Invented fairy tales in groups with onco-haematological children.

BACKGROUND: The impact of an onco-haematological illness for children is a traumatic event that opens to pain, hospitalizations and interrupts the continuity of daily life. It is difficult for the child to make meaning, to share the pain or ask a question related to the illness because, often, the parents or doctors cannot find a way to communicate in a suitable way for the child who remains in a situation of 'unspoken', where, fear, anxiety and pain cannot find a space to express. METHODS: The present research-intervention uses the methodology of invented fairy tales in groups with onco-haematological children, in the hospital, in order to explore the organization of the meanings at the base of the tales co-constructed by the participants underlying weaknesses and strengths of the invented fairy tales in groups intervention. The invented fairy tales in groups is used as a tool, such as a play, to express, share and support the experience of the illness of children. Forty-nine children participated to the invented fairy tales in groups in an onco-haematological hospital. Within a quali-quantitative framework we performed a thematic analysis of elementary context, cluster analysis, on the fairy tales considered as a unique narrative corpus of the thought of the group. RESULTS: The analysis shows four thematic clusters: fantasy as search for a meaning, 29.71%, the group as a space for illusions, 27.90%, the illness as a family problem, 25.72%, anchoring reality, 16.67%. The results highlighted three main carriers of sense: the representation of illness/the relational world/the representation of the institution. CONCLUSIONS: The use of invented-fairy-tales groups allowed the onco-haematological children to tell and share the experience of illness through a different way, which let them express symbolically their pain. The invented fairy tale in groups becomes a mediator of psychic processes which offer new solutions while improving interpersonal relationships/communication between the participants in group.

Improvement over the years of long-term survival in high-risk lymphoma patients treated with hematopoietic stem cell transplantation as consolidation or salvage therapy.

The role of hemopoietic stem cell transplantation (HSCT) is not well established in certain types of lymphoma, such as those with a high relapse risk or relapsing after initial therapy. New chemotherapeutic schemes and immunotherapy have improved survival of these patients. Nevertheless, there is not enough evidence regarding whether transplantation is the best therapeutic approach. Moreover, published data on long-term follow-up of high-risk lymphoma patients treated with HSCT are scarce. We analyzed 177 consecutive patients diagnosed with a high risk of relapse or with relapsed lymphoma who underwent HSCT after induction with standard chemotherapy in a tertiary academic center from 1989 to 2013. The median age was 40 years. Diagnoses were Hodgkin disease (n = 56), diffuse large B-cell lymphoma (n = 44), follicular lymphoma (n = 29), mantle cell lymphoma (n = 15), T-cell lymphoma (n = 18), and others (n = 15). Patients received either an autologous graft (n = 154) in first complete remission (1CR; n = 59) or more advanced stages (AS; n = 95), or an allogeneic graft (n = 23) in 1CR (n = 4) or AS (n = 19). In the autologous group, overall survival (OS) at 5 years was 57% and 75% in the periods 1989-2001 and 2002-2013, respectively (P = .05). Patients receiving an allogeneic graft presented an OS of 25% and 43% in the 2 periods. With a mean follow-up of 5 years (95% confidence interval 3.5-6.6), for patients receiving a transplant in 1CR, OS at 5 years was 80%, and for those receiving a transplant in AS it was 59% (P = .003). Nonrelapse mortality (NRM) at 5 years was 3.1% in the autologous group and 27.9% in the allogeneic group (P < .001). The main cause of NRM was infection (44%) in the whole cohort. All this leads to the conclusion that transplantation, as a therapeutic strategy, has shown a high long-term OS in this subgroup of patients with such a poor prognosis. OS improved over the years and reaching 1CR was a good prognostic feature. Infections were the main cause of NRM.

Primary systemic amyloidosis and high levels of Angiotensin-converting enzyme: two case reports.

Infiltrative heart diseases are caused by a heterogeneous group of disorders; amyloidosis and sarcoidosis are two frequent causes of myocardial infiltration, which differ in clinical and biological outcome and treatment issues. The presence of high levels of angiotensin-converting enzyme (ACE) in a patient with infiltrative heart disease may increase suspicion of sarcoidosis. Nevertheless, no mention about increased ACE levels in extracerebral primary systemic amyloidosis is available. We present two cases of primary systemic amyloidosis, which are cardiac involvement and elevated ACE levels.

Hemopoietic stem cell transplantation in childhood: reduction in mortality and improvement of survival over the years.

Stem cell transplantation (SCT) is an effective treatment for life-threatening hematologic and nonhematologic pediatric diseases. Reducing transplant-related mortality (TRM), a major complication of SCT, to improve long-term survival, therefore, is one of the main objectives of transplantation teams. We analyzed TRM and overall survival (OS) over the years in children undergoing SCT in our center. From June 1998 to October 2002, 156 consecutive children, 105 boys and 51 girls, median age 10 years (range, 2-18), with different diagnoses underwent SCT (100 autologous and 56 allogeneic). OS and TRM were analyzed in 2 different periods (June 1989-December 1998 and January 1999-October 2002) and grouped according to the different SCT modalities. The median follow-up was 18 months (range, 1-160). Autologous TRM showed a statistically significant improvement within 1999-2002 (0%) compared with 1989-1998 (12.2%) (P < .05). There were no statistical differences for allogeneic SCT. OS was 34% in the first period and 80.4% in the second period (P < .01), the improvement being for both autologous and allogeneic SCT. In our study, TRM decreased significantly for those children receiving autologous SCT in recent years. OS was significantly better in the latter period (1999-2002), both globally and for each SCT modality.

rHuEpo for the treatment of anemia in myelofibrosis with myeloid metaplasia. Experience in 6 patients and meta-analytical approach.

BACKGROUND AND OBJECTIVE: Experience with recombinant human erythropoietin (rHuEPO) in the treatment of the anemia secondary to myelofibrosis with myeloid metaplasia (MMM) is slight up to now. We present our results of the treatment of 6 patients and a review of the literature in search of possible parameters predicting response to this treatment. DESIGN AND METHODS: From January 1994 to June 1996 all transfusion-dependent patients with MMM diagnosed in our hospital were included in this study. We established a minimum period of 4 weeks of treatment and a maximum of 12 if no response was observed. Initial dosages used were 100 U/kg s.c. 3 times weekly, increasing by 50 U/kg every 4 weeks where no response was observed. Response was defined as a reduction > or = 30% of the previous transfusional needs. The review of the literature was made using a MEDLINE search (January 1990-December 1996) on the keywords erythropoietin, myelofibrosis, and agnogenic myeloid metaplasia. A statistical study was made in search of possible parameters to predict response. The parameters studied include age, sex, hemoglobin, serum erythropoietin (sEPO) levels, transfusional dependency, transfusional requirements per month prior to treatment, maximum dosages used and dosage at which response was obtained. RESULTS: Only 2 of our 6 patients responded, both at a dosage of 600 U/kg/week (200 U/kg 3 times weekly s.c.). In addition to our 6 patients we have found only 28 other patients in the literature. For statistical calculation 2 of our patients were not considered as they did not complete the period of study. The overall rate of response was 17/32 (53.1%). In the univariate analysis comparing responders and non-responders we found a tendency to significance with respect to sex (p = 0.07), sEPO (p = 0.07) and transfusional needs in units of packed red blood cells per month (PRBC/m) (p = 0.13). In this way patients with low sEPO, females and those with low transfusional needs (< 3 PRBC/m) respond better. This better response in females could be explained by the fact that their disease situation was more stable (with both lower sEPO levels and transfusional dependency). The best cut-off point in the sEPO to predict response was 123 mU/mL. No important side-effects have been observed except three cases of aggravation of splenomegaly. In two cases this condition improved when the rHuEPO was discontinued. The association of rHuEPO with hydroxyurea or interferon does not seem to affect the response. INTERPRETATION AND CONCLUSIONS: Though the number of patients is low, our data suggest that some MMM patients, in particular females and individuals with low sEPO levels and with low transfusional needs, might benefit from rHuEPO in terms of elevation of hemoglobin levels. Unfortunately, transfusion dependent-patients, i.e. those in whom a beneficial effect of rHuEPO would be most welcome, are unlikely to respond, and more generally, treatment is not cost effective in medically responsive patients.

Waldenström's macroglobulinemia complicated with acute myeloid leukemia. Report of a case and review of the literature.

The evolution of Waldenström's macroglobulinemia (WM) into chronic or acute myeloid leukemia (AML) is a rare event. Most of these cases have occurred after treatment with alkylating agents. We herein report a case of WM terminating in an acute myelomonocytic leukemia after treatment with prednimustine and chlorambucil and present a review of the literature.

[Acute myeloid leukemia in those over 70 years of age. Experience using low-dose ara-C treatment]. / Leucemia mieloide aguda en mayores de 70 años. Experiencia utilizando tratamiento con Ara-C a bajas dosis.

PURPOSE: The treatment of elderly patients with acute myeloid leukaemia (AML) remains controversial. We present the results of the treatment of a group of patients aged above 70 years with AML diagnosed in our Hospital since 1990. PATIENTS AND METHODS: We have studied retrospectively the cases of AML in patients older than 70 years diagnosed in our Service since January 1990 to June 1996. Induction treatment was performed, in all cases but one, with two cycles of Ara-C 10 mg/m2/12 h s.c. for 21 days and after haematological recuperation, if complete remission had been achieved, monthly maintenance treatment with Ara-C (25 mg/m2/12 h oral x 5 days), prednisone (40 mg/m2/day x 5 days) y vincristine (1 mg/m2 i.v. x 1 day) was begun. RESULTS: During the period of study 48 patients with AML have been diagnosed in our Service, among them 22 (45.8%) were older than 70 years. One of them could not be considered for the study as not all data from him could be compiled. Among the other 21 patients 5 presented previous haematological processes (4 myelodysplastic syndrome and 1 Waldenström's macroglobulinemia). Initial diagnosis according to FAB classification for AML was as follows: 7 M1, 6 M2, 4 M4, 2 M5 and 2 M6. From these 21 patients 2 received no treatment due to rapid progression and death, among the other 19, one was directly treated with a modification of the maintenance treatment with vincristine and prednisone without response (survival 2 months). The other 18 patients were treated with low-dose Ara-C (described above), among them 3 (16.7%) were not evaluable as they did not finish the first cycle of induction treatment; 8 (44.4%) showed no response; 2 (11.1%) achieved partial remission and 5 (27.8%) complete remission. One patient did not show any response after two cycles of low-dose Ara-C but she obtained complete remission when treated with Ara-C and idaurubicin. Overall mean survival was 5.7 months (median 2; 95% confidence interval 1.6-9.8 months). In the group of patients treated with low-dose Ara-C mean survival was 6.6 months (median 3.5; 95% confidence interval 1.9-11.2 months). CONCLUSION: We consider that the treatment with low-dose Ara-C is a valid option in the treatment of elderly patients (aged 70 or above) with AML because 28% complete remissions can be achieved, specially in those ones in which other more aggressive treatments are not possible.

[T-cell-rich B-cell lymphoma. Presentation of 3 cases]. / Linfoma B rico en células T. Presentación de tres casos.

T-cell-rich B-cell lymphoma (TBL) is a rare entity not included in the classical classifications of non-Hodgkin's lymphomas (NHL), presenting usually at diagnosis in advanced stages and with extranodal involvement. Its differential diagnosis, based on immunophenotyping technics, include other entities with different treatment and prognosis, mainly lymphocytic-predominance Hodgkin disease and peripheral T-cell lymphoma; this differential diagnosis has importance in patient's survival. We present 3 patients, two males (one of them with HIV infection) and one female, aged 38, 60 and 64 respectively, diagnosed as having TBL. The two former patients presented advanced stages at diagnosis. None of them was initially diagnosed as having TBL, the diagnosis were immunoblastic NHL in the first patient, peripheral T-cell lymphoma in the second and mixed cellularity Hodgkin's disease in the third one. The first patient was not treated due to rapid worsening and early death of septic shock; the other two cases were treated with COP-BLAM/IMVP-16 protocol achieving partial and complete remission respectively after 6 cycles of treatment. Comments about clinical, pathological and differential diagnostic aspects are made.

Sexual behaviour and multiple infections in drug abusers.

We have studied the prevalence and the serological profile of HBV, HCV, HDV and HIV infections in 137 Italian subjects addicted to the intravenous use of heroine and correlated the virological findings with sexual behaviour. HBV and HCV viremia were also measured in 114 patients. Anti-HCV was detected in 81% of the addicts, and one or more markers of HBV infection were detected in 62.8% (4.4% were carriers of HBsAg, 58.4% had evidence of past HBV infection and 13.1% of the latter also had HDV markers). Anti-HIV was positive in 23.4%; 26% of those positive for anti-HCV and 4.6% of those positive for HBV markers had no other viral marker: none had only anti-HIV. HBV-DNA was negative in the carriers of HBsAg, and HCV-RNA was not detected in any of the HBsAg carriers who also had circulating anti-HCV. Overall, 34% of the anti-HCV positive addicts had HCV-RNA in their blood. The prevalence of the virus infection correlated with the duration of drug addiction but not with sexual behaviour, and sexual behaviour did not influence the acquisition of any virus. HCV infection was most frequent and probably the first infection to occur, but exposure to HBV was also common despite a low rate of HBsAg carriage. The prevalence of HDV infection was high (50%) in the HBsAg carriers, while the overall prevalence of HIV was lower (23%) than expected. Lack of HBV-DNA and HCV-RNA in carriers of HBV with anti-HCV in serum may indicate that HBV and HCV mutually inhibit their own replication.

[Kikuchi-Fujimoto disease associated with acute infection by herpesvirus 6]. / Enfermedad de Kikuchi-Fujimoto asociada a infección aguda por virus herpes 6.

The histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease is a very rare entity in Spain. We present a 34-year-old arabic male admitted to hospital because one-month story of asthenia, anorexia, weight loss, fever and lymphadenopathies in all palpable sites. Analytic studies were all within normal limits except LDH levels and globular sedimentation rate, both raised. After cervical lymph node biopsy performance high grade Non-Hodgkin lymphoma was initially diagnosed. During admission he complained from pain in both shoulders and an erythematous desquamative eruption in trunk appeared. Some days later, a second lymph node biopsy was performed and Kikuchi-Fujimoto disease was diagnosed. Serologic tests for human herpes virus 6 were positive demonstrating active associated infection. He begun treatment with indomethicin, fever and general symptoms disappeared one week later discontinuing treatment. Two months after discharge, all lymphadenopathies had disappeared. A review on epidemiological, clinical, pathological and differential diagnosis issues is made.

[Primary testicular lymphoma. Report of two cases]. / Linfoma testicular primario. Presentación de dos casos.

Primary testicular lymphoma (PTL) is the most frequently diagnosed testicular tumor in men over 60 years, in spite of this circumstance it is a rare process. Two cases of PTL are reported, the first one in a child and the second in an adult. Both cases were intermediate grade lymphoma and had low stage (IEA), presenting initially as an enlargement of the testicle as the only symptom, the second case presented involvement by contiguity of the abdominal muscles. Immunohistological markers showed T nature in the first case, and B in the second. The treatment applied in both cases was orchiectomy and systemic chemotherapy using COP-BLAM/IMVP-16, in the child prophylaxis of the central nervous system using methotrexate was made. Response to treatment was good, the first patient achieved complete remission and his survival at present is 24 months, the second patient died 5 month after diagnosis due to stroke without having completed chemotherapy but with an important reduction of the tumoral mass. A review of the literature on clinical, diagnostic and therapeutic issues is made.

[Human recombinant erythropoietin in the treatment of myelodysplastic syndromes anemia. Meta-analytic study]. / Eritropoyetina recombinante humana en el tratamiento de la anemia de los síndromes mielodisplásicos. Estudio metaanalítico.

PURPOSE: To study the usefulness of different published epidemiological and analytic parameters to decide the treatment with human recombinant erythropoietin (rHuEPO) of anaemic patients with myelodysplastic syndromes (MDS). PATIENTS AND METHODS: We have revised 10 published series compiling 115 patients, studying age, sex, initial diagnosis, route of administration and posology, criteria of response, duration of the study, dosis with the response was obtained, response according to initial diagnosis, duration of responses, and effect of the treatment on other hematopoietic series. We have made a comparison between responders and non-responders based on epidemiological and analytical parameters. RESULTS: We have compiled 115 patients with a rate of global response of 23.5%. We have not found significative differences between the route (s.c. or i.v.) or frequency of administration, however the number of responses was higher when rHuEPO was administered three times weekly. A great variability in the criteria of response was observed among the different studies. Most of studies have a duration of three months but we have observed significative differences in the number of responses when the study is longer. We have not found significative differences between responders and non-responders with respect to age, sex, used dosis, transfusional dependency and degree of transfusional dependency, basal serum erythropoietin, time since diagnosis, transfusional period, haemoglobin level among non-transfusion dependent patients and haemoglobin level among transfusion dependent patients. We have found significative differences with respect to initial diagnosis, a higher rate of responses was observed in the refractory anaemia with excess of blasts (RAEB) group. We have not found a higher rate of transformations into acute myeloid leukaemia (AML) among these patients. The effects of the treatment on other haematopoietic series can be considered as anecdotical. CONCLUSION: The different epidemiological and analytic parameters published up to now are not useful in the decision of including an anaemic patient with MDS in the treatment with rHuEPO. Those patients with RAEB can be benefited with the treatment with rHuEPO. The concomitant use of other cytokines could improve these results.

[Chronic neutrophilic leukemia associated with myeloma. Simultaneous presentation]. / Leucemia neutrofílica crónica asociada a mieloma. Presentación simultánea.

A case of chronic neutrophilic leukaemia associated with multiple myeloma is reported. The patient had a 6 months history of bruising and weight loss, and showed mature neutrophilic leukocytosis, hepatosplenomegaly, high neutrophil alkaline phosphatase score, hyperuricaemia, neutrophils with pseudotoxic granulation and scarce Döhle bodies; moreover, a monoclonal IgG lambda was detected amounting 57.3 g/L. The bone marrow was grossly hypercellular with marked myeloid hyperplasia and aggregates of immature plasma cells. After treatment during 1 year with melphalan and prednisone she is well, although persisting with neutrophilic leukocytosis, slight splenomegaly, and the monoclonal IgG decreased to 25.8 g/L.

[Retinoblastoma with suprasellar extension: report of a case]. / Retinoblastoma de extensão supra-selar: registro de um caso.

The authors discuss a case of a two-years-old girl, who had a unilateral previous enucleated retinoblastoma, that died with a suprasellar mass and distant metastasis.