Sezary syndrome - Without erythroderma

Introduction

Sezary syndrome (SS), first described as a severe variant of cutaneous T cell lymphoma (CTCL) by Besnier and Hallopeau (1892)[1] was later defined by Sezary and Bouvrain (1938)[2] as the classical triad of pruritic erythroderma, lymphadenopathy and abnormal hyper convoluted monstrous cells in the peripheral blood. Though erythroderma is considered an invariable component of the syndrome, many recent reports showed that no single clinical feature is pathognomonic of SS and the clinical features should be correlated with histological, immunological and gene rearrangement studies.

We report case of advanced CTCL with classical haematological and histological features of SS but clinically lacking the classical component of erythrodema.

Case Report

A 66 year old man presented with multiple asymptomatic erythematous and skin coloured papules, plaques and nodules over the scalp, face, trunk and inguinal region [Figure - 1].

The lesions were hard, keratotic and fixed to the deeper structures. The lesions were of one month duration. He gave a history of jaundice for the last 6 months. He was a chronic smoker and alcoholic. Systemic examination showed generalized lymphadenopathy and a firm non- tender hepatomegaly.

The routine blood examination was normal, but the serum liver enzymes were raised. Serological investigations and serum electrophoresis were normal. Peripheral smear showed more than 60% of atypical cells with immature chromatin and inconspicuous nucleoli. FNAC from the lymph node and bone marrow aspiration cytology showed similar cells. In bone marrow trephine biopsy, there were focal infiltrations of atypical lymphocytes.

Skin biopsy from nodular lesion showed sheets of pleomorphic cells with scanty cytoplasm and hyperchroamatic nuclei and inconspicuous epidermotropism. The picture was consistent with advanced cutaneous T cell lymphoma. In immunohistochemistry the lymphocytes were CD - 45 Ro positive indicating that they were T helper cells. Thus the patient had a TNM score of T3 N3 Bt M1.

Discussion

Erythroderma in Sezary syndrome may start denovo or it may follow or complicate a plaque stage of mycosis fungoides. Histological peculiarities of Sezary syndrome as distinct from the plaque/tumour stage of MF are a highly pleomorphic inflirtate, pert vascular inflirtrate, less pronounced epidermotropism and variable infiltrate density. The diverse clinical and histopathological features in advanced CTCL makes a precise differentiation between tumour stage of MF and SS difficult.[3] Based on this Trotter et al[4] have suggested that the definition of SS can be further refined by including only patients with a circulating peripheral blood population of clonal T - cells.

In our case many peculiar features created dilemma in precise classification of the condition. A sudden onset of cutaneous lesions with rapid progression was clinically suggestive of tumour de emblee ME Enormous atypical cells with hyperconvoluted nuclei in peripheral blood provide sufficient evidence for diagnosing this as Sezary syndome. Also histopathology of the cutaneous lesions and the affected lymph nodes showing highly pleomorphic inflirtrate with deeper extension and less pronounced epidermotropism are relative criteria for SS. Immunohistochemistry has proved the cells to be CD 45 + T - helper cells. Pathological features in skin, lymph node and peripheral blood with minimal invasion of bone marrow, suggested that the origin of the lymphoma was in peripheral T - lymphocytes.

Though our patient did not satisfy all the clinical criteria for the nosological entity of Sezary syndrome especially erythroderma, the skin, lymph node and peripheral blood showed the classical features of the syndrome. Hence at least in a few cases with such features, absence of erythroderma may not be taken as a hard and fast criterion to rule out the diagnosis of Sezary syndrome.