Summary:superior to G-CSF alone based on mean CD34 ؉ cell yield per pheresis, adequate CD34 ؉ cell collections can be achieved with G-CSF alone in most MM patients The best method for peripheral blood progenitor cell (PBPC) mobilization in patients with multiple myeloma with less toxicity and with simplification of the procedure. (MM) remains controversial. We report the results of two different methods of PBPC collection for autologous Keywords: multiple myeloma; PBPC mobilization; GM-CSF; G-CSF transplantation in 40 patients with stage II or III MM. In group I (n = 18), HD-CY, 4 g/m 2 i.v., was administered followed by GM-CSF, 8 g/kg/day s.c., until the end of collection, starting the leukaphereses after hemaPeripheral blood progenitor cells (PBPC) are increasingly tological recovery (Ͼ1 × 10 9 /l WBC). In group II used for hemopoietic autologous rescue afer high-dose ther-(n = 22), G-CSF, 10 g/kg/day s.c., was used alone until apy (HDT) in multiple myeloma (MM). 1-7 The use of perthe last day of collection, starting consecutive aphereses ipheral blood as the source of hematopoietic stem cells has on the 5th day. A minimum of two aphereses were perreplaced the bone marrow due to the rapidity of the formed to collect at least 2 × 10 6 /kg CD34 + cells. Both engraftment and the difficulties in harvesting bone marrow patient groups were comparable for age, sex and clinical cells in most MM patients due to osteoporosis or to preprognostic features as well as previous therapies. In vious irradiation. Another potential advantage for the use group I, the median yields per pheresis were: MNC 1. 47 of PBPC is the lower percentage of tumor cell contami-(1.38-2.32) × 10 8 /kg, CFU-GM 0.82 (0.18-13.2) × 10 4 /kg nation when compared with bone marrow, as recently conand CD34 + cells 1.98 (0.96-6.96) × 10 6 /kg. In group II firmed by molecular methods, 8 although this point is conthese results were: MNC 2.44 (2.06-3.6 × 10 8 /kg) troversial because several recent reports have suggested that (P = 0.03), CFU-GM 0.75 (0.16-7.8) × 10 4 /kg and CD34 + plasma cells may be recruited into the peripheral blood after 1.05 (0.32-3.4) × 10 6 /kg (P = 0.02). The median number mobilizing therapy. 9,10 HDT followed by autologous PBPC of aphereses performed in each group was 5 (4-12) with in MM may induce about 40-60% of complete responses a median of 5.24 ± 2.51 in group I and 3 (2-6) with a in previously untreated patients or in first partial response median of 3.1 (±0.91) in group II (P = NS). Hospitalizafter current induction chemotherapy 1-7,11-13 and some ation for PBPC mobilization was required in all patients investigators have found an advantage regarding response in group I and the treatment-related toxicity was rate, duration of response and progression-free survival greater in this group: 12 patients (66%) developed fever when compared with conventional treatment. 11,12 requiring antibiotics during the neutropenic periodIn spite of the expansion of this strategy, the optimum after HD-CY and six (33%) patients required transsche...
Nowadays, the chemoradiotherapeutic protocols for Hodgkin's disease (HD) achieve high curability rates. Hemato-oncologists focus on both avoiding medical and psychological sequelae of the treatment and returning patients to a normal life. The quality of life and psychological well-being of Spanish patients who are long-term survivors of HD were studied and compared to the results obtained from healthy controls. Questionnaires on quality of life [European Organization for Research and Treatment of Cancer (EORTC) QLQ30] and psychological status [hospital anxiety and depression (HAD) scale] were mailed to HD patients without active disease and free of second malignancies and were also given to healthy controls. Of 67 selected patients (68.6%), 46 were included in this study. The median follow-up for these 46 patients was of 7.6 years (0.8-22.1) after being diagnosed. Although there were no differences between patients and controls with regard to their global state of health and quality of life (72.9€22.7 vs 79.3€18.7; p=0.22), patients presented a lower physical function (88.2€18.1 vs 96.5€9.7; p=0.05) and a worse social operation scale (81.5€25.4 vs 96.3€13.1; p= 0.0015) together with higher symptoms of dyspnea (8.6€14.7 vs 0€0; p=0.03) and higher economic difficulties (23.1€38.3 vs 0.7€4.9; p=0.017) when compared with healthy controls. However, we did not find differences in the scores and the proportion of cases of anxiety and depression between the two groups. The quality of life questionnaire disclosed differences between patients and controls in some functional and symptomatic scales. These differences can be read as a consequence of either the disease itself or the treatment received. However, the results of this controlled pilot study should be confirmed in a larger series of Spanish HD survivors. In the future, these results could be a reference when new therapeutic protocols are designed to reduce the impact on the quality of life of the patients. Socioeconomic support to the patients should also be provided in order to improve their medical care.
SummaryWe present the case of a full-term newborn in whom purpura fulminans developed shortly after birth. A diagnosis of homozygous protein C deficiency was established based upon undetectable plasma protein C activity and antigenemia in the newborn infant, and was later confirmed by protein C gene analysis. Specific replacement therapy with intravenous protein C concentrate was started 9 days after birth. This rapidly led to the complete regression of cutaneous lesions and consumption coagulopathy. After stabilization, oral anticoagulation was initiated in association with prophylactic treatment with intravenous protein C concentrate. However, oral anticoagulation was finally abandoned as the patient presented several thrombotic and hemorrhagic episodes clearly related to difficulties with anticoagulation. Due to the hazards related to prolonged venous access, we are currently using subcutaneous infusion of protein C concentrate for the longterm management of this condition, with satisfactory results.
Clinical outcome of 47 consecutive patients with advanced HD who underwent ASCT in our Department was analyzed retrospectively. Median age was 28 years (28 males and 19 females). At transplant, 15 (32%) patients were in CR (five in first CR after two chemotherapy regimens and 10 in second CR), eight (17%) in PR (seven without a prior CR), 22 (51%) had relapsing disease (19 with sensitive relapse) and two had primary refractory disease. The CVB regimen with two different schedules was used: 22 (47%) patients received standard CBV (CY 6 g/m2, BCNU 300 mg/m2 and etoposide 600 mg/m2) and 25 (53%) received an increased CBV dose (CY 7.2 g/m2, BCNU 440 mg/m2 and etoposide 2 g/m2). Antitumor response for 28 evaluable patients was similar for both CBV regimens: 87 and 75% (P=0.39). At 7.2 years, actuarial overall survival (OS), progression-free survival (PFS) and event-free survival (EFS) for the whole series were 51.7+/-8%, 34+/-9% and 28+/-8%, with a median follow-up for the surviving patients of 3 years (0.7-7.6). No differences in these survival functions according to the CBV regimen used were observed (P=0.57). A history of a prior CR (P=0.003), duration of first CR >1 year (P=0.04), absence of bulky nodal disease at transplant (P=0.054), absence of extranodal disease at transplant (P=0.01), and a CR status at transplant (P=0.0006) were associated with a better PFS on univariant analysis. On multivariate analysis, only CR status at transplant remained significant (P=0.05). When patients in second CR at transplant and those in first sensitive relapse were analyzed separately, no differences in clinical characteristics or in treatment received pretransplant were observed; however, PFS was significantly different (P=0.01). In conclusion, CR status at transplant is useful in identifying 'good risk' patients and is necessary to obtain the greatest benefit from ASCT independent of the CBV regimen used.
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