ABSTRACT
We present a case of acute myelogenous leukemia in a patient with severe hemophilia and our approach to the prevention of bleeding complications during chemotherapy. In the few reports of acute leukemia occurring in patients with hemophilia, management of bleeding has mostly consisted of replacement of factor and platelets on demand. By prophylactically treating our patient with Factor IX at 50% correction three times per week and maintaining his platelet count above 30 × 10(3)/mm(3), bleeding complications were avoided. However, due to the rarity of this combination, it is difficult to draw a best practice recommendation.
Subject(s)
Hemophilia B/complications , Hemophilia B/therapy , Hemorrhage/prevention & control , Leukemia, Myeloid, Acute/complications , Antineoplastic Agents/therapeutic use , Factor IX/therapeutic use , Hemorrhage/therapy , Humans , Leukemia, Myeloid, Acute/drug therapy , Male , Platelet Transfusion , Premedication , Young AdultABSTRACT
We present the case of a 2-year-old female with Wilms tumor whose initial evaluation revealed a prolonged prothrombin time (PT) and normal activated partial thromboplastin time. Mixing studies demonstrated correction of the PT and the Factor VII activity was 17% in the absence of a Factor VII inhibitor. She underwent successful resection of the tumor with fresh frozen plasma support and no excessive bleeding. Post-operative testing demonstrated normal PT at 3 days and 1-month. Although acquired von Willebrand factor deficiency has a known association with Wilms tumor, paraneoplastic factor VII deficiency associated with Wilms tumor is previously unreported.
Subject(s)
Factor VII Deficiency/complications , Wilms Tumor/complications , Child, Preschool , Factor VII/metabolism , Factor VII Deficiency/metabolism , Factor VII Deficiency/therapy , Female , Humans , Wilms Tumor/metabolism , Wilms Tumor/surgeryABSTRACT
BACKGROUND: Positron emission tomography (PET) differentiates normal from abnormal cells based on metabolic activity. Numerous studies report that PET scan offers increased sensitivity, specificity and predictive values as compared to computed tomography (CT) in adult lymphoma patients. PROCEDURE: Twenty-three consecutive pediatric Hodgkin lymphoma (HL) patients were evaluated with PET scan either at diagnosis or during treatment, then at therapy completion and in follow-up. RESULTS: Twenty two of the 23 patients had a negative PET scan at the end of therapy; however, ten later developed a positive scan for a total of 11 (47.8%) patients with a positive post treatment PET scan. Six tissue biopsies were performed in five patients; four specimens were negative for disease and two confirmed HL relapse. Six patients were monitored clinically and remained asymptomatic; four had resolution of abnormalities on repeat PET while two had persistently positive, but stable PET scan findings and continue to be in remission at 11 and 40 months following treatment. Twelve (52.2%) patients of the original cohort have had consistently negative PET scans and have not relapsed. CONCLUSIONS: PET is a sensitive (100%), but not a specific (57.1%) method for evaluating post-treatment pediatric HL patients with a strong negative predictive value (NPV; 100%), but poor positive predictive value (PPV; 18.2%). We do not recommend treatment decisions be based solely on PET scan results.
Subject(s)
Hodgkin Disease/diagnostic imaging , Positron-Emission Tomography/statistics & numerical data , Adolescent , Child , Child, Preschool , Combined Modality Therapy , False Positive Reactions , Female , Hodgkin Disease/drug therapy , Hodgkin Disease/pathology , Hodgkin Disease/radiotherapy , Humans , Male , Predictive Value of Tests , Recurrence , Remission Induction , Retrospective Studies , Sensitivity and SpecificityABSTRACT
Transcranial Doppler (TCD) is an effective method for screening patients with sickle cell disease (SCD) at risk for first stroke. Its usefulness in monitoring children with SCD receiving transfusions has not been established. The authors studied 17 children with SCD evaluated with TCDs and magnetic resonance angiograms (MRAs) while receiving transfusion therapy. Patients with normalized TCDs had normal MRAs that remained normal on transfusions. Patients with persistently abnormal TCDs had abnormal MRAs. In these children, TCD velocities decreased but rarely reverted to normal. Patients with low TCD velocities (<70 cm/s) had corresponding vasculopathy on MRA. Low velocities may be a risk factor for stroke and should be followed. Overall, there was good correlation between TCD velocity changes and MRA analysis.
ABSTRACT
Transcranial Doppler (TCD) is an effective method for screening patients with sickle cell disease (SCD) at risk for first stroke. Its usefulness in monitoring children with SCD receiving transfusions has not been established. The authors studied 17 children with SCD evaluated with TCDs and magnetic resonance angiograms (MRAs) while receiving transfusion therapy. Patients with normalized TCDs had normal MRAs that remained normal on transfusions. Patients with persistently abnormal TCDs had abnormal MRAs. In these children, TCD velocities decreased but rarely reverted to normal. Patients with low TCD velocities (<70 cm/s) had corresponding vasculopathy on MRA. Low velocities may be a risk factor for stroke and should be followed. Overall, there was good correlation between TCD velocity changes and MRA analysis.
