Autoimmune Hemolytic Anemia and Immune Thrombocytopenia as Unusual Presentations of Childhood Hodgkin Lymphoma: A Case Report and Review of the Literature

We discuss an unusual clinical presentation of childhood Hodgkin lymphoma; a 16-year-old girl was referred for Coombs-positive severe anemia, thrombocytopenia, and asymptomatic anterior mediastinal mass. Bone marrow examination showed no evidence of neoplastic disease. Biopsy of the mass was possible only after administration of both intravenous immunoglobulins and steroids resulting in a prompt rise of the platelet count and partial hemoglobin level stabilization. The identification of this clinical picture as a possible complication of an underlying Hodgkin lymphoma presents difficulties in diagnosis and management of the primary condition

Linfomi non- Hodgkin AIDS-relati (LNH-AIDS) e confezione da virus dell’epatite C (HCV)

Con lo scopo di valutare l'impatto dell'HCV sul decorso clinico dei LNH-AIDS e sulla evoluzione, in tale contesto, della malattia epatica stessa, tra il marzo 1989 e il maggio 2003, 92 casi di LNH-AIDS (22 femmine e 70 maschi, età mediana 39 anni) sono stati testati per HCV-ELISA e monitorizzati per un periodo medio di 31,8 mesi (min 1 max 168 mesi). Risultati: Una coinfezione da HCV era presente in 39/92 (42%) casi. I LNH-AIDS HCV coinfetti, rispetto a quelli non coinfetti presentavano: a) una età mediana più bassa (36 vs 41 anni, p 0,0081); b) una storia di infezione da HIV più lunga (57 vs 36 mesi, p 0,0247); c) una stretta associazione con lo stato di tossicodipendenza endovena (TE: 32/39 vs 0/53 casi, p 0,0000); d) una più elevata frequenza di alterazioni delle aminotrasferasi sia all'esordio di linfoma (26/39 vs 19/53 casi, p 0,0067) che durante il decorso clinico (23/33 vs 20/47, p 0,0300) con evoluzioni in cirrosi (9/39 vs 0/53, p 0,0002), decessi per causa epatica (4/39 vs 0/53, p 0,0392) e una riduzione alla fine del decorso clinico, rispetto ai valori basali, del valore medio dei linfociti CD4/µL (135 vs 48, p 0,0012 e 112 vs 134, p 0,05.) statisticamente significativi. Nessuna differenza è stata osservata, tra i due gruppi di pazienti, per quanto riguarda: a) la presentazione clinica e biologica del linfoma e dell'infezione da HIV; b ) il tipo di trattamento e di risposta sia alla terapia antineoplastica che antiretrovirale; c) le sopravvivenze libera da linfoma, da eventi negativi e globale calcolate a 5 anni (p 0,05). Il genotipo 3A è stato osservato nel 71% dei casi testati ed era associato alla TE. Conclusioni: I soggetti con LNH-AIDS HCV coinfetti, anche se a discapito di una aumentata tossicità epatica, hanno le stesse possibilità di cura e di lunghe sopravvivenze di quelli non coinfett

Treatment of Children With B-Cell Non-Hodgkin Lymphoma in a Low-Income Country

Background. An adapted LMB 96 derived protocol for B-cell non-Hodgkin lymphoma (NHL) was implemented at the pediatric oncology unit of the Children Welfare Teaching Hospital in Baghdad (Iraq) from 2000 to present. The purpose was to evaluate the feasibility and efficacy of this intensive therapeutic regimen in a limited resource country. Methods. Patients <15 years of age with high grade B-cell NHL were included. A modified LMB 96 regimen was employed with a reduction of cyclophosphamide and methotrexate dosages due to inadequate laboratory facilities and supportive care. Results. Between 2000 and 2005, 261 children with non-lymphoblastic NHL were registered; 239 were eligible for the analysis. Two patients had stage I disease, 20 stage II, 179 stage III, and 38 stage IV. Fifty-two patients (22%) had bulky disease. Twelve children were assigned to therapeutic group A (low risk),184 to group B (intermediate risk), and 43 to group C (high risk). One hundred, and eighty-four patients (77%) had a complete response after the COP pre-phase. Sixty-nine patients (29%) died during treatment. Twenty-nine patients abandoned treatment. At 24 months, the overall survival rate of the entire patient population was 66% (CI 95%: 62.2-70.6) and the event-free survival rate 53.3% (CI 95%: 50.0-56.8). Conclusions. The treatment schedule proved effective, but the treatment-related mortality due to infections and metabolic complications was very high owing to the limited supportive care available. The high rate of treatment abandonment was also an important cause of failure, especially for children living far away from the hospital. Blood Cancer 2011;56:560-567. (C) 2010 Wiley-Liss, Inc

Rituximab plus bendamustine as front-line treatment in frail elderly ( extgreater70 years) patients with diffuse large b-cell non-hodgkin lymphoma: A phase ii multicenter study of the fondazione italiana linfomi

We conducted a phase II study to assess activity and safety profile of bendamustine and rituximab in elderly patients with untreated diffuse large B-cell lymphoma (DLBCL) who were prospectively defined as frail using a simplified version of the Comprehensive Geriatric Assessment (CGA). Patients had to be over 70 years of age, with histologically confirmed DLBCL. Frail patients were those younger than 80 years with a frail profile at CGA or older than 80 years with an unfit profile. Treatment consisted of 4-6 courses of bendamustine [90 mg/m 2 days (d)1-2] and rituximab (375 mg/m 2 d1) administered every 28 days. Other main study end points were complete remission rate and the rate of extra-hematologic adverse events. Forty-nine patients were enrolled of whom 45 were confirmed eligible. Overall, 24 patients achieved a complete remission (53%; 95%CI: 38-68%) and the overall response rate was 62% (95%CI: 47-76%). The most frequent grade 3-4 adverse event was neutropenia (37.8%). Grade 3-4 extra-hematologic adverse events were observed in 7 patients (15.6%; 95%CI: 6.5-29.5%); the most frequent was grade 3 infection in 2 patients. With a median follow up of 33 months (range 1-52), the median progression-free survival was ten months (95%CI: 7-25). The study shows promising activity and manageable toxicity profile of BR combination as first-line therapy for patients with DLBCL who are prospectively defined as frail according to a simplified CGA, as adopted in this trial (clinicaltrials.gov identifier: 01990144)

Incidence and Predictors of Early Treatment-Related Mortality In Pediatric Acute Lymphoblastic Leukemia In Baghdad (Iraq)

The overall cure rates for children with acute lymphoblastic leukemia (ALL) treated and managed in high-income countries have reached approximately 80% over the last two decades. Unfortunately, these advances in survival have not fully translated into low-income countries where the survival rates remain significantly lower (&lt;35%). Potential reasons for these different results include higher rates of relapse, a high degree of treatment abandonment, insufficient diagnostic work-up procedures, limited availability of effective drugs and supportive measures, and, consequently, high rates of treatment-related mortality (TRM). We examined the incidence, causes and risk factors for early (&lt;60 days) TRM in pediatric patients (15 years) with newly diagnosed ALL managed at the oncology unit of the Children Welfare Teaching Hospital in Baghdad (Iraq), over a 3-year period (2007 – 2009). Data were prospectively collected in Baghdad and analyzed at the GIMEMA Data Center in Rome. From January 2007 to December 2009, a total of 319 children (median age 5.2 years, range 0.3–13.9; 171 males and 148 females) with newly diagnosed ALL were registered; the diagnosis of ALL was confirmed by BM aspirate, according to the FAB classification; patients with L3 morphology (6 cases) were included. The median duration of symptoms prior to diagnosis was 4 weeks, ranging from 1 to 76 weeks. At disease onset, 179 children (56%) presented fever and 30 (9.4%) had hemorrhages; liver and renal functions were impaired in 9/290 (3%) and 21/289 (7.3%) patients with available data. The median Hb level was 7.0 g/dl (range 2.4–14.9), the median WBC count was 16.9 x 109/l (range 0.2–900) and the median platelet count was 35.0 x 109/l (range 0.1–598). CSF was positive in 14/290 children (4.8%). Patients were defined as low (153, 48%), intermediate (127, 40%) or high (33, 10%) risk according to clinical and laboratory parameters (age, hepatosplenomegaly, mediastinal mass, WBC, Hb level, platelet count, CNS and testicular infiltration). Sixteen children were discharged following the parents’ decision (14 before any treatment and 2 after 2 days); 303 children are evaluable for early TRM. Treatment consisted of a modified BFM-95 protocol in the first 31, a modified MRC UKALL-2003 protocol in 266 and the LMB/FAB-96 protocol in the 6 children with ALL-L3 morphology. Up to September 25, 2008 all trials did not include the 7-day steroid pre-phase that was introduced thereafter. A total of 249 children (82%) achieved a complete response in the first 60 days of treatment. The cumulative incidence of early TRM was 16% (48/303); it significantly decreased throughout the study period (2007: 21/88, 24%; 2008: 15/98, 15%; 2009: 12/117, 10% p 0.009). Several variables (sex, age, symptoms duration, hepatosplenomegaly, Hb level, WBC count, platelet count, bleeding, fever, impaired liver and renal function, CNS positivity, risk group, steroid pre-phase and induction complications) were examined as potential predictors of TRM. In univariate analysis, the occurrence of induction complications significantly increased the early TRM: hemorrhage 26% vs 11% p 0.001; infection 18% vs 2% p 0.005. A highly significant favorable impact on early TRM was represented by the 7-day steroid pre-phase; 36/169 children (21%) who did not receive the pre-phase died within the first 60 days of treatment compared to 12/134 children (9%) who underwent the steroid pre-phase (p 0.003). When the steroid pre-phase was placed in multivariable models with each of induction complications or other clinical parameters, it remained an independent predictor of TRM. Our experience confirms that a protocol-based care of children with ALL has to include the prednisone pre-phase that in low-income countries may contribute to a better risk definition and also to a significant reduction of early TRM