Snapshot A 15-year-old girl presents to the emergency room with a nosebleed that has not stopped for hours. She also has bleeding in her gums. She was recently started on carbamazepine for a newly diagnosed epileptic condition. Lab results reveal decreased counts in all leukocyte counts. Reticulocyte count is decreased as well. Carbamazepine is discontinued and a bone marrow biopsy is obtained. Introduction Pancytopenia caused by diminished, absent, or destructed hematopoietic stem cells With bone marrow aplasia Epidemiology no racial or gender predisposition most cases are idiopathic - it is believed to be an autoimmune etiology Multiple different causes radiation drugs or chemicals benzene chloramphenicol anti-epileptics (phenytoin and carbamazepine) alcohol alkylating agents insecticides viruses EBV HIV CMV HCV parvovirus can cause transient aplastic crisis rarely can progress to aplastic anemia Fanconi anemia (congenital) DNA repair defect idiopathic B12 and folate deficiency PNH SLE PTU and methimazole Commonly seen in sickle cell patients who are infected with parvovirus B19 Presentation Symptoms/physical exam insidious onset, but often initial symptoms are due to anemia or bleeding (often normocytic) anemia: fatigue, malaise, and pallor thrombocytopenia: mucosal bleeding and petechiae leukopenia: infections Evaluation Diagnosis of exclusion Labs anemia leukopenia thrombocytopenia ↓ reticulocyte count Bone marrow biopsy hypocellular bone marrow with fatty infiltration Differential Diagnosis PNH Myelodysplastic syndrome Infection Treatment Withdrawal causative agent if applicable Supportive therapy RBC transfusion platelet transfusion Bone marrow transplant Antithymocyte globulin plus cyclosporine Hematopoietic growth factors (G-CSF and GM-CSF) Prognosis, Prevention, and Complications Prognosis 10-year survival rate immunosuppression – 68% stem cell transplant – 73% Complications infection bleeding complications of stem cell transplant graft versus host disease
QUESTIONS 1 of 4 1 2 3 4 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.HE.15.39) A 5-year-old boy presents for examination to his pediatrician. His mother explains that he is not growing at the expected rate. You observe the physical exam findings depicted in Figure A. Results of his complete blood count are reported as follow: WBC 3,500/microliter; Hb 9.8 g/dL; MCV 95 fL; platelets 98,000/microliter. What is the most likely underlying explanation of these findings? Tested Concept QID: 104486 FIGURES: A Type & Select Correct Answer 1 Red cell aplasia 14% (20/139) 2 Iron deficiency 1% (2/139) 3 DNA repair disorder 49% (68/139) 4 Hemoglobinopathy 27% (38/139) 5 Infection 7% (10/139) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK