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125 Cards in this Set
- Front
- Back
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What is the classic cause of Fe def. anemia?
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decreased intake (lots of cows milk and not enough solid food)
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What extra serum tests do you want to check in Fe def anemia?
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-serum iron
-ferritin -TIBC |
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What index is used to tell the difference btw Fe def anemia and thalassemia trait?
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Mentzer Index
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What are some causes of congenital suppression of erythrocyte production?
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-sideroblastic anemia
-5'nucleotidase deficiency -Diamond-Blackfan anemia |
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What are 3 secondary causes of suppression of erythrocytes?
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-inflitration of the bone
-leucopenia and thryombocytopenia -anemia of chronic disease |
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What happens to ferritin in anemia of chronic disease?
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ferritin increases
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In hemolysis, the retic count will be what?
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high
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Erythrocyte destruction results from what factors?
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intrinsic or extrinsic factors
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What happens in thalassemia?
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increased stiumulus for RBC production and increased RBC precursors in the bone marrow, spleen and liver
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Because of liver involvement in Thalassemia, what can occur?
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Jaundice
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Alpha Thalassemia:
-1 gene missing -1-2 genes missing -3 genes missing -4 genes missing |
-silent carrier
-Thalessemia triat, mild anemia -severe anemia -fetal demise, hydrops fetalis |
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Beta thalassemia is not usually expressed untill when?
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4-6 months b/c it replaces fetal Hg
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What happens in Major Thalassemia Beta?
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severe anemia and facial facies
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What are the most often encountered Thalassemias?
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a or B trait
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B Thalassemia major is most common where?
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Africa, Mediterranean area, and Asia
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Alpha trait is most common in what people?
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Asian and African
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Sickle Cell disease is what kind of anemia?
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Chronic hemolytic anemia
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What is seen in blood work for Sickle Cell disease?
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-Hg of 7
-elevated WBC -elevated retic count -target cells and poikilocytes |
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What is the most common sickling syndrome?
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Hemoglobin S
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Sickle cell anemia gives one a risk of what other diseases?
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chronic anemia, bilirubin, gallstones, and aplastic crisis
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What are 2 other causes of hemolytic anemias?
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-hereditary sperocytosis
-G6PD def. |
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In hereditary spherocytosis, most have had a __________ and can also get ______
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-splenectomy
-gallstones |
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In G6PD def, there is a sudden onset of what?
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pallor, jaundice, and dark urine
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G6PD most often effects who?
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males because it is x-linked
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What is seen on PE in hereditary spherocytosis and G6PD def?
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-CHF
-jaundice is always present -splenomegaly -retic count is elevated |
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Hemolysis is due to what?
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membrane abnormalities
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In hereditary spherocytosis MCHC is _______ and you can do an _______ ______ test.
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-MCHC is high
-osmotic fragility test |
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In Hereditary elliptocytosis hemolysis may be what?
What is seen on peripheral smears? |
mild
-pyropoikilocytosis on peripheral smear |
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Name 3 diseases that are hemolysis due to enzyme defects?
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-G6PD
-Pyruvate kinase deficiency |
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What are some other causes of G6PD other than hereditary?
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-infection
-drug -chemicals -fava beans |
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In pyruvate kinase deficiency ____ is decreased in the cell.
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ATP
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What are some causes of extrinsic hemolysis?
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-ABO or Rh incompatability
-SLE -microangiopathic hemolysis (DIC, TTP) |
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Neutropenia deals with what cells?
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myeloid and lymphocyte cells
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What is seen on hx in neutropenia?
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-retard to growth
-infections of skin, gums, sinuses -lower RTI's -delayed separation of the umbilical cord |
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What is seen on PE in neutropenia?
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-chronically ill, abnormal wt and height, diminished muscle mass, listlessness
-tenderness at infection -perianal exam is important |
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What are 4 causes of congenital neutropenia?
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-Kostmann Syndrome
-Shwachman-Diamond Syndrome -Congenital Benign Neutropenia -Cyclic Neutropenia |
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Kostmann syndrome is a maturational arrest of what?
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myeloid precursors in bone marrow
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Kostmann syndrome can cause life threatening bacterial infections when?
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at birth
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What is the tx for Kostmann Syndrome?
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-BMT
-recombinant G-CSF |
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Shwachman-Diamond syndrome is characterized by what features?
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-short stature
-exocrine pancreatic dysfunction -neutropenia -aplastic anemia develops |
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In cyclic neutropenia there are preiods of what?
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normal WBC production
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What are some things that can cause an acquired neutropenia?
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-chemo
-atniconvulsants -immunosuppressive agents -benzene -viral or bacterial infections -leukemia -tremors |
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What is something that can cause increased peripheral destruction or consumption?
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hypersplenism
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What is neutrophilia?
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increased neutrophil count often seen with infection
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Leukemoid reaction has a merkedly increased what?
seen in who? |
-WBC w/immature cells
-common in infants w/infection and Down's |
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Acute myeloid leukemia is characterized by what in peripheral smear?
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very immature blasts
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Chronic granulomatous disease (CGD) is the inability of WBC's to do what?
What often forms? |
form the bactericidal product H2O2
-lymphadenopathy and recurrent abscesses for drainage |
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In Leukocyte Adhesion deficiency why is their poor healing and recurrent infections?
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WBCs can't adhere to walls to migrate to infection
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Tell me 2 things about defects in opsonization?
(sorry, didn't know how else to ask this) |
-absent or dysfunctional spleens
-problem with complement |
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What happens in Chediak-Higashi disease?
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there is an abnormal function of neutrophil granules resulting in the formation of giant granules that interfere w/bacterial killing
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What are some congenital bone marrow filuare syndromes?
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-Fanconi anemia
-thrombycytopenia -absent radii -dyskeratosis |
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What ar esome acquired bone marrow failure syndromes?
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-aplastic anemia
-paroxysmal nocturnal hemoglobinuria |
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Petechiae indicates what?
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a platelet disorder
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Bleeding rom nose, purpuric bleeding in the skin, intracranial hemorrhage, GI tract bleeding, GU bleeding indicates what?
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platelet or clotting disorder
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Hx of fat malabsorption indicates what?
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vit K deficiency
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What are the most common heridatary disorders of hemostatsis and thrombosis?
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Factor VIII and IX deficiencies
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Bone pain on exam can indicate what?
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leukemia or malignancy
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Deep hematomoas can indicate what?
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clotting protein abnormality
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Bleeding time is used to indicate what?
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numerical and functional defects of plaetelets
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PT measures what?
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Extrinsic and common pathways
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PTT measures what pathways?
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intrinsic and common
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Quantitative disorders of platelet function occurs when platelets are below what?
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150,000 aka thrombocytopenia
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What are some causes of thrombocytopenia?
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-infection
-inflitrative bone marrow process (leukemia) -congenital magakaryocytic hypoplasia -thrombocytopenia absent radii -aplastic anemia -Wiskott-Aldrich syndrome |
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What are some quantitative disorders of platelets that is causes by increased destruction?
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-ITP
-DIC -Kasabach-Merritt syndrome |
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Tell me three characteristics about ITP.
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1-antecedent viral infection usually
2-extensive bruising 3-platelets <10,000 |
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What is the tx for ITP?
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-IVIG, steroids
-chronic: splenectomy |
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What are some causes of thrombocytosis?
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-Kawasakis
-iron deficiency -neuroblastoma -more common in adults |
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What is the most common inherited bleeding disorder?
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von Willebrand disease
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What is the tx for von Willebrand disease?
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DDAVP, or nothing
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Which hemophilia is most common?
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Factor VIII (A) is most common and then Factor IX
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What factors are vit K dependent?
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-II, VII, IX, X
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What are some causes of DIC?
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-diseases
-sepsis -hypoxia -acidosis -shock -liver disease |
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What is thrombophilia?
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a hereditary hypercoaguble state
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What are some risk factors for thrombophilia
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-immobility
-infection -smoking -dehydration -indwelling venous catheter -meds |
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What are some examples of thrombophilia?
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-protein C, protein S, and anti-thrombin III deficiencies
-Factor V Leiden |
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What are some common sites of lymphadenopathy in children?
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cervica axiallary and inguinal
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Lymphadenopathy greater than what means disease process?
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>2.5cm
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Diffuse adenopathy is more suggestive of what?
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viral infection, storage disease, leukemia, or chronic autoimmune disease
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Lymphadenopathy longer than what indicates chronic?
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1 month
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Sinus tract lymphadenopathy usually indicates what?
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TB, aspergillosis, actinomycosis
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Tell me about lymphadenopathy in systemic disease.
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-not diffuse but may be tender
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80% of cancers in children are what?
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ALL
(most are B-cell subtype) |
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CML is associated with what?
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leukocytosis and splenomegaly
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What is often seen on PE in hematopoietic malignancies?
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petechiae, ecchymoses, epistaxis
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What is seen on PE for ALL?
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-fever, fatigue, bone or joint pain, and evidence of bleeding
-lymphadenopathy, hepatosplenomegaly -superior vena cava syndrome -tracheal suppresion |
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What is seen in T-cell ALL on PE?
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-anterior mediastinal mass
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What is only seen in AML?
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-gingival hypertorphy and subQ or periosteal tumor infiltrates (chloroma)
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Down children have an increase risk of what cancer if the are under age of 4?
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AML
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Leukemias most often present with what on labs?
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-increased number of WBCs or immature or primitive cells circulating in the blood
-anemia or thrombycytopenia |
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What are the 3 phases of chemo?
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-induction
-intensification/consolidation -maintanence |
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Tell me about the induction phase of chemo.
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-intralthecal administration of meds for 4-6 weeks
-steroid plus other chemo agents -97% remission after |
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What is given in the intesification/consolidation phase?
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methotrexate
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How long is the maintanence phase of chemo?
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1-2 years
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What do peds need when going thru chemo?
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supportive therapy with aggressive fluid management
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What is tumor lysis syndrome?
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high volumes of fluid, alkalination of the urine, and allopurinol
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When is BMT usually considered?
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-only if child has poor prognosis
-otherwise use only when pts have relapsed and in 2nd remission |
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What are 3 unique subtypes of AML?
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-APL (promyelocytic leukemia)
-AML in children with Down -AML in children w/monosomy 7 |
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What tx do you want to avoid in Down children?
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BMT
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NHL and Hodgkin Disease are from what cells?
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B or T lymphocytes
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NHL predominates in what pts?
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younger children
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Hodgkin disease predominates in what patients?
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adolescents
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What is the tx for NHL?
-Hodgkin disease? |
-chemo
-chemo and radiation |
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For solid tumors in children, parents most often notice what?
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an abdominal mass or lump on the trunk
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Solid tumors may cause pain and ther is no relationship between what?
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time to diagnosis and extent of disease
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What is the most common cause of solid tumor in children under 15?
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brain tumors
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What is the median age of brain tumor?
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6.5
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Half of all brain tumors are located where?
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cerebellum or brainstem
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What is the most frequent extracranial solid tumor in children?
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neuroblastoma
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What is the most frequent malignancy dx in infants?
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neuroblastoma
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Adrenal tumors are more common in _______, and thoracic and cervical tumors are more common in ______
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-children
-infants |
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Children older than 1 year of age are more likely than infants to ahve what?
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disseminated disease
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Where are some common metastasis for cancer?
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-bone marrow, bone, liver, and skin
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Paraneoplastic syndrome is characterized by what?
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-opsoclonus-myoclonus-myclonic jerking and conjugate, shooting eye movements, intractable secretory diarrhea, hypoK, and dehydration
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What tumor is an exclusive tumor of young children less than 5?
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-Wilms Tumor
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Wilms tumors are exclusive to where?
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the kidney
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2/3 of bone cancers are what?
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osteosarcomas
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Peak occurance of osteosarcomas occurs with what?
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growth spurts
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Osteosarcomas are seen where most often?
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metaphysis of long bones
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Osteosarcomas frequently metastasize to where?
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the lungs
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What is the tx for osteosarcomas?
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-chemo and resection
-no radiation |
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Where to Ewing tumors often occur?
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as pain and swelling around a bone or joint (pelvis or femur)
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What is the most common soft tissue malignancy?
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rhabdomyosarcomas
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Germ cell tumors can cause what?
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-constipation
-urinary ostruction -respiratory difficulty -neurological dysfunction |
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Ovarian tumors occur in children older than what age?
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4
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Testicular germ cell tumors occur when?
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in infancy or adolescence
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