Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0019214 (
hepatosplenomegaly
)
4,408
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Histiocytic medullary reticulosis (HMR) is a rare, progressive, fatal reticuleondothelial proliferative disorder. It was diagnosed in a 10-year-old boy who had
pityriasis lichenoides et varioliformis acuta
of Mucha-Haberman which was controlled by dapsone for 2 years. One month after cessation of dapsone therapy, cutaneous tumors associated with fever, lymphadenopathy, and
hepatosplenomegaly
developed. Tissue biopsy specimens of skin, liver, spleen, lymph nodes, and a bone marrow aspirate demonstrated histiocytic erythrophagocytosis and atypical histiocytosis compatible with HMR. A rapidly progressing, fatal course followed despite intensive chemotherapy.
...
PMID:Histiocytic medullary reticulosis presenting as Mucha-Habermann disease. 7 36
