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X Pediatrics 2 > Hematology > Flashcards

Hematology Flashcards

1
Q

Definition of anemia

A

Reduction in RBC mass or blood Hb conc 2 standard deviations below normal for age and sex
<11 for all kids 6 mos-5 yrs

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2
Q

Reasons for normocytic, normochromic anemia

A

Anemia of chronic disease

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3
Q

Reasons for microcytic, hypochromic anemia

A

Iron deficiency, thalassemia, lead intoxication

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4
Q

Reasons for macrocytic anemia

A

Vitamin B12 and folate deficiency

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5
Q

Acute signs and sxs of anemia

A

Lethargy, tachycardia and pallor

Infants might be irritable and have poor oral intake

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6
Q

Types of bone marrow failure

A

Fanconi anemia and acquired aplastic anemia

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7
Q

What is Fanconi anemia?

A

Inherited bone marrow failure syndrome (autosomal recessive) due to defective DNA repair
Usually first 5 yrs of life

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8
Q

Manifestation of Fanconi anemia

A

Progressive pancytopenia
May have abnormal pigmentation of the skin, short stature, skeletal malformations
Increased malignancies

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9
Q

Lab findings of fanconi anemia

A

Thrombocytopenia or leukopenia (usually seen before the anemia)
Severe aplastic anemia
Bone marrow hypoplasia or aplasia

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10
Q

What is fanconis often misdiagnosed as?

A

ITP

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11
Q

Treatment for fanconis

A

Supportive

HSCT

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12
Q

Prognosis of fanconis anemia

A

Pts at high risk of developing myelodysplastic syndrome or AML
Many die of bleeding, infection or malignancy in adolescence

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13
Q

What is acquired aplastic anemia?

A

Peripheral pancytopenia with a hypocellular bone marrow (looks similar to fanconis)
Mostly idiopathic

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14
Q

Sxs of acquired aplastic anemia

A

Weakness, fatigue, pallor (anemia)
Frequent infections (leukopenia)
Purpura, petechiae and bleeding (thrombocytopenia)

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15
Q

Lab findings in acquired aplastic anemia

A

Anemia usually normocytic
Low WBC with neutropenia
Thrombocytopenia
Low retic count because of bone marrow failure

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16
Q

Leading causes of death in acquired aplastic anemia

A

Overwhelming infection and severe hemorrhage

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17
Q

Tx of acquired aplastic anemia

A

Supportive care, referral
Abx for infection
Transfusions
HSCT blah blah

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18
Q

What is the most common nutritional deficiency in kids?

A

Iron deficiency anemia

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19
Q

When is iron deficiency anemia more prevalent?

A

African Americans and Hispanic children

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20
Q

When is screening for anemia performed?

A

At 12 mos (determine hb conc and assess risk factors)

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21
Q

Risks of iron deficiency anemia

A

Low status
Premature/low birth weight
Lead exposure
Exclusive breast feeding beyong 4 mos without iron supplements
Moving to milk or food without iron
Feeding problems, poor growth, inadequate nutrition

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22
Q

Lab findings in iron deficiency anemia

A

Microcytic, hypochromic anemia
Hb < 11
Ferritin < 12
Usually RDW high

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23
Q

Tx for iron deficiency anemia

A

Hb of 10-11 (close follow)

IDA- iron 6 mg/kg/d with 3 divided daily doses

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24
Q

What causes B12 deficiency?

A 
Intestinal malabsorption (Crohns, ulcerative colitis, Celiac)
Dietary insufficiency (vegans)
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25
Q

What causes folate deficiency?

A

Increased folate requirements (rapid growth, chronic hemolytic anemia)
Malabsorptive syndromes
Inadequate dietary intake (rare)
Meds

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26
Q

Signs of macrocytic anemia

A

Pallor
Glossitis
B12- neurologic sxs in older children

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27
Q

Lab findings of macrocytic anemia

A

Elevated MCH and MCV
Neutrophils large and have hypersegmented nuclei
Macro-ovalocytes
Elevated methylmalonic acid in B12
Elevated homocysteine with B12 and folate

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28
Q

Tx of macrocytic anemia

A

Supplementation
Must treat the B12 deficiency to treat neurologic probs
*tx with folate will fix anemia picture in B12

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29
Q

Types of congenital hemolytic anemias

A

Hereditary spherocytosis
Thalassemia
Sickle cell
G6PD

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30
Q

Hereditary spherocytosis

A 
Red cell membrane defect
Hemolytic anemia (jaundice, splenomegaly, gallstones)
Spherocytes
Increased osmotic fragility
Maybe splenectomy
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31
Q

Thalassemia

A

Alpha or beta (severity based on gene deletions)
Hb electrophoresis for diagnosis
Support with RBC transfusion (iron monitoring + chelation, splenectomy, HSCT)

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32
Q

Most common sign of sickle cell disease

A

Vaso-occlusion (pain)

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33
Q

Tx of sickle cell disease

A

Hydroxyurea
Tx for painful vaso-occlusive episodes
Stem cell transplant

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34
Q

What is G6PD deficiency?

A

Red cell enzyme defect that causes hemolytic anemia

Highest among African, Mediterranean and Asian ancestry

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35
Q

Manifestations of G6PD deficiency

A

Neonatal jaundice, hyperbilirubinemia

Episodic hemolysis due to oxidant stress of infection or certain drugs/foods- pallor, jaundice, hemoglobinuria

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36
Q

Peripheral smear on G6PD deficiency

A

Bite like deformities and Heinz bodies

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37
Q

When might you suspect lead poisoning?

A

Older homes with lead paint

38
Q

Manifestations of lead poisoning

A

Mild, hemolytic and normocytic anemia

Basophilic stippling on peripheral smear

39
Q

Tx for lead poisoning

A

Chelation

40
Q

Types of polycythemia

A

Primary and secondary

41
Q

Familial polycythemia (congenital erythrocytosis)

A

Only RBCs affected
Hb may be as high as 27
Plethora (ruddy complexion) and splenomegaly maybe
HA and lethargy

42
Q

Tx of familial polycythemia

A

Phlebotomy

43
Q

Secondary polycythemia

A

In response to hypoxemia (cyanotic congenital heart disease like ToF or TGA or chronic pulm disease)
Tx: correction of disorder and maybe phlebotomy

44
Q

Initial screening tests for bleeding disorder

A

CBC
Peripheral smear
Pt/INR, aPTT
Maybe bleeding time

45
Q

Platelet count testing for bleeding disorders

A

Normal is 150,000-400,000

Risk of bleeding less than 20,000

46
Q

PT test

A

Measures extrinsic and common pathways (I, II, V, VII, Xa and tissue factor)- 7 and TF specific

47
Q

PTT/aPTT

A

Measures intrinsic and common pathways (I, II, V, VIII, IX, X, XI, XII)- 8, 9, 11, 12 specific

48
Q

INR testing

A

More accurate reflection of PT

Used to monitor Warfarin tx

49
Q

Bleeding time test

A

Screen test for platelet dysfunction

Prolonged in platelet disorders (von Willebrand) and severe thrombocytopenia

50
Q

What is the most common bleeding disorder of childhood?

A

Idiopathic thrombocytopenia purpura

51
Q

When does ITP occur?

A

Often after infection with viruses (immune-mediated)

52
Q

Why does ITP occur?

A

Immune mediated attack against its own platelets

53
Q

Manifestations of ITP

A

Multiple petechiae
Ecchymosis
Epistaxis

54
Q

Lab findings of ITP

A

Thrombocytopenia
Normal WBC
Normal Hb
PT and aPTT normal

55
Q

How do you diagnose ITP?

A

Diagnosis of exclusion

56
Q

Tx of ITP

A 
Observe when asymptomatic
Avoid meds that compromise platelets (ASA, NSAIDs)
Prednisone
IVIG
Splenectomy
57
Q

Inherited bleeding disorders

A

von Willebrand disease

Hemophilia

58
Q

What is the most common inherited bleeding disorder?

A

von Willebrand disease

59
Q

Genetic problem of von Willebrand disease

A

Decrease in level or impairment in action of vWF

60
Q

What is vWF?

A

A protein that binds to factor VIII and is a cofactor for platelet adhesion to endothelium

61
Q

Presentation of von willebrand disease

A

Prolonged bleeding from mucosal surfaces (epistaxis, menorrhagia, GI)
Easy bruising

62
Q

Lab findings of von Willebrand disease

A 
Normal PT
Prolonged or normal aPTT
Normal or decreased factor VIII
Normal or decreased vWF
Prolonged bleeding time (differentiate from hemophilia)
63
Q

Treatment of von willebrand disease

A

Desmopression (causes release of vwf and factor VIII from endothelial stores)
vWF replacement therapy

64
Q

Types of hemophilia

A

A- factor 8 deficiency (most common)
B- factor 9 deficiency (christmas disease)
male most common

65
Q

Presentation of hemophilia

A 
Bleeding from impaired hemostasis
Common sites into joints and muscles
Spontaneous hemarthrosis (into joint)- severe disease
66
Q

Presentation of mild hemophilia

A

Bleed in response to injury/trauma or surgery

May not be clinically apparent until later in life

67
Q

Presentation of severe hemophilia

A

Severe, spontaneous bleeding

Earlier age of first episode

68
Q

Lab findings of hemophilia

A

Normal platelet count, PT and bleeding time
Prolonged aPTT
Normal vWF

69
Q

Tx of hemophilia

A

Desmopression: hemophilia A

Factor replacement: both (to achieve sufficient hemostasis)

70
Q

What is DIC?

A

Hemorrhage and microvascular thrombosis (triggered by sepsis, trauma/tissue injury, malignancies)

71
Q

Signs and sxs of DIC

A

End-organ dysfunction (shock)
Persistent oozing from needle punctures or other invasive procedures (common)
Hematuria, melena, purpura, petechiae
Purpura fulminans, major vessel thrombosis

72
Q

Lab findings of DIC

A

*elevated D dimer and fibrin degradation products
Decreased platelets
Prolonged aPTT and PT
Decreased fibrinogen

73
Q

Tx of DIC

A

ID and treat triggering event
Replacement therapy for consumptive coagulopathy
Anticoagulant therapy

74
Q

What is synthesized in the liver?

A

Prothrombin, fibrinogen

Factors V, VII, IX, X, XII and XIII

75
Q

Which are the vitamin K dependent factors?

A

II, VII, IX and X

76
Q

Lab findings in liver disease

A

Platelet count normal in vitamin K deficiency and maybe low in liver disease
Prolonged PT, aPTT

77
Q

Tx of liver disease

A

Treat underlying condition

Vitamin K at birth

78
Q

Inherited thrombotic disorders

A

Protein C/S deficiency
Antithrombin deficiency
Factor V leiden mutation

79
Q

What is Protein C deficiency?

A

Protein C inactivated factors V and VIII

May develop warfarin-induced skin necrosis

80
Q

What is protein S deficiency?

A

Protein S is a cofactor for protein C (facilitates action of activated protein C)

81
Q

What is factor V leiden mutation?

A

Point mutation

Factor V polymorphism is resistant to inactivation by activated protein C

82
Q

VTE and factor V leiden mutation

A

Risk of VTE is increased
Among heterozygotes taking oral contraceptives
Among homozygotes much higher

83
Q

Antithrombin deficiency

A

It is a major physiologic inhibitor of thrombin

84
Q

Presentation of antithrombin deficiency

A

VTE

85
Q

Tx for bleeding disorders

A

Anticoagulant prophylaxis
UFH, LMWH, warfarin
First VTE for at least 3 mos (may need long term)

86
Q

What is Henoch-Schonlein Purpura?

A

Most common type of small vessel vasculitis
Mostly boys (2-7)
In spring and fall
URI often precedes diagnosis (like ITP)

87
Q

Pathophysiology of HSP

A

Deposition of IgA immune complexes in small vessels of skin, GI tract and kidneys

88
Q

Presentation of HSP

A

Palpable purpura
Arthritis/arthralgias
Abdominal pain
Renal disease

89
Q

Lab findings of HSP

A

Usually normal platelet count
Antistreptolysin O titer often elevated
Hemoccult may be positive
Hematuria (sometimes proteinuria)

90
Q

Tx of HSP

A

Supportive, good prognosis

X Pediatrics 2 (10 decks)

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