A 7-year-old boy with a history of recurrent acute lymphoblastic leukemia (ALL), in remission, presented to primary care clinic after 2 days of progressive right hip pain with weight-bearing activities. He was otherwise asymptomatic at the time of presentation. Blood cultures revealed Gram-negative diplococci, which prompted an MRI that was significant for a hip joint effusion and femoral head bone marrow edema. The patient had no sick contacts and no significant past medical history other than ALL. The patient had been given all recommended childhood vaccinations. Arthrocentesis and needle biopsy of the femoral neck were not diagnostic for malignancy and revealed only mild hip joint inflammation, leading to a diagnosis of osteomyelitis. The organism in the original blood culture was identified as Haemophilus influenzae type b, β-lactamase negative. Review of the patient's medical records showed a history of complete immunization to Haemophilus influenzae type b. An immunologic evaluation was made to determine if the patient retained immunity from his other vaccinations. Pathogen-specific antibody testing revealed detectable antibodies to polio but not measles, mumps, rubella, varicella-zoster virus, tetanus, diphtheria, pertussis, or hepatitis B. This loss of immunologic memory appears to be a rarely described side effect of ALL chemotherapy. There is currently no protocol to evaluate the immunologic memory of patients who underwent chemotherapy for ALL or to revaccinate them after their treatment. It is unclear whether the loss of immunologic memory is genuinely rare or is underdiagnosed because affected patients are protected by herd immunity.
Background: Iron overload has been implicated as a key co-morbid factor in post-stem cell transplant (SCT) outcomes. Quantifying iron burden has been a challenge as serum iron markers are imprecise. MRI is a relatively new and non-invasive tool that more accurately measures iron burden. We conducted a prospective study using MRI to assess iron burden in children undergoing SCT. Objective: To determine the prevalence of iron overload using R2 MRI of the liver in a pediatric allo SCT population both pre and post-SCT. Relationships between excess iron and adverse outcomes such as graft vs. host disease (GVHD), infection, and death were secondary objectives. Methods: Children $5 years old undergoing an allo SCT at Children's Hospital Boston from 2007-2009 were eligible for this study. Iron overload was defined as a liver iron concentration $ 1.5 mg iron/g dry liver tissue. A paired t-test was conducted with a 5 0.05 to compare iron load at the two specified time points. A two-sample two-sided t-test was conducted at each time point for association with risk factors described above with a 5 0.1. This study was IRB approved. Results: Twenty-eight patients were enrolled. The most common diagnoses were Pre-B ALL (N 5 7), AML (N 5 6), and aplastic anemia (N 5 5). Pre-SCT, 82% (95% CI: 66%-98%) of patients had iron overload by R2 MRI. At day 100 post-SCT, 95% (95% CI: 65%-100%) had iron overload. The mean iron concentration (6 std error) was 4.97 6 3.54 mg/g dwt liver at pre-SCT and 7.55 6 4.37 mg/ g dwt liver at day 100 post-SCT. The day 100 post-SCT mean value was significantly higher than the mean value at pre-SCT (2.15 mg/g dwt liver, p\0.0001). For patients who developed an infection during the post-SCT period, the mean iron concentration, compared to patients who did not develop an infection, was significantly higher at pre-SCT and at day 100 post-SCT (p 5 0.029 and p 5 0.0055, respectively). There was no statistically significant difference in iron concentration between patients who developed GVHD or died and those who did not. Conclusions: We found that a high proportion of pediatric allo SCT patients have iron overload pre-SCT. In addition, there is a statistically significant rise in iron post-SCT (p\0.0001) and an association with increased incidence of infection in patients with elevated iron. Larger multi-center studies should be conducted to further examine the consequences of iron overload and evaluate potential interventions in this patient population.
Macrophage activation in hemophagocytic lymphohistiocytosis (HLH) leads to severe infl ammation resulting in cytopenias and multi-organ dysfunction. Septo-optic dysplasia (SOD) is an as-yet unaffi liated disorder that manifests with optic, hepatic, endocrine and/or constitutional defects. We detail the fi rst reported occurrence of both HLH and SOD in one patient. This two-month old patient presented with acute hepatitis, direct hyperbilirubinemia, anemia and thrombocytopenia. Treatment followed standard of care practices for SOD and HLH. The patient subsequently underwent an allogeneic bone marrow transplant within eight months of diagnosis and remained in full remission at day + 90. We suggest considering a diagnosis of HLH in patients with SOD who present with severe liver failure refractory to standard therapy.
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