Few studies have compared the incidence of infections occurring ≥2 years after hematopoietic cell transplant (HCT) with other cancer patients and the general population. In this study, ≥2-year HCT survivors who were Washington residents treated from 1992 through 2009 (n = 1792; median age, 46 years; 52% allogeneic; 90% hematologic malignancies) were matched to individuals from the state cancer registry (n = 5455, non-HCT) and driver’s license files (n = 16 340; Department of Licensing [DOL]). Based on hospital and death registry codes, incidence rate ratios (IRRs; 95% confidence interval [CI]) of infections by organism type and organ system were estimated using Poisson regression. With 7-year median follow-up, the incidence rate (per 1000 person-years) of all infections was 65.4 for HCT survivors vs 39.6 for the non-HCT group (IRR, 1.6; 95% CI, 1.3-1.9) and 7.2 for DOL (IRR, 10.0; 95% CI, 8.3-12.1). Bacterial and fungal infections were each 70% more common in HCT vs non-HCT cancer survivors (IRR, 1.7; P < .01), whereas the risk for viral infection was lower (IRR, 1.4; P = .07). Among potentially vaccine-preventable organisms, the IRR was 3.0 (95% CI, 2.1-4.3) vs the non-HCT group. Although the incidences of all infections decreased with time, the relative risk in almost all categories remained significantly increased in ≥5-year HCT survivors vs other groups. Risk factors for late infection included history of relapse and for some infections, history of chronic graft-versus-host disease. Providers caring for HCT survivors should maintain vigilance for infections and ensure adherence to antimicrobial prophylaxis and vaccination guidelines.
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) comprises lupus anticoagulant, acquired hypoprothrombinemia, and often mild thrombocytopenia or normal platelets. It is usually associated with autoimmunity or postviral illness. We describe a case of a 10-year-old boy with oral bleeding and severe thrombocytopenia initially suggestive of immune thrombocytopenia. Secondary to bleeding, evaluation demonstrated prolonged coagulation tests and subsequently revealed the presence of lupus anticoagulant and hypoprothrombinemia, along with marked autoimmunity, suggestive of LAHPS. He was treated with intravenous immunoglobulin and hydroxychloroquine. This case report and discussion highlight the diagnostic and therapeutic challenges associated with LAHPS and coincident severe thrombocytopenia.
Introduction: Immune thrombocytopenic purpura (ITP) is the most common cause of primary isolated thrombocytopenia of childhood. Although ITP results in profound thrombocytopenia, the severity of bleeding in this disease does not correlate well with the platelet count suggesting a compensatory procoagulant state. The primary aim of this study is to evaluate thrombin generation in childhood acute ITP patients compared to age-matched controls and examine procoagulant activity in relation to bleeding severity in childhood acute ITP. Methods: After IRB consent was approved, platelet poor plasma was isolated from acute ITP patients at the time of initial diagnosis and serially for up to 6 months post-diagnosis. A single sample was obtained from age-matched controls. Thrombin generation assays were run via the Calibrated Automated Thrombogram (CAT) using reagent containing 1pM tissue factor and 4uM phospholipid. Peak thrombin levels were measured and compared to control samples using paired t-test analysis. Clinical bleeding severity was assessed using a modified overall Buchanan bleeding score at each time point (0=no bleeding, 1=minor, 2=mild, 3=moderate, 4=severe, 5=life threatening bleeding). Results: Samples from 19 childhood acute ITP patients, mean age 7 years, and 8 controls, mean age 5 years, were assessed. The figure below demonstrates that despite profound thrombocytopenia, the peak thrombin values of acute ITP patients were higher than controls throughout the first 3-4 months following diagnosis. ITP assays were significantly different from controls at the following time-points: 1-2 weeks (p =0.001), 3-4 weeks (p =0.022), 5-8 weeks (p =0.0009), 9-12 weeks (p =0.004), and 13-16 weeks (p =0.006) post-diagnosis. Peak thrombin values returned to values similar to controls 17 weeks from diagnosis. With the onset of acute ITP, higher bleeding scores were related to more severe thrombocytopenia. In ITP patients, thrombin peak values remained elevated regardless of resolving thrombocytopenia and improved bleeding scores (see figure). Conclusion: To our knowledge, this is the first study to assess longitudinal thrombin generation using the CAT in childhood ITP patients. In a highly significant pattern, acute ITP patients demonstrated sustained elevated peak thrombin levels compared to controls, even at acute onset of disease. Certainly, thrombin release, and subsequent thrombin generation play a compensatory role to decrease bleeding among childhood ITP patients when the platelet count remains in the 10-50,000 range. But, based on bleeding scores, this elevated thrombin may not be able to overcome the bleeding phenotype at presentation when platelets are most profoundly decreased or absent. Greater understanding of the etiology of this thrombin potential in childhood acute ITP is critical, as the thrombocytopenia in this disorder may be triggered or linked to a larger post infectious or inflammatory coagulopathy. Figure 1. Figure 1. Disclosures No relevant conflicts of interest to declare.
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