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An 86-year-old woman was admitted to hospital in March 2000 as a result of fatigue and typical Raynaud’s phenomenon of the hands and feet lasting for 4 weeks, and with some necrotic lesions of recent onset on the feet. She had no medical history and denied ingestion of any medication. She had lost 4 kg in weight during the month prior to admission and appeared fatigued. Physical examination was otherwise unremarkable. The white blood cell count was 6.6610/l leucocytes with 69% neutrophils and 16% lymphocytes; her haemoglobin level was 10.8 g/dl with mean corpuscular volume 97 fl (normal 84 – 98) and 15610 reticulocytes; her platelet count was 225610/l. The erythrocyte sedimentation rate was 56 mm/h. Serum liver tests and renal function were normal. Serum protein electrophoresis showed an albumin level of 32.6 g/l (36 – 55) and a polyclonal hypergammaglobulinaemia of 15.6 g/l (6.7 – 15.2). Serum lactate dehydrogenase was 253 IU/l (normalv280). Antinuclear antibodies were detected at 1/640 with a homogeneous pattern; the search for anti-doublestranded DNA, anti-extractable nuclear antigen and antiphospholipid antibodies was negative; serum C3 component of complement was 0.68 g/l (0.75 – 1.57); the C4 component was 0.18 g/l (0.24 – 0.46). No cryofibrinogenaemia or cryoglobulinaemia were detected. Direct Coombs’ test was positive for IgG and C3d. A search for cold agglutinins at a temperature of 4‡C was highly positive at a titre of 1/1024 with anti-I specificity. Coagulation studies showed normal fibrinogen level, prothrombin time. A search for lupus anticoagulant was negative, circulating anticoagulant plasma activity of protein C, protein S, antithrombin III and heparin cofactor II, and platelet aggregation tests were normal. Serologies for HIV, HCV andHBV, HTLV, Epstein-Barr virus, cytomegalovirus, syphilis and mycoplasma were negative. Electrocardiogram (24 h) monitoring and transoesophageal echocardiography were normal. Doppler examination demonstrated distal narrowing of distal arteries. Histological examination of skin specimens from the necrotic lesions of legs showed small vessel thrombosis with eosinophilic thrombi of fibrin and erythrocytes but no vasculitis, and areas of ischaemic necrosis within the dermis and the subcutaneous tissue. Computed tomography scan of abdomen and thorax was normal. Bone marrow biopsy disclosed a massive infiltration by atypical small-sized lymphoid cells. Immunohistochemical studies showed that the neoplastic lymphocytes expressed CD3 and CD4 but did not express CD19, k and l light chain consistent with the diagnosis of T-cell lymphoma. No analyses of T-cell receptor gene rearrangement were performed. Progressively, the hands and feet became cyanotic and cool and turned to dry gangrene (Fig. 1) despite intravenous pulses of methylprednisolone and cyclophosphamide, then plasmapheresis and administration of etoposide. The patient died from septic shock one month after admission. No autopsy was allowed by the family.

Raynaud's Phenomenon with Necrosis of the Extremities Induced by Cold Agglutinin Disease Secondary to a T-cell Lymphoma