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41 Cards in this Set
- Front
- Back
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Anemia in a newborn is defined as: |
Hgb < 13
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How RBC MCV varies with age: Newborns have an MCV up to___. 6 months-2 years of age have MCV of ___ to ___. Older children-adults have MCV of ___ to ___. |
110 70-90 80-100 |
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Polycythemia -defintion -when to treat -risk factors |
Hct >65% Treatment (partial exchange transfusion) not required until >70% by a venous blood sample! Risk factors: IUGR, delayed clamping of cord, twin-twin transfusion, IDM, Down Syndrome |
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Complications of massive PRBC transfusions |
hypocalcemia, hyperkalemia, thrombocytopenia |
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Physiologic Anemia -why it occurs? -when it occurs? -lower limits for term and preterm infants |
-fetal Hgb gets slowly replaced by adult Hgb (A1) which has a lower affinity for O2 -as more A1 is made and more O2 is released thereis a decreased need for erythropoietin, results in decreased RBC production andanemia -nadir occurs between 6-16 weeks (about 2 months) -Hgb should not below 9 for term or 7 for preterm infants. |
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Rh Disease -when it occurs? -what does it cause? -when to give Rhogam? |
Occurs in an Rh neg mom during her SECOND pregnancy (makes IgM during 1st but too big to cross placenta, then makes IgG during 2nd) Can cause erythoblastosis fettles Give Rhogam at 20 weeks gestation and after delivery if baby found to be Rh positive |
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ABO incompatibility -when it occurs? |
Occurs in mothers with O blood type (have natural anti-A and anti-B antibodies) can occur in G1P1 baby! FIRST pregnancy |
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G6PD Deficiency -type of inheritance -cause -clinical presentation (in newborns and older) -microscopy findings |
X-linked recessive (in males!), usually AA or Mediterranean descent Cause: hemolysis from oxidative injury (decreased reduced glutathione) Clinical presentation: Jaundice, dark urine, anemia --In newborns --> look for male with jaundice after 24 hours --In children --> after ingestion of fava beans, malaria meds, Bactrim, cipro or nitrofurantoin Microscopy --> HEINZ bodies |
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Hereditary Spherocytosis -type of inheritance -cause -blood/micro findings -diagnosis |
Usually autosomal dominant Caused by spectrin deficiency, cells are more fragile and slightly smaller Elevated MCHC (cells smaller but same amount of Hgb) Diagnose by osmotic fragility test |
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Erythema Infectiousum -cause -clinical presentation -diagnosis |
Caused by Parvovirus B19 (can lead to aplastic crisis!) Clinical presentation: slapped cheek rash first then lacy or reticular defuse macular erythema on extensor surfaces Diagnose by IgM titers |
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Paroxysmal Nocturnal Hemoglobinuria -cause -clinical presentation -diagnosis |
Complement-mediated hemolysis Dark urine in the mornings Diagnosed by flow cytometry or Ham's test (will be COOMBS Neg because antibodies not involved) |
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Sickle Cell Anemia -multiple clinical presentations or problems: ---Vasooclusive Crisis ---Acute Chest ---Aplastic Crisis ---Splenic Sequestration ---Osteomyelitis ---Dilute Urine |
Vasooclusive Crisis = acute pain, can cause dactylitis or stroke Acute Chest = triad of pain+infiltrate+hypoxia, if Hgb low then transfuse, if high exchange transfuse Aplastic Crisis = caused by Parvovirus B19 Splenic Sequestration = abd pain+splenomegaly+shock, cells trapped in spleen so lower circulating blood volume, transfuse!! Osteomyelitis = Salmonella is more common than Staph in sicklers Dilute Urine = hyposthenuria due to renal damage |
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Sickle Cell Anemia -microscopy -diagnosis |
-Howell-Jolly bodies on smear (indicates poor spleen function) -diagnosed by Hgb Electrophoresis (shows Hgb F and S but no A) -Hgb S is a Glu-Val swap in beta chain |
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Transient Erythroblastosis of Childhood -cause -clinical presentation -can be confused with what anemia? |
-unknown cause
-healthy child with slow onset of pallor, erythropenia that occurs around 2 years old (18-24 months) -can be similar to Diamond Blackfan anemia however this occurs earlier (3-12 months), is a/w dysmorphic features and the anemia is macrocytic |
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Anemia of Chronic Disease |
Chronic inflammation that leads to a defect in the production of RBCs MCV normal, low-normal, or borderline low low TIBC |
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Iron Deficiency Anemia -potential cause in infants -diagnosis (type of anemia, ferritin, transferrin, retic, RDW, Mentzer index) -treatment |
Cause in infants: cow's milk before 1yo Diagnosis: -microcytic anemia -ferritin low, transferrin low, retic low -RDW high (old normal-sized RBCs mixed with new microcytic ones) -Mentzer Index > 12 Treatment: oral iron aka ferrous sulfate 3-6mg/kg per day,expect increase in retic to begin 3-5 days after starting therapy and peaking at7-10 days, expect Hgb increase of 1-2 in the first month |
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Alpha Thalassemia -cause -type of anemia, different types (4) -diagnosis |
Microcytic anemia 1) Alpha-thal trait = one locus, asymptomatic, silent carrier 2) Alpha-thal minor = two loci, asymptomatic, MCV low, little to no anemia 3) HbH disease = three loci, moderate-to-severe hemolysis,splenomegaly, bone changes 4) Hb Bart’s = 4 loci, death in utero (hydrops fetalis) - cannot be definitely diagnosed by electrophoresis, must do genetic testing! |
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Beta-Thalassemia -cause -type of anemia, different types (minor, major) -diagnosis |
Defectsin the beta chain genes Microcytic anemia Beta-thalassema minor = heterozygotes,asymptomatic, may have mild microcytosis, HgbA2 >3.5% is diagnostic Beta-thalassemia major (Cooley anemia) = homozygous, SEVERE microcytic anemia in 1st year of life, chipmunkfacies from expansion of bone marrow in facial bones, essentially no beta production so the remaininghigh insoluble alpha chains precipitate into inclusion bodies (Heinz bodies). Blood smear with microcytosis, target cells, andbasophilic stippling . May develop hemochromatosis from frequenttransfusions and iron overload (iron deposits causing heart failure, liverfailure, renal disease) Diagnosis by electrophoresis: elevated levels of Hgb F and Hgb A2, low levels of Hgb A1 |
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Lead Toxicity -type of anemia -clinical presentation -diagnosis -when to treat |
Microcytic anemia Clinical presentation: constipation, neuro/learning/behavior issues Basophilic stippling of RBCs and LEAD lines of long bones Treatment: always confirm capillary level with venous sample, levels >45 require CHELATION therapy with EDTA (edentate disodium calcium), dimercaprol or d-penicillamine |
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Folate (B9) Deficiency -type of anemia -clinical presentation -microscopy -treatment |
Macrocytic anemia Macroglossia In pregnant women=neural tube defects Look for goat’s milk ingestion! Hyper segmented neutrophils (also in B12 def) Treatment with folic acid of 1-5 mg daily |
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B12 (Cyanocobalamin) Deficiency -type of anemia -clinical presentation -diagnosis -treatment |
Macrocytic anemia Neurologic issues (paresthesias, ataxia,decreased reflexes) – can be irreversible! In pregnant women = neural tube defects Common causes include diet (VEGANS) or inabilityto absorb B12 in terminal ileum (INTRINSIC FACTOR DEFICIENCY aka perniciousanemia or an inability to absorb after gastric surgery/short bowel resection) Diagnosed with Schilling test Treatment with B12 injections |
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Fanconi Anemia -type of anemia -age of presentation, epidemiology -cause -clinical presentation -at risk for? -treatment |
Macrocytic Aplastic anemia usually older than 4 years, higher in Ashkenazi Jews defect in proteins responsible for DNA repair Short stature, cafe au last spots, renal abnormalities, microcephaly, hypogonadism, upper limb/hand abnormalities (malformed thumb, forearms) Treatment: bone marrow transplant at risk for cancers! AML when older |
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Diamond-Blackfan Anemia |
Macrocytic Aplastic anemia Caused by pure red cell aplasia (ONLY RED CELL LINE AFFECTED) increased MCV, low retic Triphalangeal thumbs and craniofacial abnormalities, short stature, webbed necks, CHD, glaucoma Treatment with chronic steroids |
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How to differ aplastic anemia from leukemia? |
Aplastic anemias won't have lymphadenopathy, hepatomegaly, elevated LDH or uric acid |
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Causes of aplastic anemia |
50% idiopathic benzene, radiation, sulfa drugs, gold, chloraphenicol, viruses (hepatitis, CMV, EBV, HIV, parvo) |
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Maternal Immune Thrombocytopenic Purpura or neonatal alloimmune thrombocytopenia (NAIT) |
can cause thrombocytopenia in the neonate that lasts for weeks high risk of occurrence in subsequent pregnancies |
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TAR Syndrome (Thrombocytopenia and Absent Radii) -clinical presentation -microscopy -treatment |
presents in newborns, NO radii but normal thumbs amegakaryocytic = low or absent number of megakaryocytes in bone marrow treat with plt transfusion if needed, usually improves over time |
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Idiopathic Thrombocytopenic Purpura (ITP) - clinical presentation -microscopy -treatment |
petechiae and purpuric lesions occurring after viral infection (usually 1-6 weeks) thrombocytopenia but LARGE platelets with high MPV Treat with IVIG and steroids. Transfuse if <20 and signs of bleeding. |
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Wiskott-Aldrich Syndrome -type of inheritance -clinical presentation -microscopy -at risk for? -treatment |
X-linked frequent infections (low IgM, high IgA), eczema thrombocytopenia and SMALL platelets at risk for lymphoma in third decade of life treatment: BMT |
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Kasabach-Merritt Syndrome |
infants with large, congenital vascular tumors (similar to hemangiomas) causing severe consumptive coagulopathy |
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Glanzmann Thrombasthenia |
Unexplained bleeding in a patient with normal platelet counts, normal PTT and normal INR caused by dysfunctional platelets |
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Bernard-Soulier Syndrome |
deficiency of platelet glycoprotein Ib (cannot aggregate properly) Mild thrombocytopenia with GIANT platelets and prolonged bleeding time |
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Clotting stages |
1) primary hemostasis - platelets 2) secondary hemostasis - coag factors *this is why hemophiliacs often do not have deep bleed until 12-24 hours post trauma |
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Effect of aspirin and NSAIDS on platelets |
Aspirin irreversibly acetylates cyclooxygenase. NSAIDs reversibly bind cyclooxygenase. |
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Coagulation cascade: Extrinsic factors Intrinsic factors Common pathway |
Extrinsic = factors 3 and 7 (I went to WAR(farin) with my EX(trinsic) who is a PT) Intrinsic = factors 12, 11, 9, 8 (skip 10) Common = factor 10 |
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Hemophilia A -cause -type of inheritance -treatment |
-Factor VIII deficiency (so PTT increased) - X-linked recessive (more often in males) -deep bleeds (muscles, joints, etc) -risk of bleeding correlates with the serum levels of Factor VIII -Treatment: Desmopressin (DDAVP) in mild, recombinant Factor VIII if severe |
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Hemophilia B -cause -type of inheritance -treatment |
-Factor IX deficiency (so PTT increased) -X-linked recessive -much less common than A -Treatment: recombinant Factor IX |
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von Willebrand Disease -cause -type of inheritance -PTT? PT? -treatment |
-most common type (1) is due to decrease in vWF -autosomal dominant -PTT elevated (with prolonged bleeding time) and PT typically normal -frequent first manifestation in girls is heavy menstrual bleeding -Treatment: mild with DDAVP, severe with Factor VIII |
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DIC |
thrombocytopenia, low fibrinogen, elevated D-dimer, elevated PT and PTT treatment= thrombocytopenia = platelets severe anemia = pRBCs low fibrinogen = cryoprecipitate low clotting factors = FFP *treat underlying condition |
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Vitamin K dependent factors |
2+7=9...10 |
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What lab is abnormal in an infant who did NOT receive vitamin K and has bleeding? |
PT |
