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APHON IX
APHON IX: Hematologic Disease
| Question | Answer |
|---|---|
| Hematological characteristics of Diamond-Blackfan Anemia | Anemia of RBC only. Plt & WBC normal. RBC's macrocytic. Decreased reticlocytes. |
| Clinical presentation of Dimand-Blackfan Anemia. | Rare condition, presents in infancy. Anemia & pallor in 1st 3mth life. 25-30% pt have congenital anomalies (usually skeletal). High risk of malignancies. |
| Dimand-Blackfan Anemia treatment | 20% spontaneous remission, high dose steriods, RBC transfusion for life, chelation with Desferal. HSCT. |
| What % of Dimand-Blackfan Anemia patients need regualr transfusion? | 10%. 60-80% respond to steroids, 20% have spontaneous remission. |
| Long term prognosis for Dimand-Blackfan Anemia patients? | High functioning life with transfusion & chelation programs. Death usually in late teens from MSOfailure, hemosiderosis (build up of iron). High risk for infection, and AML) |
| Hematological characteristics of Fanconi's Anemia?` | Rare autosomal recessive disease. Hypoplastic marrow. Reduction of all hematopoietic precursors (congenital aplastic anemia). Associated with other congenital anomilies, CNS, hypoplastic thumbs, skin, skeletal. |
| What is the treatment & long term outlook for Fanconi's anemia? | Androgens & steroids keep symptoms in check. Max life span young adulthood. HSCT only cure & life extension. |
| Hematological characteristics of Aplastic Anemia. | Complete BM failure. Marked decrease in RBC, WBC, plt. |
| What is the congenital form of Aplastic Anemia? | Fanconi's Anemia. |
| What are the 4 suspected causes of acquired Aplastic Anemia? | 1) 50% idiopathic 2)activated suppressor lymphocytes (autoimmune responce) 3) Rx/toxin/chemicals 4)radiation |
| Treatment of Aplastic Anemia. (2) | 1) HCT is standard of care 2)Attempt to reverse autoimmune process with ATG,mythylprednisolone, CSA, Cytokines(g-csf, gm-csf, erythropoieten, oprelevekin(plts. |
| Aplastic Anemia prognosis? | Hard to predict. High risk of later malignancies. Recieve ATG 10times more likely to develop AML or MDS than those who get HCT. |
| Hemetological characteristics of Immune Thrombocytopenia Purpura (ITP)? | Acquried excess destruction of circulating plt.Def = Plt <100K, Anti-plt antibody, short plt lifespan, increased megakaryocytes in BM. Present with ecchymosis/ petechiae/ epistaxis. |
| Etology of Immune Thrombocytopenia Purpura (ITP)? | Acquried excess destruction of circulating plt. Note: not a production problem. Hx of viral infection within 2-4wk. |
| Characteristics of Acute ITP | children (2-6yrs)>adults, follows minor viral infection, duration 2-6wk, 90% normal have norm plt in 4mth. |
| Characteristics of Chronic ITP | adults>children, 3:1 female, insidious onset with lupus antigen association, duration is months to years, fluctuating chronic disease |
| Presentation of ITP | Petechiae/purpura, Decreased circulating platelets, All other blood indices normal No hepatosplenomegaly Healthy looking child |
| ITP treatment? | Observation is safe & as effective as tx. Steroids, IVIG (or IgG/WinRho), |
| How does IVIG work in ITP? | Coats the platelets making attachment of the antibody impossible, so they are not destroyed in the spleen. This is very expensive therapy and requires daily repeat IV doses for 2-3 days. |
| How does IgG/WinRho work in ITP? | Newer specific IgG product prevents attachment of the antibody to the platelet,preventing premature destruction by the spleen. For WinRho® to be effective, the child must have an RH+ blood type. Has an affinity for RBCs => transient hemolytic anemia. |
| Why don't plt transfusions work in ITP? | Antibodies attach to the new platelets & they are destroyed by the spleen. Platelet transfusions used in emergency situations. Now splenectomy is rarely done and almost all treatment is medically managed. |
| ITP prognosis? | 89-90% resolve in 4mth. 95% in 6mth. Chronic is >6mth. Recurrence in children rare. Not associated with further dx or malignancy. |
| Hemophilia A | Factor VIII deficiency. 85% of all hemophilia. 45/1 million male births. |
| Hemophilia B | Factor IX deficiency (christmas disease). 2-5/1 million births. |
| Def: Hemophilia | X-linked congenital bleeding disorders. Protien deficiency effects normal clotting. Occurs in all races. |
| Hemophilia clinical presentation | Excessive/prolonged bleeding inc circumcisional trauma. Hemarthrosis. Signs of bleeding inc tingling/pain/swelling. |
| Clinical approach to bleeding in hemophilia pt. | Give factor at earliest sign of a bleed. The worst thing that happens is that factor was wasted, but if you wait for definitive diagnosis of a bleed, it may be too late to protect them from the sequela. |
| Hemophilia treatment. | Factor replacement therapy Treat at first sign of bleed! If in doubt, treat! Prophylactic use of factor long term damage to joints Family teaching paramount to outcomes Genetic counseling |
| Hemophilia prognosis. | Excellent progress with appropriate management. Gene therapy is hope for the future Factor IX phase II study in clinical trials Need routine Hepatitis C screening HIV no longer an issue in pediatrics transplant pre 1986 risk for HIV contration |
| Def: Thalassemia | Autosomal recessive disease. Group of inherited anemias, mutated alpha or beta HGB synthesis. Deletions/deficient globin chain synthesis Variable clinical expression affected by number of gene deletions. |
| Thalassema demographics. | The most common worldwide genetic disorder 3% (> 150 million people)have beta-thalassemia gene. >50% of some populations carry alpha-thalassemia gene. Distribution similar to malaria pattern of occurrence. |
| Characteristics of a "SILENT CARRIER" of thalassemia? | alpha or beta single gene deletion resulting in normal hematopoesis |
| Characteristics of a "THALASSEMIA TRAIT (minor)" | alpha or beta 2 gene deletion resulting in RBC microcytic/hypochromic, very mild anemia. |
| Characteristics of "HBG H" type Thalassemia | alpha 3 gene deletion. Moderately severe hemolytic anemia/icterus/splenomegaly |
| Characteristics of "HYDROPS FETALIS" type Thalassemia | alpha 4 gene deletion. Death in utero. |
| Characteristics of "SEVER BETA-THALASSEMIA/Cooley's Anemia" Type thalassemia | Severe anemia, growth retardation, hepatosplenomegaly, bony deformities |
| Characteristics of "THALASSEMIA MAJOR" | transfusion dependent |
| Characteristics "THALASSEMIA INTERMEDIA" | No regular transfusion requirement |
| Presentation of Severe Beta thalassemia/ cooley's anemia | Usually apparent between 6-24 months of age Failure to thrive in infancy. Development of anemia. Increased pallor/icterus. HSM, functionally asplenic. Increased susceptibility to infection. Fatigue. |
| Thalassemia Treatment | Supportive care,Chronic pRBC transfusions, Iron chelation, NO iron supplementation, Vitamin C & E/folate supplement, Splenectomy Infection prophylaxis, Genetic counseling HCT is curative |
| Thalassemia complications/ prognosis? | Growth retardation/delayed puberty, High incidence of metabolic disorders (inc diabeties), Pathologic fractures& avascular necrosis, Cholelithiasis, Myocardial hemosiderosis, Actual lifespan related to severity of disease and therapy |
| Thalassemia future directions | Gene therapy, Hydroxyurea/butyrates, Intrauterine PRBC transfusions, with prenatal detection of hydrops fetalis |
| Sickle Cell Disease etology & incidence | Autosomal recessive genetic trait. Mandalian inhertance pattern. Each parent has 2 beta chains for making HGB 1:625 all races carry trait, 8% of African Americans have trait/carry gene. Screening at birth. 1:400 Black Americans have SCD |
| Sickle cell Pathophysiology | Abnorm HGB molecule (amino acid substitution)=> Chronic hemolytic anemia, Organ dysfunction. Sickling of cells occurs with deoxygenation=> "sticky" Sickled cell => Microvascular occlusions, ischemia, infarcts and tissue death. |
| SCD complicaitons | All due to occlusion of blood vessels by sickled cells: Vaso-occlusive crisis, sequestration, aplasia. EMERGENCIES: CVA, acute chest, splenic sequestration, priapism. |
| Presentation of Vaso-occlusive crisis in sickle cell? | Pain: cardinal symptom. Most common clinical manifestations occur in: Bones, Lungs, Liver/spleen, Brain, Penis. Occur as isolated or multiple sites. |
| Pain episodes in sickle cell. | Occur anywhere but < 2 yrs often in hands/feet (dactylitis), > 2 yrs often in longbones, joints & abdomen. Also soft tissue swelling. Associated with infections. |
| Treatment of pain episodes in sickle cell. | Hydration, NSAIDS, opioids. |
| Acute chest syndrome in sickle cell | EMERGENCY! leading cause of death in SCD pt >10y. Causes: infection, infarction, pulmonary fat embolus. May be related to pain episode. Symptoms: fever, chest or back pain, decreased 02 sat, cough dyspnea. may look like pneumonia on CXR |
| CVA in SCD | EMERGENCY! Occurs in 7% of children with SCD Signs: Convulsions/slurred speech, taxia/weakness/paralysis. Immediate Treatment: tx symptoms,exchange transfusion Later treatment: Chronic transfusion program Neuropsych follow-up |
| Two types of Priapism seen in SCD. | STUTTERING: multiple short episodes. SEVERE PROLONGED: lasting> 24 hrs |
| Treatment of priapism | hydration, opioids, irrigation of copora cavernosa, exchange transfusion, avoid temperature extremes. |
| Splenic sequestration in SCD: incidence & symptoms | Infarction of spleen at microvascular level, Occurs primarily in children < 4 years of age, high mortality. SYMPTOMS: rapidly enlarging abdomen, L side pain, Dyspnea, CV collapse, shock. 25-50% reduction in HGB possible 2ndry to sequestration. |
| Treatment of Splenic sequestration in SCD | IMMEDIATE: restore CV volume. LONG-TERM Tx: chronic transfusion program, splenectomy. |
| Defination Aplasia (as seen in SCD) | Temporary, self resolving cessation of BM function. Usually follow an infection. 10-15% drop in HGB/day without compensatory reticulocytosis. TREATMENT supportive care, RBC transfusion, tx infection, monitor closely |
| Infection in SCD | Leading cause of death from SCD. Pt's functionally asplenic (spleen makes WBC's). |
| SCD pt who are functionally asplenic will be on what drugs as prophylaxis. | Penicillin, infant H-flu/ meningoccoccal immunization, pneumococcal vaccine at 2 yrs. |
| Most common causative organisms in infection in SCD. | Streptococus pneumonaie, hemophilus influenzae. |
| Sickle cell health maintenance | Avoid hypoxic situations, prophylatic abx & immunizations, hydration, pain management, folate supplement, chronic pRBC transfusions, iron chelation, hydroxyurea, Increased HGB F production, genetic counseling, HCT. |
| Goal of chronic transfusion therapy? | keep HGB S <30%. Done q3-4 week LT needs iron chelation. |
| Def: Autoimmune hemolytic Anemia (AIHA) | Group of disorders where antibodies are produced against surface antigens on RBC's which bind and activate with complement. |
| Causes of Autoimmune hemolytic Anemia (AIHA) | 50% idopathic. Caused by other autoimmune conditions: systemic lupus erythematosus, infections, hepatitis, EBV, mycoplasma pneumonia. Medications: penicillin & quinine. Hematologic disorders: evans syndrom Paroxysmal nocturnal hemoglobiuria. |
| Clinical presentation of Autoimmune hemolytic Anemia |
Created by:
JennRN
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