Spleen function in children with sickle B+ thalassemia.

Splenic function in patients with sickle B+ (SB+) thalassemia has been poorly documented. We evaluated the clinical course and splenic function in 12 children with SB+ thalassemia with simultaneous technetium sulfur colloid spleen scans and determination of pitted erythrocytes by direct interference contrast microscopy (DICM). All patients displayed normal uptake of radiocolloid. Mean percentage of pitted erythrocytes was 2.2% compared to 0.06% in 10 normal eusplenic controls and 13.8% in 10 sickle cell patients. In this group of children, who were carefully monitored for 136 patient years, there was no episode of bacteremia/sepsis, and a low prevalence of vaso-occlusive episodes. The slight increase in percentage of pitted erythrocytes of SB+ thalassemia patients does not seem to herald any clinically relevant loss of splenic function. SB+ thalassemia children younger than 10 years of age who do not seem to present a higher risk of invasive bacterial infections than eusplenic children, should receive conservative treatment for isolated febrile episodes and should not be submitted to prophylactic penicillin.

Transient expression of trisomy 21 and monosomy 7 following cyclosporin A in a patient with aplastic anemia.

We present a patient with severe idiopathic aplastic anemia with no previous chromosomal abnormalities who developed trisomy 21 and monosomy 7 during treatment with intravenous (i.v.) cyclosporine. The abnormal karyotype disappeared when the drug was changed to the oral form. This cytogenetic aberration, previously unreported in association with cyclosporine, may reflect either a direct drug effect or the emergence of a hidden myelodysplastic cell clone subject to preferential survival during immunosuppression.

Successful treatment of disseminated Fusarium infection in an immunocompromised child.

We report the first know case of disseminated fungal infection due to Fusarium proliferatum in a bone marrow transplant recipient to our knowledge. Fusarium was cultured from the blood, a paranasal sinus, and necrotic skin lesions. The isolate was sensitive to amphotericin B and on further sensitivity testing, synergy was demonstrated using rifampin in combination with amphotericin B. The patient had this infection while she was receiving alternate-day amphotericin, rifampin, and 5-flucytosine (5-FC) therapy. The infection was documented within 48 h of discontinuing daily granulocyte transfusions, which she had received for 3 weeks. The 5-FC was discontinued when sensitivities showed the organism resistant. After 6 weeks of treatment she showed complete remission of the infection, although neutrophil counts remained below 0.25 X 10(9)/L. From this case and from a review of the literature, it appears that synergic antifungal agents combined with leukocyte transfusions may be beneficial in the successful treatment of fusariosis in the compromised host.

Squamous cell carcinoma of the tongue associated with Fanconi's anemia: MR characteristics.

Children with Fanconi's Anemia are reported to be at increased risk of associated carcinoma. The MR appearance of an 11 year old boy with complicating squamous cell carcinoma of the tongue is presented.