Clinical and Experimental Dermatology (1979) 4, 241.
Clinical meeting of the St John's Hospital Dermatological Society: 2 March 1978
Chronic lymphocytic leukaemia with cutaneous involvement
ANDREW P.WARIN AND MARION M.ROBERTS St John's Hospital for Diseases of the Skin, Lisle Street, Leicester Square, London WC2H 7BJ and Imperial Cancer Research Eund Laboratories, Lincoln's Inn Fields, London WC2
Accepted for publication 15 August 1978
Case report Mrs. L.H. (P 88104), aged 80, presented with a 3 year history of a pruritic rash which started on the hands and gradually spread over the whole body. She had lost a substantial amount of weight. Examination revealed generalized lymphadenopathy and a widespread erythematous rash with some scaling. In places, the erythematous rash was palpable and appeared to be urticated. After a few days observation, it was noted that the rash had migrated and in places resembled a figurate erythema. As the erythematous border moved, so scaling resulted. Subsequently, the erythema became more generalized and the skin appeared to be diffusely infiltrated and pigmented (Fig. i), suggesting the diagnosis of Sezary syndrome.
Investigations and progress August 1977 Hb n o g%; ESR 30 mm in first hour; WCC 27,000/mm^, lymphocytes 84%—all mature^ small cells, neutrophils 14%, no Sezary cells were seen; IgH ^?V W Figure 3. Dense infiltrate with mononuclear cells. Many have large hyperchromatic nuclei. Some cells are invading the epidermis ( x 320).
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A.P.Warin and M.M.Roberts
The sheep red blood cell rosette test was performed with i x lo^ lymphocytes in medium and 50% foetal calf serum absorbed with sheep erythrocytes (100 //I) and 100 /d of 2% suspension of neuraminidase treated sheep erythrocytes. These were centrifuged at 400 g for 4 min and left undisturbed at room temperature for i h before gentle resuspension and counting in a haemocytometer. The anti-human immunoglobulin was a fluoresceinated Fab^ preparation and was used in a direct immunofiuorescence test. The other antibodies which were raised in rabbits were used in indirect immunofiuorescence tests with fiuoresceinated goat anti-rabbit immunoglobulin as a second layer. Fluorescence was assessed on viable cells in suspension with a Zeiss Standard 18 phase contrast microscope with epifiuorescence and narrow band FITC filters. The blood and bone marrow samples were collected in heparin and mononuclear cells separated by Ficoll-Triosil density gradient centrifugation. Peripheral blood surface markers
E rosette positive 6-4%; anti-T cell positive 70%; anti-Thy positive 0%; Smig positive 5%; anti-p28, 33 positive j % . A diagnosis of T-cell lymphocytic leukaemia with cutaneous involvement was made. Because of the value of psoralens and long-wave ultraviolet light treatment (PUVA) in mycosis fungoides (Hodge et al., 1977) and in Sezary syndrome (unpublished observations) she was treated with PUVA for symptomatic reasons. Her rash improved initially, but after 4 months it was not well controlled. PUVA was therefore discontinued and prednisolone 30 mg per day was started. Her pruritus and skin involvement were well controlled, and her dose of prednisolone was gradually reduced to 15 mg per day. However, she became very Cushingoid and attempts to reduce the dose further resulted in a severe recurrence of her erythroderma. Investigations in May 1978 Hb I2-O g%; ESR 41 mm in first hour; WCC 25,900/mm'—lymphocytes 40%, neutrophils
Clinical meeting of the St John's Hospital Dermatological Society: 2 March 1978
Chronic lymphocytic leukaemia with cutaneous involvement
ANDREW P.WARIN AND MARION M.ROBERTS St John's Hospital for Diseases of the Skin, Lisle Street, Leicester Square, London WC2H 7BJ and Imperial Cancer Research Eund Laboratories, Lincoln's Inn Fields, London WC2
Accepted for publication 15 August 1978
Case report Mrs. L.H. (P 88104), aged 80, presented with a 3 year history of a pruritic rash which started on the hands and gradually spread over the whole body. She had lost a substantial amount of weight. Examination revealed generalized lymphadenopathy and a widespread erythematous rash with some scaling. In places, the erythematous rash was palpable and appeared to be urticated. After a few days observation, it was noted that the rash had migrated and in places resembled a figurate erythema. As the erythematous border moved, so scaling resulted. Subsequently, the erythema became more generalized and the skin appeared to be diffusely infiltrated and pigmented (Fig. i), suggesting the diagnosis of Sezary syndrome.
Investigations and progress August 1977 Hb n o g%; ESR 30 mm in first hour; WCC 27,000/mm^, lymphocytes 84%—all mature^ small cells, neutrophils 14%, no Sezary cells were seen; IgH ^?V W Figure 3. Dense infiltrate with mononuclear cells. Many have large hyperchromatic nuclei. Some cells are invading the epidermis ( x 320).
243
244
A.P.Warin and M.M.Roberts
The sheep red blood cell rosette test was performed with i x lo^ lymphocytes in medium and 50% foetal calf serum absorbed with sheep erythrocytes (100 //I) and 100 /d of 2% suspension of neuraminidase treated sheep erythrocytes. These were centrifuged at 400 g for 4 min and left undisturbed at room temperature for i h before gentle resuspension and counting in a haemocytometer. The anti-human immunoglobulin was a fluoresceinated Fab^ preparation and was used in a direct immunofiuorescence test. The other antibodies which were raised in rabbits were used in indirect immunofiuorescence tests with fiuoresceinated goat anti-rabbit immunoglobulin as a second layer. Fluorescence was assessed on viable cells in suspension with a Zeiss Standard 18 phase contrast microscope with epifiuorescence and narrow band FITC filters. The blood and bone marrow samples were collected in heparin and mononuclear cells separated by Ficoll-Triosil density gradient centrifugation. Peripheral blood surface markers
E rosette positive 6-4%; anti-T cell positive 70%; anti-Thy positive 0%; Smig positive 5%; anti-p28, 33 positive j % . A diagnosis of T-cell lymphocytic leukaemia with cutaneous involvement was made. Because of the value of psoralens and long-wave ultraviolet light treatment (PUVA) in mycosis fungoides (Hodge et al., 1977) and in Sezary syndrome (unpublished observations) she was treated with PUVA for symptomatic reasons. Her rash improved initially, but after 4 months it was not well controlled. PUVA was therefore discontinued and prednisolone 30 mg per day was started. Her pruritus and skin involvement were well controlled, and her dose of prednisolone was gradually reduced to 15 mg per day. However, she became very Cushingoid and attempts to reduce the dose further resulted in a severe recurrence of her erythroderma. Investigations in May 1978 Hb I2-O g%; ESR 41 mm in first hour; WCC 25,900/mm'—lymphocytes 40%, neutrophils
