[A woman with Sezary syndrome developed HTLV-1-associated myeloneuropathy. The reported case supports the theory of etiological connection].
The patient was a 58-year-old woman with a 10-year history of severely itching erythroderma, as well as enlarged lymph glands and circulating Sézary cells (i.e. Sézary's syndrome). Histological analysis of a skin biopsy revealed Pautrier's microabcesses with atypical lymphocytes characteristic of T-cell lymphoma. Her lymph glands were also found to contain atypical lymphocytes. Parallel to the skin disorder, the patient developed paraparesis with fainting strength of the musculature of the extremities. Ultimately, she was unable to walk. She had no feeling of vibration and had difficulties emptying her bladder. Babinski's sign was positive, bilaterally. Ten years before the present admission the patient's skin and blood lymphocytes were positive for sequences of human T-cell lymphoma virus-1 (HTLV-1) in a polymerase chain reaction (PCR). At the present admission, the patient presented a high titre of HTLV-1 antibodies both in the blood and cerebrospinal fluid on ELISA and Wesern blot analysis. The patient had been married to a man from Bolivia for 30 years. He was also strongly positive for HTLV-1 antibodies in blood. Adult T-leukemia (ATL) is common in areas where HTLV-1 is found endemically. ATL has skin symptoms similar to mycosis fungoides. The clinical findings in this patient indicate an etiological connection between some cases of T-cell lymphoma in the skin (Sézary's syndrome) and HTLV-1.
Roupe
Gösta
G
Avdelningen för dermatologi och venereologi, Sahlgrenska Universitetssjukhuset, Göteborg.
D020405:Dermis / Q000473:pathology; D004817:Epidermis / Q000473:pathology; D005260:Female; D015481:HTLV-I Antibodies / Q000032:analysis / Q000134:cerebrospinal fluid; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D015493:Paraparesis, Tropical Spastic / Q000276:immunology / Q000473:pathology / Q000821:virology*; D012751:Sezary Syndrome / Q000150:complications* / Q000276:immunology / Q000473:pathology / Q000821:virology; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology / Q000821:virology*
D015481:HTLV-I Antibodies