Heme Onc Flashcards
Can be caused by cows milk and foods not eriched with iron. Creates leakage of blood in the gastrointestinal mucousa. Pt presents with conjunctival pallor. Dx? Labs ? Rx?
Iron Deficiency Anemia. Decrease bone marrow hemosiderin, serum ferritin, serum iron and transferin, INCREASED TIBC, and increased FEP
Low MCV, hypocrhomic. Ferous Sulfate.
Associated with older housing before 1960. Can present with hyperactivity and aggression in children. Cognivitive/development and dysfunction. Constipation and elevated ICP. Dx? Test? Rx (3)
Lead Poisoning. Screening 12 and 24 months of age. Venous sample to confirm. >20 inform health department and recheck 45-70 health department + succimer oral (DMSA) >70 health department + hospitilization + IV EDTA and dimercaprol
Short stature, triphalangeal limbs and anemia. Dx? Labs? Rx? Definitive Rx?
Congential Pure red cell anemia (blackfan Diamond Syndrome) Macrocytosis, increased HBF, Increase RBC ADA, Very LOW RETICULOCYTES!!! Rx: Steriods and transfusion with deferoxime. Definitve is stem cell transplant.
Cafe-au-lait spots, absent hypoplastic limbs, short stature Dx? Labs? Rx? Definitive Rx?
Fanconi Anemia. Decrease in RBC, WBC and platelets. Bone marrow hypoplasia. Do Bone marrow aspirate. Steroids. Bone marrow transplant.
Transient hypoplastic anemia between 6 months and 3 years of age. Transient immune suppresion. Dx? Labs? Rx?
Transient Erythroblastopenia of Childhood. Decreased Reticulocytes and bone-marrow precursors. normal MCV and HbF
AD with abnormal shape of RBC due to spectrin deficiency leading to decreased deformability and early removal of cells by spleen. Presents with anemia and hyperbilirubinemia of newborn. Dx? Test? Rx?
Hereditary Spherocytosis and Elliptocyctosis. Spherocytes and Elliptocytes. Osmotic Fragility Test. Transfusions and Splenectomy.
2 year old boy was given Bactrim for an ear infection and 2 weeks later comes back pale with low hb and hct. Dx? Test? Rx?
G6PD deficiency. Measure of G6PD activity. Prevention.
Dactilitis, functional asplenia, avascular necrosis of hip and shoulder, acute chest syndrome, stroke, priapism, proteinuria, paplillary necrosis. Dx? Test? Rx?
Sickle Cell Anemia. Target cells, sickle, cells, howel jolly bodies. Pain and O2 with crisis. Transfusions, abx. hydroxurea.
Test and Rx for Beta thalassemia major?
Electrophoresis. Can see target cells on blood smear. Transfusions with deferuxomine. Bone marrow transplant is definitive.
Rx for HBH?
No treatment is usually indicated for alpha thalaseemia (3 gene deletion)
- With the mixing study what would correction suggest? 2. What would none or partial correction suggest. 3. More prolongation suggest? 4. What if there is no clinical bleeding but both PTT and mixing study are prolonged?
- Deficiency of a clotting factor. 2. There is an inhbitor Heparin 3. Antibody directed against the clotting factor. 4. Lupus anticoagulant
Xlinked. Easy brusing with hemathroses. Dx? Test? Rx?
Hemophilia A. Increase PTT and correction with mixing studies. Replace factor. Prophylaxis recommended in young children with severe bleeding - it prevents chronic joint disease. Mild bleeding can give desmopressin.
AD. Mucocutaneous bleeding. Dx? Test? Rx?
von Willebrand Disease. Increased bleeding time and PTT. Check quantitative assay for vWFAg, vWF acitivity. Give Desmopressin. Other types need replacement vwf that contains factor 8.
Rx for patients with liver dz that lack all clotting factors?
Fresh Frozen plasma for clotting factors and cryoprecipitate for fibrinogen and factor 8
4 year old boy presents with petechiea, purpura and excessive bleeding after falling from his bicycle. Dx? Test? Rx?
ITP. Autoantibodies against platelet surface. Platelets < 20,000 Bone marrow shoes increased Megakaryocytes. IV immunoglobuline for 1-2 days. There is no transfusion needed if no ongoing bleeding and platelets >20,000. Transfusion is contraindicated unless there is life threatening bleeding because the patients antibodies will attack the donors platelets.
