Sixteen patients with idiopathic myelofibrosis (IM) have been investigated with respect to the possibility that immune mechanisms may be of importance in the pathogenesis of bone marrow fibrosis. The following points appear relevant: (1) immune-complexes (IC) are detectable with different techniques in a high percentage of patients with IM. Their presence is associated with evidence of bone-marrow histological markers of immune activity. (2) IgG is the main Ig class in the composition of IM IC. The results obtained favour the hypothesis that autoimmune mechanisms are involved in IM patients.
In a mature ovarian cystic teratoma (MOCT) in a 67-year-old woman we found associated invasive squamous cell carcinoma and nodular amelanotic malignant melanoma. The finding of foci of typical and atypical melanocytic proliferation at the junctional level of the dermal component together with the absence of other possible sources supports an ovarian origin of the melanoma. A comparative analysis of the reported MOCT-associated malignant melanomas emphasizes the singularity of our case in the amelanotic character of the melanoma, its lymphotropism and the coexistence of invasive squamous cell carcinoma.
Primary pigmented micronodular disease is a peculiar form of ACTH-independent Cushing's syndrome characterized by the familial occurrence, the frequent association with malformations and the pathological adrenocortical picture consisting in micronodules with cellular deposition of lipofuscinic pigment. We describe here a case occurring in a 14-year-old girl.
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