loading...| Preferred Name |
T-cell prolymphocytic leukemia |
|
| Synonyms |
T-PLL T-cell chronic lymphocytic leukemia |
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| Definitions |
A rare mature T-cell neoplasm characterized by proliferation of small to medium-sized prolymphocytes with a mature post-thymic T-cell phenotype, involving the peripheral blood, bone marrow, lymph nodes, liver, spleen, and sometimes the skin. T-cell receptor genes are clonally rearranged. Patients typically present with hepatosplenomegaly, generalized lymphadenopathy, high leukocyte count with normal serum immunoglobulins, anemia, and thrombocytopenia. HTLV-1 serology is negative. The disease course is aggressive with generally poor prognosis. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_86871 |
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| alternative_term |
T-PLL T-cell chronic lymphocytic leukemia |
|
| definition |
A rare mature T-cell neoplasm characterized by proliferation of small to medium-sized prolymphocytes with a mature post-thymic T-cell phenotype, involving the peripheral blood, bone marrow, lymph nodes, liver, spleen, and sometimes the skin. T-cell receptor genes are clonally rearranged. Patients typically present with hepatosplenomegaly, generalized lymphadenopathy, high leukocyte count with normal serum immunoglobulins, anemia, and thrombocytopenia. HTLV-1 serology is negative. The disease course is aggressive with generally poor prognosis. |
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| definition_citation |
Orphanet |
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=86871 |
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| has_age_of_onset |
Adult Elderly |
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| has_inheritance |
Not applicable |
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| hasDbXref |
ICD-10:C91.6 UMLS:C2363142 MedDRA:10042985 ICD-11:2A90.0 MeSH:D015461 |
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| label |
T-cell prolymphocytic leukemia |
|
| notation |
ORPHA:86871 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_86871 |
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| prefLabel |
T-cell prolymphocytic leukemia |
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| present_in |
Worldwide AND has_annual_incidence_range : Unknown Worldwide AND has_point_prevalence_range : Unknown |
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| treeView | ||
| subClassOf |