Brs Pedes Heme

Front 1

Anemia

Back 1

low RBC number or low Hb

Front 2

when does fetal Hgb dissapear by?

Back 2

6-9mo

Front 3

low retic count

Back 3

bone marrow failure or diminished hematopoiesis

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5 types of microcytic, hypochromic (low MCV) anemias

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1. Iron deficiency

2. thalasemia

3. sideroblastic

4. lead toxicity

5. chronic dz

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major cause of Fe++ def anemia in 9mo-1yo

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iron-poor cow milk or mom's milk, meckel's, IBDz, PUDz

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major cause of Fe++ def anemia in adolescent girl

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menstrual blood loss

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spoon nails

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Fe++ def anemia

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what lab finding is an EARLY* finding of Fe++ def anemia?

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LOW FERRITIN* (is also an acute phase reactant, could be increased in infxn, dz, stress!!!)

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as iron levels fall, what happens to transferrin

Back 9

more transferrin, but less transferrin saturiation because iron binds to transferrin

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Tx for Fe++ def anemia

Back 10

-elemental iron (with vC for absorption)

-maybe transfuse

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alpha Thalaseemia

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defective a-globin chain synthesis

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B-thalassemia

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defective B-globin chain synthesis

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why do bones in the face/skull, etc increase with thalassemia

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because thalassemias lead to hemolysis==>more bone marrow activity, which enlarges bone marrow spaces and thus, bones

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deletion*, asians

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alpha

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one a globin gene deleted

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no anemia, no symptoms

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two a-globin genes deleted

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mild anemia (thalasemmia minor)

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3 a-globin genes deleted

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Hgb H Dz: severe anemia at birth, with more Barts hemoglobin

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four a-globin genes deleted

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ONLY Hgb Barts are made, profound anemia, CHF, death

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only 2 beta-globin genes in each cell

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only two types of B-thalassemia: major and minor

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normal (or high*) Fe++ level, but microcytic anemia

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could possibly be B-Thal minor (don't give iron, won't do anything)

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total abscence of B-globin chains or deficient production

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B-thal MAJOR

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hepatosplenomegaly since infancy*, b.m. hyperplasia* (thalassemia "facies"), target cells, poikilocytes

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B-thal MAJOR

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Tx for B-thal MAJOR

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-transfusions for life, often splenectomy

-transfusions could lead to hemachromatosis (from too much iron in heart, liver, lungs, pancrea, skin)

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ring sideroblasts in bone marrow d/t accum of iron* in mitochondria* of RBC precursors

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sideroblastic anemia

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inflamm bowel dz could lead to decreased absorption of folate

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or too much goats milk

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causes of B12 deficiency

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-no terminal ileum, no absorption

-no I.F. by parietal cells (pernicious anemia)

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macrocytic anemia with RED tongue & neuro***

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B12 deficiency

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no RBC membrane protein spectrin*, so RBC is shaped weird

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hereditary spherocytosis (normo)

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splenomegaly (spherocytes are trapped and destroyed0, gallstones, aplastic crises

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spherocytosis: look for abnormal fragility with osmotic fragitility* studies

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normo anemia with positive direct Coombs test

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autoimmune hemolytic anemia

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two types of Allo*immune hemolytic anemia

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1. Rh hemalytic Dz

2. ABO hemolytic Dz

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Rh- Mom makes Abs to Rh+ fetus, next time she has an Rh+ fetus, these Abs will pass to the fetus and result in:

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JAUNDICE (kernicterus), anemia, hydrops fetalis*** with positive Coombs***

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Mom's blood is O, baby is A, B, or AB and mom makes antibodies against baby and passes it to baby

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direct Coombs test is weakly* positive. this one can ALSO OCCUR IN FIRST*** pregnancy (unlike the Rh one)

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SCDz

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single AA substitution of Valine* for Glutamic acid* on B-globin chain

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SCDz

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substitution causes Hb chain to "stack" when exposed to low O2* or acidosis*

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patient is asymptomatic, without anemia, until exposed to severe hypoxemi

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sickle cell trait*

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when does SCDz present

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usually after 6 months once fetal Hb starts declining

Front 38

always cosider SCDz in any patient with PRIAPISM*

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or swelling of digits, etc

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leading cause of death from SCDz

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infxn: sepsis/meningitis due to decreased splenic funtion

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what bacteria are SCDz patients at risk for

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encapsulated: Hib, strep pneumo, salmonella, N.M.

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fever in patient with scdz

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urgent!!! culture blood and urine, CXR to r/o pna, abx prophylactically

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osteomyelitis in sickle cell

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salmonella

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what drug to give scdz patient to decrease incidence of vasoocclusive crises?

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hydroxyurea

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daily PO pcn ppx is started in first few months of life in patient with scdz

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decrease risk for strep pneumo

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serial doppler U/S starting at 2 yo to identify

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scdz patients at risk for stroke

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Fanconi anemia

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congenital aplastic anemia

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7 year old has ecchymosis/petechia, pancytopenia, and no thumb/radius**, kidney abnormalities, skin hyperpigmentation*

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congenital aplastic anemia (Fanconi)

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secondary polycythemia

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increased EPO produciton** leading to lots of Hb and Hct but WBC and plts are normal*

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Tx for polycythemia

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phlebotomy

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two factor VIII clotting factor disorders

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1. hemophilia A

2. von Willebrands Dz

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Hemophilia A has normal plt function*

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vWDz has bad plt function because vWF makes it impossible for the plt to stick to blood vessel wall

Front 52

deep soft tissue bleeding*, hemarthroses*,

-long PTT, that's it

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Hemophelia A. Tx with DDAVP

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inheritance of hemophelia A: x linked

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VWDz: AD

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epistaxis, menorrhagia, bruising/bleeding after dentist or tonsillectomy*, NO HEMARTHROSES****

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vWDz!!!! Tx: DDAVP

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vK is essential for which clotting factors

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II, VII, IX, X, proteins C & S

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serious bleeding in newborn (ex: circumcision or umbilical cord*)

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hemorrhagic disease of the newborn d/t vK deficiency*** Tx: IM vK

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DIC

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clotting==>procoag factors are all consumed===>hemmorhage

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elevated fibrin degradation products (d dimer) with long PT, long PTT and LOW FIBRINOGEN***

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DIC

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thrombocytopenia, eczema*, defects in cell immunity

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Wiskott-Aldrich

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Immune mediated thrombocytopenia often follows a viral infxn

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-could be that virus triggers Abs that cross-react with plts===>destruction and removal by spleen

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Tx ITP

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IVIg (no plt transfusion bc plts will be destroyed anyway)

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hypercoagulability d/t

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-protein C, S, antithrombin III, or factor V Leiden deficiency*

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purpura fulminans: NON*thrombocytopenic purpura with fever, shock, rapidly spreading skin bleeding and intravascular thrombosis

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protein C deficiency (not enough C to anticoagulate)

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deep venous or CNS thrombosis

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protein C deficiency

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Tx protein C deficiency

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heparin, FFP, warfarin

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most common cause of neutropenia during childhood

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infxn

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chronic benign neutropenia of childhood (CBN)

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-increased incidence of mild* infxns

-low ANC with normal/slighlty low WBC*

-otherwise healthy*

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fever, oral ulcers, stomatitis during cyclic episodes of neutropenia

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cyclic neutropenia

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oculocutaneous albinism*, large blue-gray granules in cytoplasm of neutrophils*, neutropenia, blond/brown hair with silver streaks

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chediak -higashi syndrome

Front 70

exocrine pancreatic insufficiency with malabsorption*, short*, metaphyseal dysplasia*, neutropenia

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Schwamman Diamond