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Treatment with alemtuzumab in a case of refractory primary cutaneous CD30-negative CD8-positive cytotoxic large T-cell lymphoma

, : Treatment with alemtuzumab in a case of refractory primary cutaneous CD30-negative CD8-positive cytotoxic large T-cell lymphoma. European Journal of Haematology 72(5): 377-378

Type 3 hemochromatosis is a rare autosomal recessive disorder due to mutations of the TFR2 gene. We describe clinical, biochemical and histopathologic findings of a patient with type 3 hemochromatosis at presentation and during a follow-up of more than 20 yr and we evaluate the effect of an associated beta-thalassemia trait on phenotypic expression. At the age of 33 yr the patient showed a marked iron overload and severe iron-related complications. After removal of 26 g of iron by subcutaneous deferoxamine infusion a marked clinical improvement was observed. Liver biopsies, performed at the age of 34 and 49 yr, indicate that in type 3 hemochromatosis there is a progressive hepatocellular iron accumulation from Rappaport's zone 1-3 and that iron loading in sinusoidal and portal macrophages occurs only in the more advanced stage. As observed in HFE hemochromatosis, the beta-thalassemia trait seems to aggravate the clinical picture of patients lacking TFR2, favoring higher rates of iron accumulation probably by activation of the erythroid iron regulator.


PMID: 15059077

DOI: 10.1111/j.1600-0609.2004.00232.x

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