Acute and chronic renal impairment are important complications after HSCT. A prospective study was conducted to investigate the glomerular renal function in children who received allogeneic HSCT from matched related donors. Non-radiation conditioning regimens were used in all but one patient. CrCl and serial measurements of serum creatinine were evaluated prior to HSCT, within the first 100 days and one yr after. AKI was defined as at least a 1.5-fold rise in pre-HSCT serum creatinine within the first 100 days and classified as grade 1 to 3 according to the new definition criteria proposed by "AKI Network." Fifty-seven patients were enrolled in the study and 24 patients (42%) had AKI. CsA, amphotericin B, and SOS were found as risk factors for AKI. One yr after HSCT five patients (10%) had CKD and none of them required dialysis. None of the parameters were found as a predictor for CKD. We conclude that AKI is an important complication of HSCT. Careful monitoring of renal function, minimizing the use of nephrotoxic medication, prophylaxis, and effective treatment of SOS might be effective preventive measures to decrease the incidence of AKI.

Summary:We report a unique case of brucellosis transmitted by BMT. An 8-year-old boy with the diagnosis of Fanconi's anemia received an allogeneic BMT from his HLAidentical sibling. Routine culture from the infused marrow suspension grew Brucella abortus on day +4 post BMT. Spiking fevers occurred on days +2 and +16. The first febrile episode responded to broad-spectrum antibiotic therapy. However, the second episode did not. B. abortus was isolated from blood cultures taken during the second febrile episode. The Brucella agglutination titer was negative. Antibiotic therapy with oral doxycycline and i.v. gentamycin was successful with no recurrence of infection during 13 months of follow-up. The donor's blood culture was also positive for B. abortus and Brucella antibodies were detectable at 1:320 titer when he presented with fever and hepatosplenomegaly on day +32. We emphasize the need to consider brucellosis in patients undergoing BMT. We suggest that donor and recipient be evaluated for brucellosis especially in countries where the incidence of this infection is relatively high. Bone Marrow Transplantation (2000) 26, 225-226. Keywords: brucellosis; bone marrow transplantation; infection Brucellosis continues to be an important health problem worldwide, mainly in the Mediterranean area and in developing countries. It is primarily a zoonotic infection which can be transmitted to humans through direct contact with infected animals and through consumption of unpasteurized milk or milk products. Human to human transmission of brucellosis is extremely rare. Transmission of Brucella by blood transfusion has been described, 1,2 however, there are no reports in the literature concerning brucellosis occurring in a recipient of bone marrow transplantation. A patient who received bone marrow cells containing Brucella melitensis has been reported, although brucellosis in this recipient could not be demonstrated. 3 We report the case of a patient with bacteriologically documented brucellosis transmitted by BMT. Case reportAn 8-year-old boy with Fanconi's anemia received an allogeneic BMT from his HLA-identical brother. Conditioning consisted of oral busulphan 6 mg/kg/total dose for 4 days and intravenous cyclophosphamide 40 mg/kg/total dose for 4 days. Since there was a bidirectional ABO incompatibility, bone marrow was infused after RBC and plasma depletion. The nucleated cell dose of marrow was 2 × 10 8 /kg. A routine culture was sent from the bone marrow suspension. Selective gut decontamination with ciprofloxacin and fluconazole was administered during neutropenia. Graft-versus-host disease (GVHD) prophylaxis was cyclosporin A 3 mg/kg/day i.v. and methotrexate 5 mg/kg i.v. for three doses after transplant. An absolute neutrophil count (ANC) of over 500/mm 3 occurred on day +11, and a platelet count of over 20 000/mm 3 on day +28. Grade I acute GVHD occurred on day +28.The early post-transplant course was complicated by fever that developed on day +2. Broad-spectrum antibiotic therapy with meropenem was started and th...

From January 1991 to June 2009, 245 children with beta thalassemia major who underwent their first allogeneic HSCT in Turkey and who were followed for a minimum of one yr post-transplantation were enrolled this study. The median age of the patients was 6.6 yr old (range, 1-22 yr). The distribution of Pesaro risk class I, II, and III categories was 41, 130, and 63 children, respectively. The median serum ferritin level was 2203 ng/mL. Eighty-eight patients received bone marrow (BM) stem cells; 137, peripheral blood (PB) stem cells; and 20, cord blood (CB) stem cells. The donors were HLA-matched siblings or parents. Median engraftment times were shorter in PBSCT patients compared with the BMT group (p < 0.001). Grade II-IV acute GvHD was observed in 33 children (13.5%), while cGvHD was observed in 28 patients (12.5%), eight of whom had the extensive form. Thalassemic reconstitution was observed in 43 (17%) of the transplant patients. Post-transplant aplasia occurred in three patients, and the TRM rate was 7.75%. Seventeen patients were lost after 100 days. The thalassemia-free survival and OS rates were 68% (95% CI, 61.8-74.2) and 85.0% (95% CI, 80.2-89.8), respectively. We believe that this study is important because it is the first multicenter national data for children with beta thalassemia major receiving HSCT.

Haematopoietic stem cell transplantation (HSCT) remains the best therapeutic option for many acquired and inherited paediatric haematological disorders. Unfortunately, the probability of finding an HLA matched donor is limited. An alternative technique is PGD combined with HLA matching, which offers the possibility of selecting unaffected embryos that are HLA compatible with the sick child, with the aim of possible use of stem cells from the resulting baby in future. Since the first successful report for Fanconi anaemia a decade ago, the therapeutic success of this technique was reported in a few cases and for a limited number of disorders. Here, we report full recovery of 44 sick children who received HSCT from healthy infants conceived after pre-implantation HLA matching for the following 10 indications; beta-thalassaemia, Wiskott-Aldrich syndrome, Fanconi anaemia, sickle cell anaemia, acute myeloid leukaemia, acute lymphoblastic leukaemia, Glanzmann's thrombasthaenia, Diamond-Blackfan anaemia, X-linked adrenoleukodystrophy and mucopolysaccharidosis type I. No serious complications were observed among recipients and donors. Graft failure occurred in four children with beta-thalassaemia where a second HSCT was planned. Preimplantation HLA matching is a reliable technique and provides a realistic option for couples seeking treatment for an affected child when no HLA-matched donor is available.

The term "ES" has been widely used for describing a clinical condition consisting of skin rash, fever, and weight gain that occur during neutrophil recovery period following HSCT. In this study, the incidence, clinical features, risk factors, and outcomes of ES were evaluated in 169 children following allogeneic HSCT from full-matched related donor according to the Spitzer criteria. Seventeen patients (10.1%) presented with clinical conditions suggesting ES. In both univariate and multivariate analysis underlying malignant disease and early release of monocytes to the PB, and in univariate analysis using only CsA for GVHD prophylaxis were found to be the significant risk factors for the development of ES. Patients with ES experienced significantly higher incidence of acute and chronic GVHD and propensity toward a higher rate of TRM. OS did not differ between the patient groups. Thirteen of 17 patients received steroid therapy, and all but one patient responded to therapy. Monitoring for early detection of ES and early intervention with steroid therapy is the key for recovery. The most crucial approach for this purpose mainly is to find out and use the most useful and feasible diagnostic criteria for routine medical practice.

Summary:There is an appreciable mortality associated with BMT in patients with SCID and advanced BCG infection. We present a girl with T-B+ SCID complicated by spina ventosa and disseminated BCG osteitis after receiving a fully matched sibling marrow transplant. The severe combined immunodeficiencies are a group of genetic disorders characterised by profoundly defective T lymphocyte (with or without B lymphocyte) differentiation that leads to early death in the absence of hematopoietic stem cell transplantation.1,2 BCG vaccine derived from multiple passages of wild-type Mycobacterium bovis is administered throughout the developing world to attenuate the effects of natural tuberculosis infection.3,4 However, in individuals with defects of the host immune response, serious BCG infections can occur. 4,5 We report a patient with SCID who developed disseminated BCG disease after BMT and who improved after successful engraftment in conjunction with intensive, long-term multiagent anti-mycobacterial treatment. Case reportA 6-month-old girl was admitted to our clinic with persistent thrush, diarrhea and cough. Her past medical history was unremarkable other than for a BCG vaccination on her left deltoid muscle during the early neonatal period, according to the standard practice in Turkey. Her symptoms had begun in the first month of life and an 8-month-old female sibling had died with similar symptoms. She was the fourth child of consanguineous parents. Immunological testing revealed T-B+SCID (Table 1). She received a fully matched sibling marrow according to EBMT protocol 1997, 4 weeks after the diagnosis. Standard doses of Isoniazid (INH) were administered prophylactically. Engraftment followed by grade I aGVHD was achieved on day 13. During follow-up, persistent fever occurred for 4 weeks which could not be explained by any viral, bacterial or fungal infection. Subsequently, disseminated BCG infection presented as spina ventosa, hepatosplenomegaly and soft tissue abscess (Figures 1 and 2) and was diagnosed by EZN positive smear, PCR and mycobacterial culture (Mycobacterium tuberculosis complex) (Figures 3 and 4). She had received an antimycobacterial chemotherapy regimen comprising four agents (INH 10 mg/kg/day, rifampicin (Rif) 15 mg/kg/day, streptomycin (SM) 40 mg/kg/day, clofazimine 1 mg/kg/day for 2 months followed by just INH and Rif for 4 months to which the organism was sensitive. During this period she did well with full immune reconstitution and both clinical and radiological regression of the tuberculosis infection for 6 months. However, since the BCG disease relapsed in the 6th month of antimycobacterial chemotherapy, she started alternative agents amikasin (Amc) 15 mg/kg/day, ciprofloxacin (Cipro) 30 mg/kg/day, clofazimine 1 mg/kg/day, in addition to two major (INH and Rif) drugs. Amikasin was stopped in the 12th month of therapy when she became afebrile for the second time. She did well, with significant clinical and radiological regression of the tuberculosis infection for another 6 months on anti-tube...

h A very unusual clinical presentation of Hodgkin disease with immune thrombocytopeni a and autoimmune hemolytic anemia is reported. A 6.5-year-old boy presented with thrombocytopenia, Coombs' positive hemolytic anemia, and multiple small posterior cervical lymph nodes. After a course of high-dose methylprednisolon e therapy with a diagnosis of Evans syndrome, complete response for thrombocytopenia and partial response for anemia was achieved. Six weeks later there was a sudden increase in the size of left posterior cervical lymph nodes and a biopsy was compatible with Hodgkin disease, mixed cellularity type. The child was successfully treated with chemotherapy and radiation therapy. He has been off therapy for 28 months and has no clinical or laborator y evidence of autoimmune cytopenia. A combination of immune thrombocytopenia and autoimmune hemolytic anemia may be associated with Hodgkin disease. The recognition of this clinical picture as a complication of Hodgkin disease has important implications. This complication appeares to be managed best by the de® nitive treatment of Hodgkin disease.

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