 MTH:NOCODE MDR:10071580 MEDCIN:351297 SNOMEDCT_US:234437005 SNOMEDCT_US:190958003 NCI:C34792 MDR:10071583 ICD10:D76.1 ICD10AM:D76.1 NCI_NCI-GLOSS:CDR0000488415 RCD:X20E8 NCI_NCI-GLOSS:CDR0000523322 DO:DOID:0050120 PDQ:CDR0000486910 MSH:D051359 ICD10CM:D76.1 |
|
| Definition |
A lymphatic system disease that is characterized by an expansion of the monocyte-macrophage population and intense hemophagocytosis. It can occur de novo, but more often occurs in the setting of another disorder, usually an infection or a malignancy. A clinical picture of fever, hepatosplenomegaly, lymphadenopathy and peripheral pancytopenia. The morphologic hallmark of this syndrome is the phagocytosis of hematopoietic elements by morphologically normal macrophages. |
|
| label |
Lymphohistiocytosis, Hemophagocytic |
|
| MCID |
MC00007753 |
|
| PMOID |
PMO:00037399 |
|
| prefixIRI |
pmo:PMO_00037399 |
|
| prefLabel |
Lymphohistiocytosis, Hemophagocytic |
|
| Synonym |
Hemophagocytic Syndromes Haemophagocytic lymphohistiocytosis (disorder) Haemophagocyt lymphohistiocyto Lymphohistiocytosis, Hemophagocytic [Disease/Finding] Hemophagocytic Lymphohistiocytosis Hemophagocytic Lymphohistiocytoses Hemophagocytic Disorder Hemophagocytic Syndrome hemophagocytic lymphohistiocytosis (diagnosis) Hemophagocytic lymphohistiocytosis Lymphohistiocytoses, Hemophagocytic HLH HPS Haemophagocytic lymphohistiocytosis haemophagocytic syndrome Hemophagocytic lymphohistiocytosis (disorder) hemophagocytic lymphohistiocytosis Hemophagocytic syndrome |
|
| Tree Number |
T9.5.1.11.1.3 |
|
| subClassOf |