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Peds > hematology > Flashcards

hematology Flashcards

1
Q

hgb v age

A

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

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2
Q

retic count

A

high in anemia. low if BM failure or diminished hematopoiesis

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3
Q

microcytic

A

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

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4
Q

macrocytic anemia

A

B12, folate, thiamine

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5
Q

normocytic

A

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

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6
Q

Fe def

A

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

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7
Q

thalassemia

A

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

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8
Q

alpha thalassemia

A

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

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9
Q

beta thalassemia major

A

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.

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10
Q

beta thalssemia minor

A

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.

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11
Q

sideroblastic anemia

A

ring sideroblast in BM.

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12
Q

folic acid def

A

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

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13
Q

B12 def

A 
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.
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14
Q

hereditary spherocytosis lead to

A

pigmented gallstone, aplastic crises associated with parvovirus 19

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15
Q

glycolytic enzymatic defects of RBC

A

pyruvate kinase def and glucose 6 phosphate deH def

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16
Q

pyruvate kinase def

A

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

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17
Q

AI hemolytic anemia

A

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

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

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18
Q

G6PD def

A

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

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19
Q

alloimmune hemolytic anemia

A

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

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20
Q

microangiopathic hemolytic anemia

A

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

21
Q

sickle cell

A

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)

22
Q

SCD labs

A

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

23
Q

SCD die

A

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

24
Q

scd preventative care

A

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.

25
Q

pancytopenia

A

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.

26
Q

fanconi anemia

A
  • 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
27
Q

parvovirus 19

A

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

28
Q

TEC

A

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

29
Q

polycythemias

A

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

30
Q

F 8 D

A

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

31
Q

factor 8 or 9 def

A

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

32
Q

vWb D

A

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

33
Q

thrombocytopenia

A

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

34
Q

plot function defect

A

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

35
Q

vit K def

A

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

36
Q

DIC

A

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

37
Q

hemaphilia A

A

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

38
Q

decr plt production

A

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

39
Q

incr plt destruction

A

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

40
Q

Immune thrombocytopenic purpura (ITP)

A

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

41
Q

neonatal immune mediated thrombocytopenia

A

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

42
Q

hypercoag

A

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

43
Q

protein C def

A

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

44
Q

neutropenia classify

A

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

45
Q

neutropenia etio

A

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.

46
Q

chronic benign neutropenia of childhood

A
47
Q

severe congenital agranulocytosis= kostmann syndrome

A

AR. ANC

48
Q

cyclic neutropenia

A

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

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