hematology

Question 1

hgb v age

Answer 1

high at birth. physiologic nadir at 2-3mo. adult lvl at puberty

Question 2

retic count

Answer 2

high in anemia. low if BM failure or diminished hematopoiesis

Question 3

microcytic

Answer 3

Fe def, thalassema, sideroblastic anemia, lead, chronic disease

Question 4

macrocytic anemia

Answer 4

B12, folate, thiamine

Question 5

normocytic

Answer 5

high retic: hemangioma, DIC, hemolytic anemia, SCD, blood loss
low retake: red cell aplasia, malig, falcon anemia

Question 6

Fe def

Answer 6

most common blood disease of infancy and childhood. most cause by inadequate intake. Fe store deplete by 6-9mo so sx start 9-24mo. occult blood loss
P: spoon shaped nails, diminished attension and ability to learn

Question 7

thalassemia

Answer 7

defective sun of Hgb chain. major hg is hgA1= 2 alpha + 2 beta
hemolysis –> incr BM activity –> BM enlarge –> incr size of bone in face skull etc

Question 8

alpha thalassemia

Answer 8

southeast asians. 4 alpha globin gene per diploid cell.

1) silent carrier- 1 deleted. no anemia, asx
2) alpha T minor: 2 delted. mild anemia
3) Hgb H disease: 3 delated. severe anema at birth. elevated Hgb Bart’s (bind O2 strongly and doesn’t release), lifelong severe anemia
4) fetal hydrops- 4. death

Question 9

beta thalassemia major

Answer 9

2 beta gene in each cell. mediterranean. cooler’s anemia - total absence of beta global chain or def production. profound hemolytic anemia + hepatosplenomegaly and BM hyperplasia = thalassemia facies. deal growth and puberty. labs- severe hypochromia and microcytosis, target cell, poikilocytes, incr uncongugated bili, LDH, serum Fe, low or absent HgbA and elevated Hgb F. tx with lifelong transfusions
C: hemachromatosis due to incr Fe absorption from intestine and from transfusion.

Question 10

beta thalssemia minor

Answer 10

mild asx anemia with hub 2-3g/dl below norm. hypo chromic, microcytosis, target cell, anisocytosis= vary size. no tx. Fe is normal or elevated so need to dd Fe def anemia.

Question 11

sideroblastic anemia

Answer 11

ring sideroblast in BM.

Question 12

folic acid def

Answer 12

anemia sx + FTT, chronic diarrhea, irritability. dx low serum folic acid. tx- diet with folic acid

Question 13

B12 def

Answer 13

combine with intrinsic factor secreted by gastric parietal cells absorb in terminal ileum
bad diet (vegan), no IF (juvenile pernicious anemia) can't absorb (crohn). 
P: sx of anemia + smooth red tongue, neuro ataxia, hyporeflexia, pos babinski.

Question 14

hereditary spherocytosis lead to

Answer 14

pigmented gallstone, aplastic crises associated with parvovirus 19

Question 15

glycolytic enzymatic defects of RBC

Answer 15

pyruvate kinase def and glucose 6 phosphate deH def

Question 16

pyruvate kinase def

Answer 16

AR. decr production of PK isoenzyme. ATP depletion. incr RBC survival.
P: pallor, j, splenomegaly, kernicterus in neonates. polychromic RBC on smear. dx by decr PK activity in RBC.
tx: transfusion and splenectomy

Question 17

AI hemolytic anemia

Answer 17

primary AIHA- idiopathic. secondary is underlying disease process like lymphoma, SLE

dx: direct coombs test is positive.
tx: transfusion- transient benefit, corticosteroids

Question 18

G6PD def

Answer 18

critical for protecting RBC from ox stress. trigger of hemolysis include infection, fav beans, drugs
P: sx for 1-2d after exposure to oxidant. hemolysis –> abd pain, V/D, fever, hemogloninuria, jaundice, hsm
lab: hemoglobinuria, incr retic, smear show bite cells and heights, heinz bodies
dx: low G6PD in RBC
tx: transfusion. no splenectomy

Question 19

alloimmune hemolytic anemia

Answer 19

newborn Rh and ABO hemolytic disease.

1) mom is Rh neg= has Rh ab. baby Rh pos so attacked. hydrops, J direct comb positive
2) ABO hemolytic disease: O mom v baby AB, A,B. weakly pos direct coomb test. can occur in first preg unlike Rh. light tx

Question 20

microangiopathic hemolytic anemia

Answer 20

mechanical damage to RBC due to passing through injured vascular endothelium. et- severe HTN, artificial heart valve, hemangioma, DIC.
P: anemia+ thrombocytopenia

Question 21

sickle cell

Answer 21

stacking when low O or acidosis

dx: hgb electrophoresis
crises: vasooclussive crises, bone crises, acute abdominal crises usually in mesenteric A, stroke, priapism, acute chest syndrome, sequestration crises, aplastic crisis (dec RBC production after parvovirus 19- decr retake and hgb) hyper hemolytic crisis (decr hub, incr retic and bili)

Question 22

SCD labs

Answer 22

rbi life span -10-50d
hg- 6-9, hct- 18-27, WBC 12-20, plt incr, bili incr, sicle cell, target cell, howel jolly bodies n smear. bone marrow erythroid hyperplasia

Question 23

SCD die

Answer 23

most from infection - 30% sepsis or meningitis in 1st 5y. so fever in SCD pt need complete workup. osteomyelitis can mimic bone crises.
salmonella infection common

Question 24

scd preventative care

Answer 24

1) hydroxyurea - chemotherapy that incr Hgb F - decr vasooclusive crisis.
2) daily oral penicillin prophylaxis in first mo to def s pneumonia infection
3) daily folic acid
4) routine immunizations
5) serial transcranial doppler US or MRA.

Question 25

pancytopenia

Answer 25

congenital aplastic anemia- fanconi anemia acqruired aplastic anemia- drugs, infection, chemical, rad. idiopathic. sx: bruising, petechiae, pallor, serious infection due to neutropenia. see hypocellualr BM.

Question 26

fanconi anemia

Answer 26

- AR. onset of 7yo with ecchymosis and petechiae. skeletal abd - short, absence of hypoplasia of thumb and radius, skin hyper pig, renal abn lab: decr hgb retic, plt,. incr hgb F. tx by RBC transfusion, corticosteroid, BM transplant

Question 27

parvovirus 19

Answer 27

not sx. associated URI s and facial rash- slapped cheeks. decr retic and hgb. normal plot spontaneous recovery in 2wk

Question 28

TEC

Answer 28

possible postviral AI. anemia after 1yo, slow onset, sx of anemia. decr hgb and retic. normal plot. no tx. spontaneous recovery

Question 29

polycythemias

Answer 29

incr in RBC relative to total blood V. hct>60%. primary polycythemia is rare, malig. secondary polycythemia due to incr erythropoietin production that could be appropriate (chronic hypoxemia 2/2 CHF or pul disease, high altitude) or inappropriate (being malig tumor) P: ruddy red facial complexion with normal liver and spleen lab: incr Hct and hgb. normal plot and wbc, EPO high tx: ID etio. phlebotomy C: thrombosis, bleeding

Question 30

F 8 D

Answer 30

include hemophilia A: defect in VIII. plt is normal. and vWb D.

Question 31

factor 8 or 9 def

Answer 31

prolong aPTT, normal PT, normal bleeding time/plt. no petechiae. has hemarthroses.

Question 32

vWb D

Answer 32

plt activity is defective bc of decr in vWf needed for plt to adhere to vessel wall. AD. type I is mild quantitive def in F8 and wWf. II: qualitative. III- absent vWf=worst P: epistaxis, menorrhagia, bruising, prolong pTT, bleeding time. normal PT/plt. no petechiae, rare hemarthroses tx: DDAVP induce vWf release from endothelial cells - use of prophylaxis bf surgery. cryoprecipitate has vWf

Question 33

thrombocytopenia

Answer 33

normal PTT, PT. prolong bleeding tim. low plt. petechiae. no hemarthroses

Question 34

plot function defect

Answer 34

normal PTT, PT, plt. prolong bleeding time. petechiae. no hemarthroses

Question 35

vit K def

Answer 35

F2,7,9,10, S,C. etio- diarrhea, pancreatic insuf biliary obstruction decr absorbtion. hemorrhage disease of newborn. 24hr after birth- present with cutaneous bleeding, hematemesis, from circumcision site, umbilical cord. prolong PTT, PT. normal bleeding time/plt. petechiae and hemarthrosis tx: prevent with IM it K. FFP for severe

Question 36

DIC

Answer 36

accelerated fibrinogenesis and fibrinolysis. consume procoag factors and result in hemorrhage. Occur 2/2 to local factors (large hemangioma) or systemic (sepsis, hypothermia, malig, heat stroke). prolong PTT, PT, bleeding time. low plot. petechiae. sometimes hemarthroses tx: tx underlying cuase. transfuse fibrinogen, FFP, plt. heparin if underlying defect can't be corrected

Question 37

hemaphilia A

Answer 37

deep soft tissue bleeding and hemarthrosi are hallmarks severe- spontaneous pleeding 5%. C: CNS bleeding is worst tx: DDAVP desmopressin acetate stimulate release of stored F8

Question 38

decr plt production

Answer 38

congenital- wiskott aldrich (thrombocyotpenia , usually small plt, eczema), thrombocytopenia absent radius syndrome (TAR):

Question 39

incr plt destruction

Answer 39

immune mediated: ITP, neonatal immune mediated thrombocytopenia drug, DIC, enlarge spleen hemolytic uremic syndrom large hemangioma sequester and destroy plt- kasabach merritt syndrome.

Question 40

Immune thrombocytopenic purpura (ITP)

Answer 40

viral, drug induced, idiopathic =most common. viral infection --> 1-4wks later, ab that cross react with plt P: cutaneous bleeding, petechiae, mucous mem bleeding (epistaxis, gum), internal bleeding is rare. lab- sticky plt. tx: supportive,IVIG if active bleeding, corticosteroid. antiD ig is second line. prog: 80% self resolve in mo. chronic if >6mo--> splenctomy

Question 41

neonatal immune mediated thrombocytopenia

Answer 41

1) passive AI thrombocytopenia (mom has ITP, ab cross placenta and kill fetal plt. mom has thrombocytopenia 2) isoimmune thrombocytopenia: mom make ab agianst fetal plt due to sensitatization of ag her own plt lack. mom's plt count is nl

Question 42

hypercoag

Answer 42

def of protein C, S or antithrombin III or must in factor V

Question 43

protein C def

Answer 43

most potent anticoag protein. homozygote = no C= purpura fulminans- non thrombocytopenia purpura- fever, shock, rapidly spreading skin bleeding and IV thrombosis hertozygote- present later with DVT or CS thrombosis dx- test for C tx: heparin, FFP, warfarin, purified C protein S, aT III, F V leiden def present the sam way. specific test for lvl and function of each factor is diagnostic

Question 44

neutropenia classify

Answer 44

risk of infection is directly related to ANC mild- 1000-15000 mod-500-1000: infect mucous me and skin, cellulitis, gingivitis, stomatitis severe

Question 45

neutropenia etio

Answer 45

in kids, infection is most common. HIV, EBC, CMV, hep A/B. bacteria (rocky mouton, typhus), malaria all suppress BM, marginate neutrophil or exhaust receive.

Question 46

chronic benign neutropenia of childhood

Question 47

severe congenital agranulocytosis= kostmann syndrome

Answer 47

AR. ANC

Question 48

cyclic neutropenia

Answer 48

fever, oral ulcer stomatitis in periods. cycles last on av of 21d. dx by cyclic nature of neutropenia- serial neutrophil counts