Cutaneous lymphoma—A spectrum

Abstract

In recent years evidence has been accumulating that mycosis fungoides and Sezary syndrome come under the ‘umbrella’ of cutaneous lymphoma. Cutaneous lymphoma cells are ‘T’ lymphocytes which have a tendency to localize in the skin and T cell regions of lymphoid tissue, and usually spare the bone marrow. Between 29/12/78 and 13/3/79 4 cases of cutaneous lymphoma presented to the Haematology-Oncology Unit in our hospital. They comprise a broad clinical and morphologic spectrum. Case 1 A 73-yr-old man with a 10 yr history of phenytoin treated petit mal epilepsy presented with a 6 mth history of a worsening maculopapular rash on legs, thighs, lower abdomen and back, and generalized lymphadenopathy and hepatosplenomegaly. Blood picture was Hb 12.2%, platelets 147,000/ml, W.C.C. 236,000/ml (96% lymphocytes). Skin biopsy revealed histological features of mycosis fungoides and marrow biopsy revealed diffuse involvement by the lymphoma. Cytogenetic analysis of the blood was grossly abnormal. This patient is currently responding very well to weekly courses of cyclophosphamide/vincristine/leucapheresis. Case 2 A 70-yr-old woman presented with a severe exfoliative dermatitis thought to be due to indomethacin. It was associated with generalized lymphadenopathy and hepatomegaly. Blood picture revealed Hb 4.5%, platelets 140,000/ml, and W.C.C. 61,000 (lymphocytes 9%, abnormal monocytes 10%, lymphoid blasts 14%). Skin biopsy-revealed histological features of mycosis fungoides, and in bone marrow aspirate lymphoid blasts comprising 50%, of nucleated cells. Seven per cent of cells were atypical monocytes with cleaved nuclei. Cytogenetic analysis of the marrow was abnormal. The patient has responded well to intermittent chemotherapy with prednisolone, cyclophosphamide and vincristine but therapy has been complicated by intercurrent problems. Case 3 A 55-yr-old man with progressive bilateral deafness, difficulty in speaking and dysphagia over 4 mth. At presentation he had generalized lymphadenopathy with very large tonsils and hepatosplenomegaly. He also complained of severe pruritus and developed a morbilliform rash on back, buttocks and anterior chest. Blood picture was Hb 15.1%, platelets 302.000/ml, W.C.C. 19,500/ml (12% monocytes—smear was otherwise normal). There was diffuse hyperglobulinaemia. Lymph node and bone marrow biopsy revealed a diffuse poorly differentiated lymphocytic (small cell non-cleaved) T cell lymphoma. Skin biopsy revealed features of mycosis fungoides. The patient has responded dramatically to intermittent COP therapy. Case 4 A 66-yr-old woman presented with progressive long standing skin lesions over L. scapula, upper anterior chest, right back and thighs. There was no lymphadenopathy or hepatosplenomegaly. Blood picture and bone marrow were normal. Biopsy of one of these lesions revealed deep skin infiltration by mononuclear cells which were characterized by regular non-convoluted nuclei. T-cell disease was diagnosed on skin and blood specimens. There was improvement with 150 rads TBI.