Response to splenectomy is durable after a certain point in time in adult patients with chronic immune thrombocytopenic purpura

Splenectomy may lead to a good response in 60-80% of adult patients with corticosteroid refractory idiopathic thrombocytopenic purpura (ITP) but, the long-term response to splenectomy still remains less well defined. We assessed the long-term efficacy and safety of splenectomy in adult patients with chronic ITP. A cohort of 59 splenectomised ITP patients (M/F = 25/34; median age 39 yr; range 14-75) were followed up for a median of 18 yr (range 2-32). No life-threatening surgical complications were observed. The overall response rate was 78% with 59% complete remission (CR) and 19% partial remission (PR). CR and PR patients were younger than non-responding patients at time of diagnosis (median age: 36 yr vs 48 yr, P = 0.03) and at splenectomy (median age: 38 yr vs 51 yr, P = 0.02). Among the 46 responding patients, eventually 17 had relapse. No disease progression occurred after 12.1 and 7.3 yr for patients in CR or PR, respectively. One case of fatal septicaemia was recorded. We conclude that splenectomy is an effective and safe treatment in adult patients with chronic ITP failing to respond to corticosteroid treatment and importantly, our findings support the view that response to splenectomy is durable after a certain point in time

Response to Splenectomy Is Durable after a Certain Point in Time in Adult ITP Patients

Abstract Background. Splenectomy is the recommended treatment in adult patients with immune thrombocytopenic purpura (ITP) who fail to respond to corticosteroid treatment or who require unacceptably high doses to maintain a safe platelet level. For these patients splenectomy may produce a good response in 60–80% of patients. However, the observation time after splenectomy has in most published studies been relatively short. Thus, the true long-term response to splenectomy still remains less well defined. The efficacy and safety of splenectomy were assessed in a cohort of adult ITP patients followed for a very long period of time. Patients and methods. The study included 59 ITP patients (M/F=25/34, median age at splenectomy; 39 yrs, range 14–75 yrs). The median time from diagnosis to splenectomy was 5.5 mos (range 0.4–199 mos). Criteria used to define response to splenectomy were: CR; platelet count >150 x 109/l lasting ≥4 weeks, PR; platelet count ≥50–150 x 109/l lasting ≥4 weeks and, NR; failure to achieve platelet counts ≥50 x 109/l. The criterion for relapse in CR/PR patients was a decrease in platelet counts to <50 x 109/l. Results. The median follow-up time after splenectomy was 18 yrs (range 2–32 yrs). No serious surgical complications occurred. The overall response rate was 78% with 59% CR and 19% PR. CR and PR patients were younger than NR patients at time of diagnosis (median age: 36 yrs vs 48 yrs, p=0.03) and at splenectomy (median age: 38 yrs vs 51 yrs, p=0.02). Among the 46 responding patients 17 eventually relapsed. A plateau in the progression free survival curve was identified after 12.1 and 7.3 years for patients in CR or PR, respectively. Figure Figure At follow-up, 5 of 17 patients relapsing after splenectomy were in CR. Bacteremia was recorded in two patients during a total of 1023 patient years at risk. Conclusions We conclude that splenctomy is an effective and safe treatment in adult patients with chronic ITP failing to respond to corticosteroid treatment. The complication rate was low and our findings support the view that response to splenectomy is durable after a certain point in time

A prospective comparison of fine-needle aspiration cytology and histopathology in the diagnosis and classification of lymphomas

INTRODUCTIONSurgical biopsy examination is the gold standard for lymphoma diagnostics. However, fine-needle aspiration cytology (FNAC) offers several advantages in that it is quick, inexpensive, and the aspiration procedure has very few complications. This prospective study compares the diagnostic outcome between FNAC and surgical biopsy.MATERIALS AND METHODSA total of 103 patients (>15 years) with lymphadenopathy and accessible lymph nodes underwent both diagnostic procedures. Immunophenotyping was performed on both FNAC and histopathological specimens. The updated KIEL classification was used for primary diagnosis and the WHO classification for reclassification.RESULTSFNAC- and histopathology-based diagnoses were concordant in 76 patients. In 10 patients, there was a major diagnostic discordance: four differed with regard to degree of malignancy (low- versus high-grade NHL), three lymphoma versus reactive changes, and three regarding Hodgkin's lymphoma versus anaplastic large cell lymphoma. In 10 patients there was some (minor) discordance regarding subclassification: in eight patients the results of immunophenotyping differed, in two cases there were discrepancies in the cell type classification. In the remaining seven cases, there were diagnostic difficulties due to an insufficient sample. two serious adverse events occurred following surgical biopsy.CONCLUSIONSFNAC is an accurate method in the diagnosis of lymphomas when the cytologic diagnosis is corroborated by immunophenotyping. However, an increasing use of FNAC for primary diagnosis and classification of lymphomas may result in a loss of archival tissue for complementary analyses, reclassification, and research purposes. In addition, some of the lymphoma entities are impossible to diagnose with use of the FNAC technique

Successful mobilization of Ph-negative blood stem cells with intensive chemotherapy plus G-CSF in patients with chronic myelogenous leukemia in first chronic phase

The aim of the study was to investigate the feasibility of mobilizing Philadelphia chromosome negative (Ph-) blood stem cells (BSC) with intensive chemotherapy and lenograstim (G-CSF) in patients with CML in first chronic phase (CP1). During 1994-1999 12 centers included 37 patients 556 years. All patients received 6 months' IFN, stopping at median 36 (1-290) days prior to the mobilization chemotherapy. All received one cycle of daunorubicin 50 mg/m(2) and 1 hour infusion on days 1-3, and cytarabine (ara-C) 200 mg/m(2) 24 hours' i.v. infusion on days 1-7 (DA) followed by G-CSF 526 mu g s.c. once daily from day 8 after the start of chemotherapy. Leukaphereses were initiated when the number of CD 34(+) cells was > 5/ml blood. Patients mobilizing poorly could receive a 4-day cycle of chemotherapy with mitoxantrone 12 mg/m(2)/day and 1 hour i.v infusion, etoposide 100 mg/m(2)/day and 1 hour i.v. infusion and ara-C 1g/m(2)/twice a day with 2 hours' i.v infusion (MEA) or a second DA, followed by G-CSF 526 mg s.c once daily from day 8 after the start of chemotherapy. Twenty-seven patients received one cycle of chemotherapy and G-CSF, whereas 10 were mobilized twice. Twenty-three patients (62%) were successfully (MNC > 3.5 x 10(8)/kg, CFU-GM > 1.0 x 10(4)/kg, CD34(+) cells > 2.0 x 10(6)/kg and no Ph+ cells in the apheresis product) [n=16] or partially successfully ( as defined above but 1-34% Ph+ cells in the apheresis product) [n=7] mobilized. There was no mortality during the mobilization procedure. Twenty-one/23 patients subsequently underwent auto-SCT. The time with PMN < 0.5 x 10(9)/l was 10 (range 7-49) and with platelets 520610 9/1 was also 10 (2-173) days. There was no transplant related mortality. The estimated 5-year overall survival after auto-SCT was 68% (95% CI 47-90%), with a median follow-up time of 5.2 years. We conclude that in a significant proportion of patients with CML in CP 1, intensive chemotherapy combined with G-CSF mobilizes Ph-BSC sufficient for use in auto-SCT