Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
70 Cards in this Set
- Front
- Back
Anemia |
low RBC number or low Hb |
|
when does fetal Hgb dissapear by? |
6-9mo |
|
low retic count |
bone marrow failure or diminished hematopoiesis |
|
5 types of microcytic, hypochromic (low MCV) anemias |
1. Iron deficiency 2. thalasemia 3. sideroblastic 4. lead toxicity 5. chronic dz |
|
major cause of Fe++ def anemia in 9mo-1yo |
iron-poor cow milk or mom's milk, meckel's, IBDz, PUDz |
|
major cause of Fe++ def anemia in adolescent girl |
menstrual blood loss |
|
spoon nails |
Fe++ def anemia |
|
what lab finding is an EARLY* finding of Fe++ def anemia? |
LOW FERRITIN* (is also an acute phase reactant, could be increased in infxn, dz, stress!!!) |
|
as iron levels fall, what happens to transferrin |
more transferrin, but less transferrin saturiation because iron binds to transferrin |
|
Tx for Fe++ def anemia |
-elemental iron (with vC for absorption) -maybe transfuse |
|
alpha Thalaseemia |
defective a-globin chain synthesis |
|
B-thalassemia |
defective B-globin chain synthesis |
|
why do bones in the face/skull, etc increase with thalassemia |
because thalassemias lead to hemolysis==>more bone marrow activity, which enlarges bone marrow spaces and thus, bones |
|
deletion*, asians |
alpha |
|
one a globin gene deleted |
no anemia, no symptoms |
|
two a-globin genes deleted |
mild anemia (thalasemmia minor) |
|
3 a-globin genes deleted |
Hgb H Dz: severe anemia at birth, with more Barts hemoglobin |
|
four a-globin genes deleted |
ONLY Hgb Barts are made, profound anemia, CHF, death |
|
only 2 beta-globin genes in each cell |
only two types of B-thalassemia: major and minor |
|
normal (or high*) Fe++ level, but microcytic anemia |
could possibly be B-Thal minor (don't give iron, won't do anything) |
|
total abscence of B-globin chains or deficient production |
B-thal MAJOR |
|
hepatosplenomegaly since infancy*, b.m. hyperplasia* (thalassemia "facies"), target cells, poikilocytes |
B-thal MAJOR |
|
Tx for B-thal MAJOR |
-transfusions for life, often splenectomy -transfusions could lead to hemachromatosis (from too much iron in heart, liver, lungs, pancrea, skin) |
|
ring sideroblasts in bone marrow d/t accum of iron* in mitochondria* of RBC precursors |
sideroblastic anemia |
|
inflamm bowel dz could lead to decreased absorption of folate |
or too much goats milk |
|
causes of B12 deficiency |
-no terminal ileum, no absorption -no I.F. by parietal cells (pernicious anemia) |
|
macrocytic anemia with RED tongue & neuro*** |
B12 deficiency |
|
no RBC membrane protein spectrin*, so RBC is shaped weird |
hereditary spherocytosis (normo) |
|
splenomegaly (spherocytes are trapped and destroyed0, gallstones, aplastic crises |
spherocytosis: look for abnormal fragility with osmotic fragitility* studies |
|
normo anemia with positive direct Coombs test |
autoimmune hemolytic anemia |
|
two types of Allo*immune hemolytic anemia |
1. Rh hemalytic Dz 2. ABO hemolytic Dz |
|
Rh- Mom makes Abs to Rh+ fetus, next time she has an Rh+ fetus, these Abs will pass to the fetus and result in: |
JAUNDICE (kernicterus), anemia, hydrops fetalis*** with positive Coombs*** |
|
Mom's blood is O, baby is A, B, or AB and mom makes antibodies against baby and passes it to baby |
direct Coombs test is weakly* positive. this one can ALSO OCCUR IN FIRST*** pregnancy (unlike the Rh one) |
|
SCDz |
single AA substitution of Valine* for Glutamic acid* on B-globin chain |
|
SCDz |
substitution causes Hb chain to "stack" when exposed to low O2* or acidosis* |
|
patient is asymptomatic, without anemia, until exposed to severe hypoxemi |
sickle cell trait* |
|
when does SCDz present |
usually after 6 months once fetal Hb starts declining |
|
always cosider SCDz in any patient with PRIAPISM* |
or swelling of digits, etc |
|
leading cause of death from SCDz |
infxn: sepsis/meningitis due to decreased splenic funtion |
|
what bacteria are SCDz patients at risk for |
encapsulated: Hib, strep pneumo, salmonella, N.M. |
|
fever in patient with scdz |
urgent!!! culture blood and urine, CXR to r/o pna, abx prophylactically |
|
osteomyelitis in sickle cell |
salmonella |
|
what drug to give scdz patient to decrease incidence of vasoocclusive crises? |
hydroxyurea |
|
daily PO pcn ppx is started in first few months of life in patient with scdz |
decrease risk for strep pneumo |
|
serial doppler U/S starting at 2 yo to identify |
scdz patients at risk for stroke |
|
Fanconi anemia |
congenital aplastic anemia |
|
7 year old has ecchymosis/petechia, pancytopenia, and no thumb/radius**, kidney abnormalities, skin hyperpigmentation* |
congenital aplastic anemia (Fanconi) |
|
secondary polycythemia |
increased EPO produciton** leading to lots of Hb and Hct but WBC and plts are normal* |
|
Tx for polycythemia |
phlebotomy |
|
two factor VIII clotting factor disorders |
1. hemophilia A 2. von Willebrands Dz |
|
Hemophilia A has normal plt function* |
vWDz has bad plt function because vWF makes it impossible for the plt to stick to blood vessel wall |
|
deep soft tissue bleeding*, hemarthroses*, -long PTT, that's it |
Hemophelia A. Tx with DDAVP |
|
inheritance of hemophelia A: x linked |
VWDz: AD |
|
epistaxis, menorrhagia, bruising/bleeding after dentist or tonsillectomy*, NO HEMARTHROSES**** |
vWDz!!!! Tx: DDAVP |
|
vK is essential for which clotting factors |
II, VII, IX, X, proteins C & S |
|
serious bleeding in newborn (ex: circumcision or umbilical cord*) |
hemorrhagic disease of the newborn d/t vK deficiency*** Tx: IM vK |
|
DIC |
clotting==>procoag factors are all consumed===>hemmorhage |
|
elevated fibrin degradation products (d dimer) with long PT, long PTT and LOW FIBRINOGEN*** |
DIC |
|
thrombocytopenia, eczema*, defects in cell immunity |
Wiskott-Aldrich |
|
Immune mediated thrombocytopenia often follows a viral infxn |
-could be that virus triggers Abs that cross-react with plts===>destruction and removal by spleen |
|
Tx ITP |
IVIg (no plt transfusion bc plts will be destroyed anyway) |
|
hypercoagulability d/t |
-protein C, S, antithrombin III, or factor V Leiden deficiency* |
|
purpura fulminans: NON*thrombocytopenic purpura with fever, shock, rapidly spreading skin bleeding and intravascular thrombosis |
protein C deficiency (not enough C to anticoagulate) |
|
deep venous or CNS thrombosis |
protein C deficiency |
|
Tx protein C deficiency |
heparin, FFP, warfarin |
|
most common cause of neutropenia during childhood |
infxn |
|
chronic benign neutropenia of childhood (CBN) |
-increased incidence of mild* infxns -low ANC with normal/slighlty low WBC* -otherwise healthy* |
|
fever, oral ulcers, stomatitis during cyclic episodes of neutropenia |
cyclic neutropenia |
|
oculocutaneous albinism*, large blue-gray granules in cytoplasm of neutrophils*, neutropenia, blond/brown hair with silver streaks |
chediak -higashi syndrome |
|
exocrine pancreatic insufficiency with malabsorption*, short*, metaphyseal dysplasia*, neutropenia |
Schwamman Diamond |