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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
Background: The clinicopathologic characteristics of malignant lymphomas may vary according to geography. We previously described Adult T -cell leukaemia/lymphoma (ATLL) cases associated with human T-cell lymphotropic virus type-I (HTLV-I) in their different clinical presentation: acute, lymphomatous, chronic and smoldering and the recently primary cutaneous subtype in Peru (EHA2001: abstract 129). The aim of this study is to determine the relative frequency of cutaneous lymphomas and evaluate the clinical relevance of the new WHO/EORTC classification in a General Hospital in Lima-Peru Methods: We conducted a clinicopathologic retrospective study of primary cutaneous lymphomas diagnosed from 1997 to 2004 in our General Hospital. Clinical records, haematoxylin & eosin-stained slides and immunohistochemical stains from 78 patients were reviewed. HTLV-1 serology was made using ELISA and Western Blot method. The statistical method was descriptive and survival was calculated using the Kaplan-Meier method. Results: The mean age at time of presentation was 62 years and the female/male ratio 1,5:1. T-cell lymphomas were 88.6% and 11.4% were B-cell lymphomas. Eight-six percent (67/78) were primary cutaneous lymphomas and fourteen percent (11/78) were secondary cutaneous lymphomas. The most frequent primary cutaneous lymphomas was mycosis fungoides (MF): 44.7% (30/67); cutaneous / smoldering ATLL sutypes included 13/67 (19.4%) patients; unspecified peripheral T-cell lymphoma 4/67 (6%), lymphomatoid papulosis 2/67 (3%), leg-type diffuse large B-cell lymphoma 2/67 (3%), diffuse large B-cell lymphoma 2/67 (3%), subcutaneous panniculitis-like T-cell lymphoma 2/67 (3%), one case of the following lymphomas: anaplastic large cell, Sézary syndrome, nasal type extranodal NK/T-cell lymphoma, marginal zone B-cell lymphoma, follicle center lymphoma and intravascular lymphoma; finally unclassifiable lymphomas 5/67 (7.4%). Most frequent secondary cutaneous lymphomas were acute and lymphomatous subtypes of ATLL with 72% of the cases. Five-years overall survival for MF was 77%. The 5-years overall survival for primary cutaneous ATLL lymphomas was 18% and 0% for the secondary cutaneous ATLL group. Conclusions: In this retrospective analysis, both ATLL and MF are the most frequent cutaneous lymphomas in our General Hospital. ATLL has a poor overall survival.
Background: fludarabine (F) is licensed for the management of indolent non Hodgkin’s lymphoma in countries such as Canada and Switzerland. Clinical evidence suggests that fludarabine monotherapy is as least as effective, than conventional therapies such as cyclophosphamide, vincristine, prednisone (CVP) for the first and second line treatment of B-cell low grade NHL achieving objective response rates. Better response rates can be achieved combining F with Mitoxantrone (M) in low grade NHL even in refractory relapsed (RR) patients (pts). The Latin American Cooperative Oncology Hematology Group (LACOHG) proposed a multicenter study in Latin American countries in 2002 to use FM in RR B-cell low grade NHL. Aims: to assess the response rate, safety, disease free survival (DFS) and overall survival (OS) of FM in RR B-cell low grade NHL during (2003–2006). Methods: Forty-eight patients in the period of January 2003 to February 2006 were evaluated. Forty-four pts. had follicular lymphoma and 4 small lymphocytic lymphoma. Median age 63.5 years old (range: 24–83). Gender: female 56% and male 44%. Inclusion criteria for low grade NHL-LG was: any previous treatment excluding autologous transplantation, Ann Arbor stage II to IV, age > 18 years old, ECOG performance status 0–2 and written informed consent. ECOG performance status 0: 2%, 1: 71% and 2: 27%. Ann Arbor staging: II: 2%, III: 29% and IV: 69%. International Prognostic Index (IPI): 0–1: 19%, 2–3: 71% and 4–5: 10%. Median previous treatment was 1 (range: 1–3). FM treatment consisted of F 25 mg/m2 i.v. (day 1–3) and M 10 mg/m m2 i.v. (day 1) each 28 days for 6–8 cycles. Results: on this low grade NHL cohort the overall response rate (ORR was 81%; progressive disease and non-response 19%. With a median follow up of 17 months, OS at 24 months was 86% (DE 5.2%) and DFS at 24 months 57.1% (DE 11.3%). LDH in serum was not an adverse prognostic factor for DFS and OS. Safety: on the 286 cycles in 48 pts, the toxicity was: 18 episodes of grade 3-4 neutropenias, 15 episodes of grade 3-4 thrombocytopenia, 7 episodes of grade 1–2 nausea/vomiting, grade 1–2 diarrhea in 4 pts, 8 pts were admitted to the clinic, 11 fever episodes, 2 allopecia, 4 pts developed grade 1–2 peripheral neuropathy and infections 7%: one case herpes zoster. Mortality rate: 12,5% (6/48 patients), 5 of them because progressive disease. No cardiac toxicity was reported. Conclusions: FM is an effective and safe treatment for RR low grade NHL. A longer follow up and a larger trial, might be needed to confirm these results in a multicenter, randomized study. DFS with FM in RR low grade NHL : LACOHG DFS with FM in RR low grade NHL : LACOHG
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