Adult T -cell leukaemia/lymphoma (ATL) is an aggressive disease associated with human T-cell lymphotropic virus type-I (HTLV-I) with heterogeneous clinical presentation and outcomes, described in Southern Japan, Europe, Caribbean and previously on Pacific coast of South America including Peru (EHA 2001, abst. 304). Shimoyama’s ATL classification (BHJ1991:79) includes four types: acute, lymphomatous, chronic and smoldering; recently a new clinical type cutaneous had been described (BJD2005:152). Herein we show our experience in ATL in our institution between October 1997 and May 2005 All our 55 cases shown positivity to HTLV-I Western Blot test; immune-histopathology, blood smears and flow cytometry showed mature T-cell lymphocyte. Median age at diagnosis 61 years old (range 23–84), female/male ratio: 1.2. Thirty-one (56%) patients had ECOG status performance at diagnosis ≥ 2. Clinical types: acute (n=26), lymphomatous (n=22), smouldering (n=2), cutaneous (n=5) with no chronic type observed. Median haemoglobin diagnosis was 12.1 g/dl (range 6.9–17), median albumin was 3.2 g/dl (range 1.8 – 4.9), median beta-2 microglobulin was 4.6 g/dl (range 1.5 – 16.9), median globulin was 2.7 g/dl (range 1.5 – 8.2) and DHL was 796 UI/ml (range 331 – 13000). Twenty-five per cent (9/36) debuted with hypercalemia (acute type=7, lymphomatous=2). Acute ATL showed median leukocytes of 48,900 cell/mm3 (range 6830–259,000). IPI risk score was: low (n=6), low intermediate (n=7), high intermediate (n=16) and high (n=27). Treatment for acute ATL was based on acute leukaemia and lymphoma regimens without any response but one, interestingly this patient was treated with Fludarabine 25 mg/mt2 day 1–5 each 28 day for 6 cycles and got complete remission. Twenty-one over 24 lymphomatous ATL type were evaluable for treatment response: overall response 38% (9/24) with 26% complete response. Smouldering and cutaneous ATL types received mainly topic treatments. Stratify analysis for IPI, DHL, beta-2 microglobulin, globulin and albumin for acute and non-acute ATL did not show any statistic difference. Median overall survival was: acute type (2.0 months DE 0.2), lymphomatous type (10.2 months DE 3.6), smouldering type (17.2 months) and cutaneous type (36.2 months DE 19.5) (Log Rank 30,76 p=0.0) ATL persist is a poor prognostic peripheral T-lymphocytic malignancy with bad clinical and therapeutically outcomes mainly in the acute type. Cutaneous type seems to be less aggressive. Figure Figure

HCL is a chronic leukemia of B-lymphocyte with variable clinical course: symptomatic cytopenia and/or marked splenomegaly. Concomitant infections disease are often and transfusion are quite necessary. Current therapy includes interferon, 2′deoxycoformycin, 2′chlorodeoxyadenosine which induce profound and durable CD4 and CD8 T cells cytopenia inducing major infection risk. Fludarabine phosphate (F-AMP) had been extensively used in chronic B-cell leukemia and lymphomas; previous reports have shown response in pre-treated HCL patients (Kraut, 1991; Kantarjian, 1991). Herein we show our experience with 3 high risk-HCL patients treated with F-AMP in our institution between May 2001 and August 2002. Pt 1: male 52 yo, debuted with splenomegaly (200 mm) and pancytopenia in 12/2000. Massive HCL involment in bone marrow (BM), flow showed positivity to CD20, CD 11c, CD 25 and CD103; TRAP test positive. At that point patient was at first splenectomized, with transient moderate blood count recovery. Five months later patient showed severe cytopenia and was started on F-AMP 25mg/m2 i.v. d1-5 each 28 days 6 cycles. Patient got complete haematological response with residual infiltration for HCL in BM; with a follow up of 51 months after F-AMP without cytopenias and infections. Pt 2: male, 53 yo, debuted with splenomegaly (over 200 mm), pancytopenia and diffuse B-cell BM involvement in 03/2002. Lucid spleen spots were observed at CT scan, followed by fever and haemolytic anemia. Splenectomy was performed, final diagnosis included extra-pulmonary TBC for M. Tuberculosis and HCL. Flow showed: CD20 (+), CD11c (+), CD 25(+), CD103 (+), CD5 (−), CD10 (−); TRAP test positive. TBC treatment started and mild blood count recovery was observed, 10 months after splenectomy; villous lymphocytes appeared in peripheral blood. Patient was started on F-AMP 25mg/m2 i.v. d1-5 each 28 days 6 cycles. Patient got Complete Response (CR) with a follow up of 40 months without leukaemia or infection recurrence. Pt 3: male 63 yo, with previous medullar thyroid carcinoma diagnosis three years ago. He debuted with splenomegaly (200 mm), mild cytopenia and serum creatinine 1.26 mg/dl by 01/2002. Typical HCL involvement in BM, flow showed positivity to CD20, CD 11c, CD 25 and CD103. Patient was started on F-AMP 25mg/m2 i.v. d1-5, one cycle (08/2002). Ten weeks later, previous to restart treatment a BM assessment showed CR with creatinine clearance range between 30–40 ml/min, therapy was stopped. Actually with a follow up of 36 months patient is without recurrence. Our experience strongly suggests and confirms potential role of F-AMP, in the management of high risk HCL previously splenectomized or post infection disease. Interestingly one patient responded to a total dose of 125mg/m2. Base in our and others data, F-AMP should be considered for HCL treatment. Table: Blood counts at diagnosis and 6 months post treatment Neutrophils Platelets Hb mpt = months post treatment Pt 1 Dx 225 22 K 6.5 6 mpt 4450 213 K 16.2 Pt 2 Dx 686 57 K 8.3 6 mpt 2750 251 K 16.2 Pt 3 Dx 1330 331 K 11.6 6 mpt 3400 176 K 12.4