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BackgroundEcthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate immunity, is an autosomal recessive disorder characterized in its severe phenotype by a complete defect in CD18 expression on neutrophils, delayed cord separation, chronic skin ulcers mainly due to recurrent bacterial and fungal infections, leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low number of neutrophils at sites of infection.Case PresentationWe report at our knowledge the first case of a child affected by LAD-1, who experienced during her disease course a multi-bacterial and fungal EG lesion caused by fusarium solani. Despite targeted antibiotics and anti-fungi therapy, the lesion extended for as long as 18 months and only massive granulocytes pockets transfusions in association with G-CSF had the capacity to cure this lesion.ConclusionWe propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely immunocompromised patients.

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Gene frequencies for the human platelet antigens HPA-1, -3 and -5 in the Tunisian population were determined by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) on 93 volunteer blood donors (78 were tested for HPA-1, 90 for HPA-3 and 93 for HPA-5). This study shows the highest frequencies of the HPA-1b (0.25) and HPA-5b (0.22) yet recorded. These antigens are considered as markers of a high risk of platelet alloimmunisation in other populations, and for this reason particular attention should be paid in the case of pregnancy or blood transfusion in this population. The 9 base pair deletion located in intron 21 of the GPIIb gene associated with HPA-3b determinant is present in this population. No individual showed the polymorphism associated with HPA-1b (T-->G at codon 40 of the GPIIIa).

DMSO is used as a cryoprotectant for long-term storage of haematopoietic progenitor cells from the marrow or blood. 1 Until recently, there has been little information on the neurological toxicity of DMSO other than headache. 2 Recent reports suggest that this may be a more frequent problem than currently appreciated. [3][4][5][6][7] A 50-year-old woman developed a Durie-Salmon stage III IgA multiple myeloma in October 2007 and received first-line therapy consisting of thalidomide and dexamethasone. A PBSC collection was performed (highdose CY (4 g/m 2 ) and G-CSF (5 mg/kg/day)). Three million CD34 cells per kilogram were collected. The cells were cryopreserved in 10% DMSO (B. Braun Medical SA, Boulogne, France) using a controlled-rate freezer (Nicool Plus PC, Air Liquide, France) and stored at À150 1C. The final volume cryopreserved was 480 ml in six bags, with approximately 8 g of DMSO per bag (0.13 g/kg DMSO per bag). After PBSC collection, she underwent autologous PBSCT (day 0 ¼ 11 April 2008). The conditioning regimen consisted of melphalan at a dose of 200 mg/m 2 . The bags were thawed in a 37 1C water bath and infused at a rate of 10 ml/min. After the infusion of the last bag, she developed skin pallor, and loss of consciousness (Glasgow Coma Scale 4/15) with hypoxia. Pulse rate was 70/min and blood pressure 120/75 mm Hg. She had no localizing neurological signs. No obvious tonic-clonic activity was noted although she had bitten her tongue and had an evidence of trismus. A seizure was suspected and she was administered with a 15 mg/kg loading dose of phenytoin. The patient was transferred to intensive care unit for ventilation. Blood count, electrolytes, liver function tests and coagulation screen were normal. An urgent brain CT scan was unremarkable. Electroencephalogram showed diffuse abnormalities in the electric activity with irregular paroxysms of slow waves of generalized projection.The patient recovered consciousness in o3 h after assistance ventilation was started. Finally, she was extubated within 24 h, and was discharged from the ICU on day þ 1. She engrafted on day þ 11, and was discharged from our unit on day þ 19. She is now 7 months post transplant, and in CR.Various complications during infusion of cryopreserved haematopoietic stem cells have been described, including nausea, vomiting, cardiac symptoms, transient hypertension or hypotension and anaphylaxis. 8,9 Our patient developed a profound but rapidly reversible coma after infusion of 48 g of DMSO (the equivalent of 0.78 g/kg). She developed this severe complication despite an acceptable dose of DMSO reinfused at a rate generally considered to be safe. The maximal recommended dose of DMSO to be reinfused in one session is 1 g/kg of body weight or 10 ml/kg of a 10% DMSO cryopreserved solution.Other probable causes of seizures, such as metabolic abnormalities, structural brain lesions and sepsis, were ruled out. In the absence of other causes, we attribute the reaction to the DMSO toxicity. Although the pathophysiology of DMSO neurotoxici...