Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Progressive nodular histiocytosis is a proliferative process of histiocytes, the main element of which is the dermal dendrocyte. It is considered to form part of a group of histiocytic disorders related to juvenile xanthogranuloma, which also includes xanthoma disseminatum, benign cephalic histiocytosis, spindle cell xanthogranuloma and generalized eruptive histiocytosis; disorders which perhaps represent the spectrum of one single entity. We present the case of a 57-year-old man who, for 26 years, had had a progressively deforming process of cutaneous lesions, with systemic involvement, including chronic myeloid leukaemia, hepatosplenomegaly, hypothyroidism, hyperuricaemia and hypocholesterolaemia. We have not been able to establish precisely the relationship between these features.
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PMID:Progressive nodular histiocytosis accompanied by systemic disorders. 1153 23

In three patients, a girl aged six weeks and two boys aged eight and three months, juvenile xanthogranuloma was diagnosed. This is a rare cutaneous disorder that predominantly occurs during infancy and early childhood. It is one of the non-malignant, non-Langerhans cell histiocytoses (class II histiocytoses). It is characterized by a circumscript, firm, yellow-brown and sometimes pink-red nodule or papule located mainly on the face or scalp. Diagnosis can be made on the clinical appearance. However, this may need to be confirmed by a histopathological investigation, which includes immunohistochemical stains. In contrast to generalized histiocytoses, the prognosis of juvenile xanthogranuloma is excellent: the cutaneous tumour disappears spontaneously in one to six years. However, extracutaneous involvement, which is most frequently located in the eye, can occur. Moreover, juvenile xanthogranuloma is associated with neurofibromatosis type I and juvenile chronic myeloid leukaemia. This rare but possibly life-threatening systemic involvement justifies meticulous follow-up.
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PMID:[Juvenile xanthogranuloma: a form of histiocytosis with an excellent prognosis]. 1130 14